Musculoskeletal Fibromatoses: Radiologic-Pathologic Correlation1

Abstract

Musculoskeletal fibromatoses represent a wide spectrum of fibroblastic and myofibroblastic neoplasms with similar pathologic appearances and variable clinical behavior. These lesions can be categorized by location (superficial or deep) or by the age group predominantly affected. Superficial fibromatoses in adults (palmar and plantar) and children (calcifying aponeurotic fibroma, lipofibromatosis, and inclusion body fibromatosis) are often small slow-growing lesions; their diagnosis is suggested by location. Deep fibromatoses in adults (desmoid type and abdominal wall) and children (fibromatosis colli and myofibroma and myofibromatosis) are frequently large and more rapidly enlarging; location of these lesions may be nonspecific. Radiographic findings typically are nonspecific. Cross-sectional imaging (ultrasonography, computed tomography, or magnetic resonance [MR] imaging) reveals lesion location, extent, and involvement of adjacent structures for staging and evaluation of local recurrence. MR imaging findings of predominantly low to intermediate signal intensity, nonenhancing bands of low signal intensity on long repetition time MR images that represent collagenized regions, and extension along fascial planes ("fascial tail" sign) add specificity for diagnosis. Additional features that aid in diagnostic specificity include an abdominal wall location related to pregnancy (abdominal wall fibromatosis), a lower neck location in a young child (fibromatosis colli), an adipose component (lipofibromatosis), or multiple lesions in young children (myofibromatosis). Treatment may be conservative or surgical resection, depending on the specific diagnosis. Local recurrence is common after surgical resection owing to the infiltrative growth of these lesions. Recognition that the appearances of the various types of musculoskeletal fibromatoses reflect their pathologic characteristics improves radiologic assessment and helps optimize patient management.

Full text

2143
AFIP ARCH IVES
Mark D. Murphey, MD • Chad M. Ruble, MD • Sean M. Tyszko, LCDR,
MC, USN • Andrew M. Zbojniewicz, MD • Benjamin K. Potter, MAJ,
MC, USA • Markku Miettinen, MD
Musculoskeletal fibromatoses represent a wide spectrum of fibroblastic and
myofibroblastic neoplasms with similar pathologic appearances and vari-
able clinical behavior. These lesions can be categorized by location (super-
ficial or deep) or by the age group predominantly affected. Superficial fi-
bromatoses in adults (palmar and plantar) and children (calcifying aponeu-
rotic fibroma, lipofibromatosis, and inclusion body fibromatosis) are often
small slow-growing lesions; their diagnosis is suggested by location. Deep
fibromatoses in adults (desmoid type and abdominal wall) and children (fi-
bromatosis colli and myofibroma and myofibromatosis) are frequently large
and more rapidly enlarging; location of these lesions may be nonspecific.
Radiographic findings typically are nonspecific. Cross-sectional imaging
(ultrasonography, computed tomography, or magnetic resonance [MR]
imaging) reveals lesion location, extent, and involvement of adjacent struc-
tures for staging and evaluation of local recurrence. MR imaging findings
of predominantly low to intermediate signal intensity, nonenhancing bands
of low signal intensity on long repetition time MR images that represent
collagenized regions, and extension along fascial planes (“fascial tail” sign)
add specificity for diagnosis. Additional features that aid in diagnostic spec-
ificity include an abdominal wall location related to pregnancy (abdominal
wall fibromatosis), a lower neck location in a young child (fibromatosis
colli), an adipose component (lipofibromatosis), or multiple lesions in
young children (myofibromatosis). Treatment may be conservative or surgi-
cal resection, depending on the specific diagnosis. Local recurrence is com-
mon after surgical resection owing to the infiltrative growth of these lesions.
Recognition that the appearances of the various types of musculoskeletal
fibromatoses reflect their pathologic characteristics improves radiologic as-
sessment and helps optimize patient management.
radiographics.rsna.org
From the Archives of the AFIP
Musculoskeletal Fibromatoses:
Radiologic-Pathologic Correlation1
LEARNI NG
OBJECTIVE S
FOR TEST 6
After reading this
article and taking
the test, the reader
will be able to:
Identify the radio- ■
logic manifestations
of the musculoskele-
tal fibromatoses.
Describe the ■
pathologic basis
of the radiologic
features of the mus-
culoskeletal fibro-
matoses.
Discuss the patho- ■
logic appearance
and variations of
the musculoskeletal
fibromatoses as well
as the treatment op-
tions and prognoses.
RadioGraphics 2009; 29:2143–2176 • Published online 10.1148/rg.297095138 • Content Code:
1From the Department of Radiologic Pathology (M.D.M., C.M.R.) and Department of Soft Tissue and Orthopedic Pathology (M.M.), Armed Forces
Institute of Pathology, 6825 16th St NW, Building 54, Room M-133A, Washington, DC 20306; Department of Radiology and Nuclear Medicine,
Uniformed Services University of the Health Sciences, Bethesda, Md (M.D.M.); Department of Radiology, National Naval Medical Center, Bethesda,
Md (S.M.T.); Department of Radiology, West Virginia University, Morgantown, WV (A.M.Z.); and Integrated Department of Orthopaedics and
Rehabilitation, Walter Reed Army Medical Center, Washington, DC (B.K.P.). Received June 30, 2009; revision requested July 29 and received August 21;
accepted August 26. All authors have no financial relationships to disclose. Address correspondence to M.D.M. (e-mail: murphey@ap.osd.mil).
The opinions or assertions contained herein are the private views of the authors and are not to be construed as official nor as reflecting the views of
the Departments of the Army, Navy, or Defense.
See last page
TEACHING
POINTS
CME FEATURE
See accompanying
test at http://
www.rsna.org
/education
/rg_cme.html
Note: This copy is for your personal non-commercial use only. To order presentation-ready
copies for distribution to your colleagues or clients, contact us at www.rsna.org/rsnarights.

2144 November-December 2009 radiographics.rsna.org
Introduction
The musculoskeletal fibromatoses represent a
wide range of fibroblastic to myofibroblastic pro-
liferations that are grouped together because of
their similar pathologic appearances. The clinical
behavior of these tumors is intermediate between
that of benign and malignant fibrous lesions; they
commonly manifest infiltrative growth, resulting
in frequent local recurrence but lacking meta-
static potential. The World Health Organization
(WHO) Committee for Classification of Soft
Tissue Tumors in 2002 (1) categorized these le-
sions as superficial or deep, based on their ana-
tomic location. The superficial fibromatoses in-
clude palmar and plantar fibromatosis. The deep
fibromatoses include desmoid type and abdomi-
nal wall fibromatosis. Several types of fibroma-
tosis primarily affect children, and these include
fibromatosis colli, lipofibromatosis, calcifying
aponeurotic fibroma, inclusion body fibromatosis,
and myofibroma and myofibromatosis.
The superficial fibromatoses, whether they
occur in adults (palmar and plantar) or children
(calcifying aponeurotic fibroma, lipofibromato-
sis, and inclusion body fibromatosis), are typi-
cally small lesions that grow slowly. Diagnosis
of these superficial lesions is often suggested by
their clinical appearances and location. The deep
fibromatoses in both adults (desmoid type and
abdominal wall) and children (fibromatosis colli
and myofibroma and myofibromatosis) are usu-
ally larger and often enlarge more rapidly. The
diagnosis of deep fibromatoses may be suggested
by their clinical characteristics, particularly
anatomic location and patient age: fibromatosis
colli, which typically involves the lower neck and
sternocleidomastoid muscle in a young child; ab-
dominal wall fibromatosis, which manifests as a
mass involving the rectus abdominus muscle and
is often related to pregnancy; or myofibromatosis,
which occurs as multicentric disease in a young
child. However, the clinical findings of desmoid
type fibromatosis are often nonspecific. Cross-
sectional imaging (ultrasonography [US], com-
puted tomography [CT], or magnetic resonance
[MR] imaging) reveals lesion location, extent,
and involvement of adjacent structures and thus
is useful for tumor staging and evaluation of lo-
cal recurrence. Additional MR imaging features,
which are related to underlying pathologic char-
acteristics, provide increased specificity for diag-
nosis of musculoskeletal fibromatoses. Treatment
of musculoskeletal fibromatoses may range from
conservative management to surgical resection
and is influenced by the specific diagnosis and
lesion extent determined at imaging evaluation.
Local recurrence is common after surgical resec-
tion, owing to the infiltrative growth seen patho-
logically in these lesions.
In this article, we review, illustrate, and cor-
relate the clinical, pathologic, and radiologic
features of the various types of musculoskeletal
fibromatoses and discuss their treatment and
prognosis.
Superficial Fibromatoses
Palmar Fibromatosis
Clinical Features.—Palmar fibromatosis was
originally described in 1831 by the French
physician Dupuytren and is often referred to
as Dupuytren disease or contracture (1–5). It
is the most common of the superficial fibroma-
toses, affecting 1%–2% of the general population
(1–5). The disease is seen almost exclusively
in Caucasians and is particularly frequent in
those of Northern European ancestry and in
lands settled by immigrants from these areas.
The highest prevalence of palmar fibromatosis
is seen in northern Scotland, Iceland, Norway,
and Australia (5). Palmar fibromatosis is rare in
populations of African or Asian descent. The dis-
ease most commonly occurs in patients over 65
years of age, with a frequency of 20% in this age
group (1–6). Men are three to four times more
likely to be affected by the disease than women,
and lesions are bilateral in 40%–60% of patients
(1–5). The etiology of palmar fibromatosis is
not completely understood, but it is thought to
be multifactorial, including associations with
trauma, microvascular injury, immunologic
processes, and genetic factors (up to 68% of pa-
tients may have a family history of musculoskel-
etal fibromatoses) (1–5).
Patients present clinically with painless, sub-
cutaneous nodules. These nodules may progress
slowly (over months to years) to fibrous cords or
bands that attach to and cause traction on the
underlying flexor tendons, resulting in flexion
contractures of the digits (Dupuytren contrac-
tures) (Fig 1). The ulnar sided rays, particularly
the fourth and fifth digits, are most commonly
involved in palmar fibromatosis (1). Patients with
this disease commonly have other types of fibro-
matoses, including plantar fibromatosis (5%–20%
of cases), Peyronie disease, and knuckle pads (3,
5). Knuckle pad involvement (or knuckle pad
fibromatosis) is caused by focal fibrous thickening
dorsally at the proximal interphalangeal (PIP) or
metacarpalphalangeal (MCP) joint (Fig 2), may
precede the development of palmar fibromatosis,
and is usually asymptomatic (7). Additional dis-
eases that are associated with palmar fibromatosis
include diabetes mellitus (20% of patients), epi-
lepsy (50% of male patients and 25% of female
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RG ■ Volume 29 • Number 7 Murphey et al 2145
Figure 2. Knuckle pad fibromatosis in a 29-year-old man. (a) Clinical
photograph shows nodular thickening over the dorsum of the PIP joint (ar-
row). (b) Lateral radiograph reveals nodular thickening over the third and
fourth PIP joints of the hand (arrows). (c) Sagittal T1-weighted (516/10)
MR image demonstrates an intermediate-signal-intensity soft-tissue mass
dorsal to the PIP joint of the third digit (arrowhead). (d) Sagittal fat-
suppressed T1-weighted (567/10) postcontrast MR image shows moderate
diffuse enhancement of the knuckle pad mass (arrowhead). (e) Axial fat-
suppressed proton density–weighted (2850/25) MR image shows intermedi-
ate signal intensity in the soft-tissue mass (arrowhead).
Figure 1. Palmar fibromatosis in a 62-year-old man with
ulnar digit contractures (Dupuytren contractures). (a) Clinical
photograph reveals thick bands that cause flexion contractures
of the ulnar sided digits and associated puckering of the pal-
mar skin (arrows). (b, c) Axial (b) and sagittal (c) T1-weighted
(repetition time msec/echo time msec = 633/20) MR images
show low-signal-intensity superficial bands (arrows) that cause
flexion contractures because of their attachment to the flexor
tendons (T). (d) Intraoperative photograph demonstrates
initial release and resection of the band of palmar fibromatosis
(arrowheads) from the flexor tendon sheath (T).

2146 November-December 2009 radiographics.rsna.org
of tendon) on T1- and T2-weighted images (Fig
1). In 18% of cordlike lesions, the signal intensity
was slightly higher than that of tendon (intermedi-
ate to low signal) on T1- and T2-weighted images
(13). The nodular masses had more variable signal
intensity, with 85% revealing intermediate signal
intensity on both T1- and T2-weighted images and
15% showing low signal intensity with all pulse
sequences (Figs 1, 2). The corresponding histo-
logic analysis revealed that the lesions of low signal
intensity with all pulse sequences contained rela-
tive hypocellularity and abundant dense collagen.
In contradistinction, the lesions of intermediate
signal intensity on both T1- and T2-weighted im-
ages were more cellular or mixed, with less abun-
dant collagen (13). Lesions with a higher cellular
component have been shown to have a higher local
recurrence rate following local excision (13,14).
This information is important because preop-
erative MR imaging may assist the surgeon in
determining the appropriate timing for excision
(13,14). Postcontrast MR imaging (ie, performed
after intravenous administration of contrast mate-
rial) often reveals diffuse enhancement of variable
degree—but, in our experience, more prominent-
ly—in lesions with increased cellularity (Fig 2).
Treatment and Prognosis.—There is no signifi-
cant evidence supporting the efficacy of any non-
operative treatment of palmar fibromatosis such
as splinting, topical agents, or steroid injections
(15,16). Although spontaneous regression of pal-
mar fibromatosis has been reported in rare cases,
surgical excision remains the treatment of choice
(Fig 1). Surgical intervention is usually indicated
for disease that causes flexion contracture greater
than 20° at the MCP joints or more than 30° at
the PIP joints (15). More recently, indications for
surgery have depended more directly on patient
symptoms, although the aforementioned criteria
remain useful guidelines. Operative treatments
have included fasciotomy (selective or segmental)
and radical fasciectomy. However, use of radi-
cal fasciectomy has been largely abandoned, and
selective fasciectomy of only diseased locations is
now most commonly advocated (15,17). The low-
est rate of recurrence (less than 10%) has been re-
ported in those patients who undergo dermatofas-
ciectomy with skin grafting, although this extensive
surgery should be reserved for patients with severe
disease (18–20). Surgical complications, including
infection, digit ischemia, and digital nerve injury,
occur in approximately 17% of patients, with high-
er rates reported in cases of recurrent disease (16).
Local recurrence is common, affecting 30%–
40% of patients undergoing local excision and
as high as 40%–50% at 5 years after surgery
patients), alcoholism (particularly liver disease
related to alcoholism), and keloids (1–5).
Pathologic Features.—At gross pathologic exam-
ination, palmar fibromatosis lesions appear gray-
white or gray-yellow, depending on their collagen
content. The nodules in palmar fibromatosis are
typically very small (<1 cm) and often coalescent.
Lesions are usually intimately related to the palmar
aponeurosis and may be adherent to the overlying
skin, causing puckering or dimpling (Fig 1).
At histologic analysis, palmar fibromatosis dem-
onstrates a uniform fibroblastic-myofibroblastic
proliferation of spindle-shaped cells with variably
prominent vascularity, although the vascularity is
typically less than that seen in desmoid type fibro-
matosis (6,8,9). The degree of cellularity depends
on the age of the lesion, with younger lesions (pro-
liferative phase) revealing hypercellularity. Older,
more chronic lesions demonstrate much less cel-
lularity, with relatively more prominent collagen
content. Moderate mitotic activity can be present
but is not indicative of malignancy. Cytogenetic
aberrations, particularly a gain at chromosome 7
or 8, have been detected in approximately 10% of
cases of palmar fibromatosis (1–5).
Imaging Features.—Radiographic findings of
palmar fibromatosis are typically normal, other
than the flexion contractures. US reveals hy-
pervascular, hypoechoic nodules in the palmar
subcutaneous tissues, superficial to the flexor ten-
dons (10–12). US also allows real-time dynamic
assessment of the integrity of the flexor mecha-
nism of the digits. CT shows nonspecific, nodular
regions of thickening with attenuation values
similar to or slightly higher than those of muscle.
At MR imaging, palmar fibromatosis appears as
multiple nodular (including knuckle pad fibro-
matosis) or cordlike, superficial soft-tissue masses
that arise from the proximal palmar aponeurosis
and extend superficially in parallel with the flexor
tendons (Figs 1, 2). Lesion length varies from 10
to 55 mm (2–10 mm in diameter), and lesions ter-
minate in either a branching or nodular configura-
tion at the level of the distal metacarpal (13).
The intrinsic appearance of palmar fibromatosis
is variable. Yacoe and co-workers (13) evaluated
the MR imaging appearance of palmar fibromato-
sis in 11 patients and correlated the findings with
the histologic cellularity of the lesions (3). These
authors observed 22 cordlike and 13 nodular soft-
tissue masses on MR images of these patients (13).
In 82% of the cordlike masses, the signal intensity
was predominantly hypointense (similar to that
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RG ■ Volume 29 • Number 7 Murphey et al 2147
(18,20). Despite the significant incidence of lo-
cal recurrence, flexion contractures are reliably
improved with surgical intervention, and only
approximately 17% of patients with recurrent
disease require reoperation (19). In the study of
Yacoe and colleagues (13), active lesions with
increased cellularity (with intermediate signal
intensity on T1- and T2-weighted MR images)
correlated with a higher local recurrence rate.
We believe that MR imaging, in addition to de-
termining lesion extent, may help indicate which
lesions are optimal for excision and may help
lessen the likelihood of local recurrence following
surgery (21). Lesions demonstrating higher de-
grees of cellularity at MR imaging may be better
managed by delaying surgical intervention, thus
allowing them to mature with increased collagen-
ization before the initial resection (21). Follow-up
MR imaging may reveal evidence of this matura-
tion, as the lesions progressively demonstrate in-
creasingly lower signal intensity (13,21).
Plantar Fibromatosis
Clinical Features.—Plantar fibromatosis was
reported by Dupuytren in 1832 but was more
extensively described by Ledderhose in 1897 (3).
It is frequently referred to as Ledderhose disease.
Plantar fibromatosis occurs less often than palmar
Figure 3. Plantar fibromatosis in a 27-year-old
woman with a slowly enlarging soft-tissue mass.
(a) Clinical photograph shows a medial plantar soft-
tissue mass (arrows). (b) Short-axis CT image of the
foot reveals a soft-tissue-attenuation mass (*) in the
medial aspect of and extending deep to the plantar
aponeurosis (arrowheads). (c) Photomicrograph
(original magnification, ×200; hematoxylin-eosin
[H-E] stain) reveals a relatively hypercellular
tumor composed of fascicles of fibroblasts that
represent the more proliferative phase of plantar
fibromatosis.
disease, with a prevalence of 0.23% (22). Although
the frequency of the condition increases with age
(similar to palmar fibromatosis), plantar disease
is seen most often in patients aged 30–50 years
(1–5). In a large study from the Armed Forces
Institute of Pathology (AFIP) by Fetsch and col-
leagues (6), 44% of patients were younger than 30
years of age. Men are affected twice as commonly
as women, although there is a marked female pre-
dilection among the pediatric age group. Bilateral
involvement is seen in 20%–50% of patients and
is typically metachronous with a 2–7-year inter-
val (1–5). Concomitant palmar disease occurs in
10%–65% of patients and is usually metachronous
with a 5–40-year interval (only rarely synchro-
nous) (1–5). Knuckle pads are seen in 42% of pa-
tients (5). Similar to palmar fibromatosis, plantar
disease is believed to have a multifactorial etiology,
including genetic and traumatic causes. The dis-
ease is also seen more commonly in patients with
diabetes mellitus, epilepsy, keloids, and alcoholism
with liver disease (1–5).
Patients typically present with a soft-tissue
mass composed of one (plantar fibroma) or more
firm, subcutaneous nodules on the medial as-
pect of the sole of the foot (Fig 3). Nodules may
be multiple in 33% of cases (3,5). Patients are
frequently asymptomatic; less commonly, they
may experience pain, which is usually associated

2148 November-December 2009 radiographics.rsna.org
Figure 4. Plantar fibromatosis in a 6-year-old girl with a palpable mass along the plantar aspect of the foot.
(a) US image of the foot shows an elongated hypoechoic mass (arrows) along the plantar aponeurosis. (b) Short-
axis T1-weighted (467/14) MR image reveals an intermediate-signal-intensity soft-tissue mass (arrows) along the
plantar aponeurosis (arrowheads). (c) Short-axis proton density–weighted (3000/60) MR image (obtained more
distally than b) demonstrates intermediate signal intensity within the lesion (arrows) adjacent to the plantar
aponeurosis (arrowheads). (d) Sagittal fat-suppressed T1-weighted (700/20) postcontrast MR image shows dif-
fuse enhancement of the lesion (arrow) that extends along the plantar aponeurosis, a characteristic referred to as
the fascial tail sign (arrowheads). (e) Photomicrograph (original magnification, ×150; H-E stain) reveals a rela-
tively hypocellular tumor that represents a more chronic stage of disease. The lesion is composed of fibroblasts in
a prominently collagenized stroma (pink areas), elongated vessels, and extracellular keloidal collagen (arrows).

RG ■ Volume 29 • Number 7 Murphey et al 2149
(100%) and T2-weighted (78%) MR images (29)
(Fig 4). Although plantar lesions less frequently
manifest with higher signal intensity on T2-
weighted MR images (22% of cases), they often
reveal higher signal intensity with fat suppression
pulse sequences (83%) (29). Enhancement follow-
ing contrast material administration is common
and was reported in 93% of lesions by Morrison
and co-workers (29) (Fig 4). The degree of en-
hancement was marked in 64% and mild in 36%
of these lesions (29). In our experience, linear tails
of extension (“fascial tail” sign) along the plantar
aponeurosis are common and often best seen on
postcontrast images (Fig 4) (3,14).
Treatment and Prognosis.—In contradis-
tinction to the therapy for palmar fibromatosis,
treatment of plantar fibromatosis is conservative
in the majority of patients and consists of foot-
wear modifications, pads, or orthotics aimed at
relieving symptoms (30). Intralesional steroid
injections have been successful in the treatment
of some cases. Surgical resection is reserved for
large, infiltrative lesions that cause significant
disability and that are refractory to nonopera-
tive management. Historically, surgical treatment
consisting of simple excision resulted in high
rates of local recurrence (20%–40%), with the
majority of recurrent lesions developing within
the first postoperative year (1–5). Thus, wide
excision, including resection of the normal fascia
(ie, at surgical inspection) proximal and distal to
the lesion or lesions, is now advocated (31,32).
The weight-bearing surface of the plantar pad
should generally be avoided, and incisions should
conform to the cutaneous blood supply, when
practicable (31). Radiation therapy in the foot is
often poorly tolerated and is therefore typically
reserved as an adjuvant to augment treatment
following wide reexcision or for unresectable
recurrent lesions (3,33). An increased rate of lo-
cal recurrence has been associated with multiple
nodules, bilateral lesions, postoperative neuro-
mas, and a positive family history (1–5).
with prolonged standing or walking. Unlike pal-
mar fibromatosis, plantar lesions typically do
not cause symptoms such as contracture. In rare
cases, large lesions may affect or invade adjacent
muscles or neurovascular structures (23).
Pathologic Features.—At gross pathologic and
histologic examination, the appearance of plantar
fibromatosis is identical to that already described
for palmar fibromatosis (Figs 3, 4). Plantar fibro-
matosis frequently forms larger masses (2–3 cm)
and often coalescent nodules, compared with pal-
mar lesions (6,24). Lesions are typically intimately
related to the plantar aponeurosis and may be ad-
herent to the overlying skin. Mitotic activity can be
more prominent in larger lesions. Plantar fibroma-
tosis may also show cytogenetic aberrations, with
trisomies at chromosomes 8 and 14 (1,5).
Imaging Features.—Radiographic findings are
invariably normal in patients with plantar fibro-
matosis. At US, plantar lesions appear similar to
palmar fibromatosis, typically as hypoechoic or
mixed echogenic nodules in the subcutaneous tis-
sues superficial to the plantar aponeurosis (which
is often thickened), either medially (60% of cas-
es) or centrally (40%) (Fig 4) (12). These lesions
may be well defined (64% of cases) or ill defined
(36%), and frequently they are fusiform (76%)
(Fig 4) (25–27). Color Doppler sonography re-
veals hypervascularity in 92% of cases (25–28).
CT demonstrates a nonspecific soft-tissue mass
with attenuation similar to or mildly higher than
that of muscle (3,4) (Fig 3).
The typical MR imaging appearance of plantar
fibromatosis is a well- or ill-defined superficial
soft-tissue mass occurring along the deep plantar
aponeurosis (Fig 4). The lesion is usually in-
separable from the adjacent plantar musculature,
although deep invasion may occur in a minority of
cases. The medial aspect of the plantar aponeu-
rosis is involved most frequently (78% of cases)
(Fig 4), with the lateral portion affected less often
(22%) (29). The lesion typically shows heteroge-
neous signal intensity (92% of cases) and predom-
inantly low to intermediate signal intensity (similar
or equal to that of skeletal muscle) on T1-weighted
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2150 November-December 2009 radiographics.rsna.org
Figure 5. Desmoid type fibromatosis in the thigh of a 51-year-old man with recent sciatic nerve symptoms
and a history of a soft-tissue mass in the posterior left thigh and multitrauma. (a) Doppler US image of the
thigh reveals a nonspecific soft-tissue mass with heterogeneous echogenicity (curved arrows) and mild inter-
nal flow (straight arrows). (b) Axial CT image of the thigh shows an intermuscular soft-tissue-attenuation
mass (arrows) with a surrounding rim of fat (“split fat” sign) in the posterior compartment. (c, d) Coronal
T1-weighted (609/13) MR image (c) and coronal fat-suppressed T2-weighted (5740/43) MR image (d) dem-
onstrate the heterogeneous soft-tissue mass (arrowheads), which has bands of low signal intensity (straight
arrows), the intermuscular split fat sign in c, and predominantly low to intermediate signal intensity in d. Both
MR images show linear extension inferiorly (curved arrow) along the fascia (fascial tail sign). (e) Photograph
of the sectioned gross specimen demonstrates a well-defined mass with whitish bands (arrows) that represent
the more collagenized regions and correspond to the MR imaging features.

RG ■ Volume 29 • Number 7 Murphey et al 2151
Deep Fibromatoses
Desmoid Type Fibromatosis
Clinical Features.—The WHO Committee for
Classification of Soft Tissue Tumors in 2002
designated the term desmoid type bromatosis for
this lesion (1). Previous terminology has includ-
ed aggressive fibromatosis, musculoaponeurotic
fibromatosis, and extraabdominal desmoid tu-
mor. The term desmoid was coined in 1838 from
the Greek desmos (meaning band or tendon) to
emphasize the bandlike or tendonlike morphol-
ogy of these lesions (1–5).
Desmoid type fibromatosis occurs most fre-
quently in patients in the 2nd to 4th decades of
life, with a peak incidence between the ages of
25 to 35 years (1–5). An estimated two to four
people per million of population are affected by
this lesion. Children are uncommonly involved,
with less than 5% of lesions affecting this age
group (5). Overall, the disease has a female
predilection, although this tendency is more ap-
parent among younger patients. Desmoid type
fibromatosis in older patients occurs with equal
distribution in men and women.
Desmoid type fibromatosis typically manifests
as a deeply seated but poorly circumscribed soft-
tissue mass. Slow insidious growth is common,
and lesions are usually painless. Additional symp-
toms of decreased mobility, reduced joint motion,
and neurologic complaints (numbness, tingling,
sharp pain, or motor weakness) are less frequent.
The most commonly encountered locations of
desmoid type fibromatosis in a large AFIP se-
ries of benign soft-tissue tumors included the
shoulder or upper arm (28% of cases), chest wall
or paraspinal region (17%), thigh (12%), neck
(8%), knee (7%), pelvis or buttock (6%), lower
leg (5%), forearm or hand (5%), and head (2%)
(1–5). Multicentric disease occurs in 10%–15%
of cases (1–5). Shoulder and neck lesions are
most frequent about the scapula, deltoid, axilla,
and supraclavicular regions. These lesions com-
monly insinuate about vital structures, including
the axillary vessels, trachea, and brachial plexus,
a tendency that limits the extent of surgical
resection. Pelvic lesions are usually posterior
and affect the gluteal regions. Lower extremity
desmoid type fibromatosis can involve the ante-
rior quadriceps musculature or, more often, the
popliteal fossa. Head and neck lesions account
for up to 23% of cases and are more common in
children (up to 30% of lesions) (5). In the head,
desmoid type fibromatosis affects, in order of
decreasing frequency, the face, oral cavity, scalp,
paranasal sinus, and orbit. Similar to lesions in
the neck, desmoid type fibromatosis involving the
head may encroach on vital structure, including
the skull base, a characteristic that limits surgi-
cal resection. Lesions affecting younger patients
(<30 years old) and involving the head and neck
have more aggressive behavior than those found
in other sites and age groups. Such lesions have
increased local recurrence rates.
Pathologic Features.—Desmoid type fibro-
matosis is a benign fibroblastic tumor that is
capable of locally aggressive growth, but it does
not metastasize. At gross pathologic examina-
tion, these lesions are usually recognized as solid,
firm, coarsely trabeculated, whitish masses that
resemble a scar and have a gritty texture when
cut (Figs 5, 6). The firm consistency of desmoid
type fibromatosis may be observed during biopsy,
at which time a needle may glance off the lesion
rather than penetrate it. These tumors are usually
5–10 cm in size but vary widely from relatively
small focal lesions to large bulky masses that can
exceed 15 cm in maximum diameter. Tumor
margins are often spiculated and infiltrative, both
into muscle and subcutaneous tissue (Figs 6,
7). In addition, these lesions may extend along
fascial planes for substantial distances from the
main tumor focus (Fig 7).

2152 November-December 2009 radiographics.rsna.org
Figure 6. Desmoid type fibromatosis involving the chest wall in a 54-year-old woman with a 5-month history of
discomfort and swelling in the right infraclavicular region. (a) Axial CT image shows a nonspecific soft-tissue mass (ar-
rows) with central areas of low attenuation (*), compared with that of adjacent muscle, that correspond to myxoid de-
generation seen in histologic specimens (not shown). There is extrinsic erosion of the adjacent rib (arrowhead) and in-
volvement of the brachial plexus region. (b–d) Axial T1-weighted (b, 667/20), T1-weighted postcontrast (c, 516/20), and
T2-weighted (d, 6000/42) MR images reveal the heterogeneous chest wall mass (arrowheads), which has ill-defined
infiltrative margins. The mass shows predominantly low to intermediate signal intensity on the T1- and T2-weighted
images (b, d), and diffuse enhancement following contrast material administration (c). Low-signal-intensity bands (*)
are best seen on the postcontrast image because they lack enhancement. (e) Photograph of the sectioned gross speci-
men demonstrates white collagenized bands (between arrowheads) and irregular spiculated margins (arrows) that cor-
respond to the MR imaging findings. Scale is in centimeters. (f) Photomicrograph (original magnification, ×100; H-E
stain) also illustrates the marginal invasion of muscle (M) and fat (A) by the collagenized tumor (T).

RG ■ Volume 29 • Number 7 Murphey et al 2153
Desmoid type fibromatosis is composed of
uniform spindle-shaped cells and often forms
poorly defined fascicles within a collagenous
stroma. The degree of cellularity is generally
moderate; however, the extent of cellularity and
collagen is variable. Keloidal-like collagen may
be present. In our experience, the trabeculated
pattern seen at gross pathologic examination
corresponds to the more collagenized regions
observed histologically (Figs 5, 6, 8). These le-
sions typically have prominent, often mildly
dilated, thick-walled intratumoral capillaries.
Areas of myxoid degeneration and hemorrhage
may be seen. Nuclear pleomorphism is not a
feature of desmoid type fibromatosis. Mitotic
activity is relatively low and necrosis is rare.
All forms of desmoid type fibromatoses are
believed to have similar pathogenesis: activation
of the β-catenin signaling pathway. Activation
can occur either by APC-gene mutations (gene
in the long arm of chromosome 5q21-22), usu-
ally in connection with familial polyposis syn-
drome (also referred to as Gardner syndrome),
or by somatic β-catenin mutations (1–5,34). In
either event, β-catenin is overexpressed, which
leads to strongly positive nuclear immunohis-
tochemical staining. Additional cytogenetic
aberrations in these lesions include trisomies of
chromosome 8 or chromosome 20, a character-
istic seen in 30% of patients (1–5).
Figure 7. Local recurrence of desmoid type fibroma-
tosis of the popliteal region in a 28-year-old woman.
(a, b) Sagittal T1-weighted (a, 500/16) and axial T2-
weighted (b, 3000/100) MR images show a large hetero-
geneous soft-tissue mass (arrowheads) in the popliteal
fossa that contains numerous dense fibrous bands
(straight arrows). These bands are hypointense with both
pulse sequences, but the lesion has predominantly high
signal intensity on the long repetition time image (b).
The lesion extends inferiorly along the superficial fascia
(fascial tail sign) beyond the imaged region and initial
surgical resection (curved arrows in a). (c) Sagittal
T1-weighted (750/16) MR image obtained 18 months
after resection reveals recurrence of the lesion at the
nonresected inferior margin (between arrowheads).
(d) Photograph of the sectioned gross specimen dem-
onstrates the recurrent mass (*) and linear extension
(arrowheads) for a significant distance from the largest
component of the lesion.

2154 November-December 2009 radiographics.rsna.org
Imaging Features.—Radiographic findings
in patients with desmoid type fibromatosis are
often normal. A nonspecific soft-tissue mass
may be apparent in patients with larger lesions.
Calcification is uncommon, although underlying
osseous involvement may be seen in 6%–37%
of cases (3,14,23,35–37). Osseous involvement
typically manifests as extensive pressure ero-
sion or cortical scalloping but without extension
into the medullary canal (38). A skeletal dyspla-
sia has been reported in 19% of patients with
multicentric desmoid type fibromatosis (38).
Radiographic features include an erlenmeyer
flask deformity that is either polyostotic or only
on the affected side, cortical thickening, focal
lucent lesions, bone islands, and osseous excres-
cences (38). Bone scintigraphy usually demon-
strates increased radionuclide uptake on blood
flow and blood pool images and frequently more
limited activity on delayed images (Fig 8) (3,37).
Typically, these lesions are prominently hypervas-
cular, which is reflected by marked vascular stain-
ing at angiography (Fig 8) (39,40).
Figure 8. Desmoid type fibromatosis about the shoulder in a 23-year-old woman who presented with an enlarging
soft-tissue mass. (a) Images from three-phase bone scintigraphy reveal prominent increased radionuclide uptake (ar-
rowheads) during the blood flow (left) and blood pool (middle) phases but only mild activity in the delayed phase
(right), findings that reflect increased blood flow in the mass. (b) Angiogram shows dense tumor blush (arrowheads)
in this hypervascular mass. (c) Sagittal T2-weighted (3500/19) MR image demonstrates a relatively well-defined
soft-tissue mass (curved arrows) with numerous low-signal-intensity bands (straight arrows) and predominant interme-
diate signal intensity. (d) Photograph of the sectioned gross specimen shows the white collagenized bands (arrows) that
correspond to the MR imaging features. Scale is in centimeters.

RG ■ Volume 29 • Number 7 Murphey et al 2155
The US appearance of desmoid type fibroma-
tosis is nonspecific and has not been extensively
described. The disease usually manifests as a
poorly defined, hypoechoic soft-tissue mass, and
large lesions may demonstrate posterior acoustic
shadowing (Fig 5). Hypervascularity may be seen
at Doppler US examination (Fig 5).
CT of desmoid type fibromatosis reveals a
soft-tissue mass of variable attenuation. These le-
sions most frequently are similar in attenuation to
that of skeletal muscle (Fig 5). Lesions with more
prominent collagen content may reveal mildly
higher attenuation compared with that of muscle.
Low attenuation is the least common CT ap-
pearance, is usually associated with myxoid com-
ponents, and in our experience is more frequent
in chest wall lesions (Fig 6). Lesion margins are
often indistinct, a finding that reflects the infil-
tration seen histologically. CT performed after
administration of intravenous contrast material
typically demonstrates enhancement of variable
degree, although it may be prominent. This en-
hancement pattern likely reflects the abundant
capillary network frequently apparent pathologi-
cally in desmoid type fibromatosis.
The optimal radiologic modality for evaluat-
ing desmoid type fibromatosis, similar to other
soft-tissue masses, is MR imaging. These lesions
are usually centered in an intermuscular loca-
tion with a rim of fat (“split-fat” sign) (Fig 5),
although invasion of surrounding muscle is fre-
quent (Figs 6, 9). The lesions may be well defined
(49%–54% of cases) or have irregular infiltrative
margins (46%–51%) (Figs 5–8) (23,41). In our
experience, linear extension along fascial planes
(the fascial tail sign) is also a common manifesta-
tion of this lesion (83% of cases) (Figs 5, 7, 10)
(41). The fascial tail sign can be quite prominent,
extending a significant distance from the pri-
mary neoplastic focus, and is important to detect
as a diagnostic feature because it is an unusual
growth pattern for other lesions. More important,
adequate staging to guide complete resection re-
quires detection of this extension (Fig 7).
The signal intensity of desmoid type fibroma-
tosis is variable, likely dependent on the extent
of collagen and degree of cellularity of the lesion.
This variability in signal intensity was initially
reported by Sundaram and colleagues (42), who
observed lesions with low signal intensity on
T2-weighted MR images and lesions with high
signal intensity (Figs 5–7). In the two cases with
low signal intensity, the corresponding histologic
findings demonstrated hypocellularity and abun-
dant collagen; marked cellularity and abundant
collagen was seen in the lesion that had high
signal intensity on T2-weighted MR images (42).
Sundaram and co-workers (42) concluded that
the combination of low cellularity and high col-
lagen content produced the low signal intensity
Figure 9. Desmoid type fibromatosis in an 18-year-old man treated with radiation therapy. (a) Sagittal T2-
weighted (2,500/100) MR image obtained before radiation therapy shows a large soft-tissue mass (arrowheads) that
involves the infraspinatus and subscapularis muscles and that contains low-signal-intensity collagenized bands (ar-
rows). (b) Sagittal T2-weighted (2,500/120) MR image obtained after irradiation (2 months later) shows a decrease
in both size and signal intensity of the lesion (arrows), findings that indicate a good response to this form of treat-
ment. (c, d) Photomicrographs (original magnification, ×200; H-E stain) obtained before (c) and after (d) radiation
treatment show decreased cellularity and increased fibrosis (pink areas in d), an appearance that represents a good
response to the radiation therapy and correlates with the imaging findings.

2156 November-December 2009 radiographics.rsna.org
on T2-weighted MR images, with the cellularity
being the more important factor. We concur with
this hypothesis and also believe that the relative
extent of cellularity versus collagen is the cause
of low-signal-intensity regions on long repetition
time MR images. Similar to palmar and plantar
varieties of fibromatosis, desmoid type fibro-
matosis tumors have been described as having
three stages of histologic evolution over time as
they mature. In the first stage, lesions are more
cellular with large extracellular spaces (which
appear as decreased T1 and increased T2 MR
signal intensity) and have relatively decreased col-
lagen content (3,41,43,44). In the second stage,
there is an increase in the amount of collagen
deposition (which increases the heterogeneity of
T2 signal) in the central and peripheral areas of
the tumor (3,41,43,44). In the final stage, there
is an increase in the fibrous composition (which
decreases the T1 and T2 MR signal intensity),
with a decrease in cellularity and decrease in
the volume of the extracellular spaces and water
content. In addition, the higher signal intensity
on T2-weighted MR images may be related to
myxoid components. Larger studies of patients
have shown that the most common MR imaging
appearance of desmoid type fibromatosis is inter-
mediate signal intensity on T1-weighted images
(similar to that of muscle), seen in 83%–95% of
cases, and intermediate signal intensity on T2-
weighted images (lower than that of fat and high-
er than that of muscle with non–fat-suppressed
sequences), seen in 46%–77% of cases (Figs 5, 6,
8–10) (23,41,45–48). Predominant low sig- nal
intensity (similar to that of muscle), seen in
24%–31% of cases, or high signal intensity (simi-
lar to that of fat or fluid), seen in 30% of cases,
on non–fat-suppressed T2-weighted MR images
are less frequent findings (Figs 5–7) (23,41). Fat-
suppressed T2-weighted MR pulse sequences of-
ten reveal higher signal intensity within these le-
sions. Regions of low signal intensity are not spe-
cific for desmoid type fibromatosis, because other
neoplastic processes (such as pigmented villonod-
ular synovitis, granular cell tumor, fibrosarcoma,
and malignant fibrous histiocytomas) may reveal
similar MR imaging features. However, in our
opinion and experience, the bandlike morphology
of some areas of low signal intensity can add sig-
nificant specificity in diagnosis and are optimally
seen on T1-weighted postcontrast or T2-weighted
MR images (Figs 5–8). These low-signal-intensity
bands are common (62%–91% of cases) in des-
moid type fibromatosis, compared with other
neoplastic lesions, and are related to the collagen-
ized, hypocellular bands seen at gross pathologic
examination (23,41). In our experience, these
Figure 10. Desmoid type fibromatosis
in the calf of a 23-year-old woman that did
not respond to multiple treatment methods,
including surgical resection, cryogenic
ablation, chemotherapy with imatinib, and
radiation therapy. Amputation was per-
formed for definitive treatment. (a) Sagittal
T1-weighted (5430/10) MR image shows
a large recurrent soft-tissue mass (*) with
low-signal-intensity collagenized bands (ar-
rowheads) and fascial extension (arrow)
inferiorly (fascial tail sign). (b) Photograph
of the sagittally sectioned amputation speci-
men reveals the large recurrent tumor (*)
and linear extension inferiorly (arrow),
findings that correspond to the imaging
appearance.
Teaching
Point

RG ■ Volume 29 • Number 7 Murphey et al 2157
Figure 11. Desmoid type fibromatosis overlying the right trapezius muscle in a 33-year-old woman who
underwent cryoablation. (a) Axial postcontrast T1-weighted gradient-echo (5.4/2.2 with a 30-msec inver-
sion pulse and 120° flip angle) MR image obtained before cryoablation reveals a nodular, diffusely enhancing
soft-tissue mass (arrows) that involves the trapezius muscle. (b) Axial postcontrast T1-weighted fat-saturated
(400/15) MR image obtained after cryoablation demonstrates marked reduction in lesion size (arrows) as a
result of treatment. (Case courtesy of Patrick Liu, MD, Mayo Clinic, Scottsdale, Arizona.)
intrinsic features typically cause substantial het-
erogeneity in desmoid type fibromatosis on both
T1- and T2-weighted MR images. Postcontrast
MR images typically reveal heterogeneous and
often moderate to marked enhancement of these
lesions (Figs 6, 11). In fact, only 10% of these
lesions lack significant enhancement on MR im-
ages obtained after administration of contrast
material (49). Interestingly, the hypocellular, col-
lagenized bands do not enhance and therefore are
often accentuated at postcontrast MR imaging.
Treatment and Prognosis.—Historically, the
primary treatment of desmoid type fibromatosis
has been attempted resection with a wide margin,
although there are rare reports of spontaneous
regression. Unfortunately, complete resection is
often not achievable without substantial loss of
function in the involved limb, and the excision
required to achieve wide margins is frequently
greater than can be justified for management of
a benign disease process with no metastatic po-
tential. Soft-tissue closure or coverage following
resection of an extremity desmoid type fibroma-
tosis can generally be accomplished with primary
closure or local tissue advancement.
The local recurrence rate for desmoid type
fibromatosis has varied widely from 19% to 77%,
with an average of approximately 30%–40%,
typically within the first 12–18 months after
surgery (50–54). Parameters associated with an
increased risk of local recurrence include large
tumor size at presentation, patient age younger
than 30 years, female gender, and positive opera-
tive margins (1–5). Much of the surgery literature
focuses nearly exclusively on operative margins
and local recurrence rates, with little mention of
the morbidity and functional outcomes of wide
resection (55). This conclusion is reflected by a
preponderance of studies that show a local re-
currence rate of 20%–50% with wide resection,
as opposed to 90% with incomplete resection
(50–54). Although it is controversial, we also
believe that lesions of increased cellularity (and
relatively less collagen) that have higher signal
intensity on T2-weighted MR images and that
show more prominent enhancement may also be
at higher risk for local recurrence and more rapid
growth at follow-up imaging (Fig 7).
Nonetheless, wide surgical excision remains
an important treatment option and should be the
primary therapy for those select patients with fo-
cal disease in whom wide margins can ostensibly
be achieved with minimal functional, medical,
and cosmetic morbidity. Amputation may be re-
quired in rare instances for recurrent, progressive
lesions involving critical neurovascular bundles or
anatomic sites that are refractory to multimodal
adjuvant therapies (Fig 10).
Because of the routine difficulty in achieving
wide margins and the higher recurrence rates
following marginal excision, surgery augmented
with adjuvant radiation therapy or radiation
therapy alone has been advocated as an alterna-
tive treatment method (55). Local control rates
achieved with this approach have been reported

2158 November-December 2009 radiographics.rsna.org
Figure 12. Abdominal wall fibromatosis in a 30-year-old woman who presented with an enlarging soft-tissue mass
in her abdominal wall 6 months after delivery. (a) Axial CT image shows a soft-tissue mass (*) that involves the rec-
tus abdominus muscle. The mass contains a central area of lower attenuation and prominent areas of higher attenua-
tion (relative to adjacent muscle), owing to the high collagen content of the lesion. Linear extension along the fascia
(fascial tail sign) is seen medially (arrowheads). (b) Intraoperative photograph reveals the abdominal wall soft-tissue
mass (M) and linear extension (arrow), findings that correspond to the imaging appearance.
to be superior to those for excision alone, at 94%
versus 72% for patients with negative surgical
margins and 75% versus 41% for those with posi-
tive margins (Fig 7) (56). The results of treating
desmoid type fibromatosis with radiation ther-
apy alone have been similarly favorable, as this
method consistently produces local control rates
of approximately 80%, which are comparable to
surgical results (54,56–59). Radiation therapy
doses are most commonly 50–60 Gy, with a de-
monstrable dose-response relationship occurring
between 30 and 60 Gy (59). In our experience,
MR imaging can be useful for monitoring the
success of radiation therapy, which is evidenced
by progressive collagenization of these lesions
and appears as decreased signal intensity with
water sensitive pulse sequences and a reduction
or stabilization of tumor size (Fig 9). In contra-
distinction, failure of radiation therapy is por-
trayed as persistent increased signal intensity with
water sensitive sequences and progressive lesion
growth (Fig 10). Complications associated with
radiation therapy of these tumors, in decreasing
order of frequency, include fibrosis, paresthesias,
edema, pathologic fracture, cutaneous ulceration,
and secondary malignancy (56). Recently, radio-
frequency ablation of desmoid type fibromatosis,
particularly local recurrences, was shown to yield
a successful outcome, as reported by Lui and co-
workers (oral communication, Society of Skeletal
Radiology Meeting, March 2009) (Fig 11).
In the past 20 years, several series have demon-
strated the potential efficacy of chemotherapy for
treatment of desmoid type fibromatosis. Cytotoxic
doxorubicin-based regimens have been described
(60), and low-dose regimens of vinblastine and
methotrexate, which are well tolerated and have
few side effects, have also been advocated recently
(51,61,62). Although complete responses are
relatively rare, low-dose combination therapy has
demonstrated disease improvement or stabilization
rates of around 70% at 10 years follow-up (61).
Lastly, another chemotherapeutic agent imatinib
(Gleevec; Novartis, East Hanover, NJ) has demon-
strated potential efficacy in treating patients with
desmoid type fibromatosis (63).
Abdominal Wall Fibromatosis
Clinical Features.—Abdominal wall fibroma-
tosis or abdominal wall desmoid was initially
described in 1832 (1–5). Its relative frequency is
similar to that of desmoid type fibromatosis, and
in the study by Reitamo and co-workers (64),
its prevalence was slightly higher (49% vs 43%
of lesions, respectively). These lesions are distin-
guished from the other deep musculoaponeu-
rotic fibromatoses because of their location and
distinct predilection to develop in women of
childbearing age. In fact, 87% of these lesions oc-
cur in women, and 95% develop in women who
have had at least one child (the lesions usually
occur during the first year after childbirth) (Fig
12) (1–5). Abdominal wall desmoids may occur
in patients of any age, but their peak prevalence
is in patients in the 3rd decade of life (1–5).
Abdominal wall desmoids are solitary slow-
growing neoplasms that are recognized for their

RG ■ Volume 29 • Number 7 Murphey et al 2159
Figure 13. Abdominal wall desmoid in a 6-year-old girl with a history of posttraumatic C2 paraplegia who developed
a soft-tissue mass in her anterior abdominal wall adjacent to the insertion site of a percutaneous endoscopic gastros-
tomy (PEG) tube. The mass was originally assumed to be a hematoma related to the tube placement. (a) Doppler US
image shows a nonspecific soft-tissue mass with moderate internal flow. (b) Sagittal CT reconstructed image shows an
abdominal wall soft-tissue mass (straight arrow) adjacent to the PEG tube (curved arrow). (c, d) Axial T1-weighted (c,
621/8) and postcontrast T1-weighted (d, 621/8) MR images show a low- to intermediate-signal-intensity soft-tissue
mass (straight arrows) with diffuse enhancement, a finding that excludes the possibility of a hematoma. Nonenhancing
collagenized fibrous bands (arrowheads) are seen only on the postcontrast MR image (d). Curved arrow = PEG tube.
(e) Intraoperative clinical picture shows the abdominal wall soft-tissue mass (*) adjacent to the PEG tube insertion site
(arrowheads). Scale is in centimeters.
progressive, locally infiltrative, and aggressive
behavior (65). It is rare to find an abdominal wall
desmoid in a patient younger than 10 years of age
(66). Although these lesions are typically solitary,
cases with associated desmoid type fibromatosis
have been described.
The etiology of these tumors is uncertain.
Although the majority of cases are idiopathic,
estrogenic hormones, trauma (including sur-
gery), and genetic abnormalities have also been
implicated as potential causative factors (67).
These tumors are particularly more frequent
in women with a history of taking birth control
pills and in the third trimester of pregnancy
(Fig 12) (14). Abdominal wall desmoids tend to
regress after menopause or oophorectomy (67).
Studies have shown increased formation of these
tumors in guinea pigs after prolonged estrogen
administration and prevention of these tumors
by administering testosterone, progesterone,
and desoxycorticosterone (5,68). Antiestrogen
agents such as tamoxifen have also been shown
to have inhibitory effects. These findings support
the role of hormonal factors in the development
of this disease (5,69).
As with desmoid type fibromatosis, trauma
may serve as a contributory cause of abdominal
wall desmoids (1–5). These lesions arise following
a surgical procedure in 20% of cases, with 50%
of these occurring within the first 4 postoperative
years (67). Abdominal wall desmoids have a pre-
dilection to develop near areas of postoperative
scarring at prior incision sites, particularly those
from a cesarean section (Fig 13). These tumors
have also been reported to arise at the sites of
colostomies, laparoscopic trocar placements, and

2160 November-December 2009 radiographics.rsna.org
Figure 14. Affects of irradiation on abdominal wall fibromatosis involving the rectus abdominis muscle in a
65-year-old man. (a) Axial CT image of the pelvis shows a well-defined soft-tissue mass in the abdominal wall (ar-
rows) that displaced the bladder and has attenuation similar to that of adjacent muscle. (b, c) Coronal postcontrast
T1-weighted (b, 597/11) and coronal T2-weighted (c, 4810/78) MR images also reveal the abdominal wall soft-
tissue mass (arrowheads), which appears with predominant high signal intensity on the T2-weighted MR image (c)
and diffuse enhancement on the postcontrast T1-weighted image (b). Collagenized bands (arrows) are seen on T2-
weighted (c) and postcontrast T1-weighted (b) MR images as low-signal-intensity areas and nonenhancing regions,
respectively. (d, e) On sagittal postcontrast T1-weighted (d, 486/11) and T2-weighted (e, 5030/104) MR images
obtained after radiation therapy, the lesion contains a central area of necrosis (arrows) that does not enhance on the
postcontrast study (d) and that shows increased signal intensity on the long repetition time study (e). The low-signal-
intensity bands are less apparent than on the pretreatment images. (f) Photograph of the sectioned gross specimen,
which was resected after irradiation, shows the large area of central necrosis (arrows). Scale is in centimeters.

RG ■ Volume 29 • Number 7 Murphey et al 2161
catheter insertions for peritoneal dialysis (Fig 13)
(70–73). Such lesions have been referred to as
cicatricial fibromatosis. However, the majority of
patients with abdominal wall desmoids have no
history of gross injury. Minor undetected trauma
such as minute muscle tears may theoretically
serve as a contributing factor to development of
these lesions in a hormonally or genetically pre-
disposed individual (1–5).
Abdominal wall desmoids may also arise in pa-
tients with familial adenomatous polyposis (FAP)
(1–5), a characteristic that supports the genetic
etiology of some of these tumors. FAP is inherited
in an autosomal dominant fashion. Patients with
a phenotypic variant of FAP known as Gardner
syndrome may develop extraabdominal, abdomi-
nal wall, and intraabdominal desmoid tumors, in
addition to polyposis coli and colon carcinoma
(34,67,74,75). The prevalence of desmoid tumors
in FAP ranges from 3.6% to 34%. Patients with
FAP have an approximately 1000-fold increased
risk to develop desmoid tumors compared with
the general population (67,74). As opposed to
sporadic desmoid tumors, FAP-associated des-
moid tumors occur with equal distribution in men
and women, are multiple in 40% of cases, and
tend to be smaller in size (34,67,76,77).
Abdominal wall desmoids arise from the mus-
culoaponeurotic structures of the abdominal
wall, most frequently the rectus abdominis and
internal oblique muscles and their fascial cover-
ings (Figs 12, 14) (1–5). Abdominal wall desmoid
is the most common soft-tissue neoplasm of the
abdominal wall (1–5). Less often, these lesions
originate from the external oblique and the trans-
versalis muscles or fascia. Abdominal wall des-
moids occasionally cross the midline to involve
both rectus abdominis muscles (66). Rectus and
fascial desmoids may be associated with intraab-
dominal extension (66).
These desmoids most typically manifest as a
palpable, firm, soft-tissue mass. Abdominal wall
desmoids are deep-seated, soft-tissue masses that
grow slowly and cause little or no focal symptoms
initially and thus manifest late in the course of
their disease (66).
Pathologic Features.—The pathologic appear-
ance of abdominal wall desmoids, both at gross
and microscopic examination, is virtually identi-
cal to that of desmoid type fibromatosis in other
locations. Desmoid tumors are usually recognized
as solid, firm, whitish masses that often have an
infiltrative, spiculated margin to skeletal muscle
and the subcutis. These lesions, which have an
average size of 3–7 cm, are often smaller at detec-
tion than other desmoid type fibromatoses (1–5).
Estrogen receptors are common in abdominal
wall desmoids and were found in 79% of lesions
in the study by Lim and colleagues (78).
Imaging Features.—Similar to the pathologic
appearance, the radiologic features of abdominal
wall desmoid, with all imaging modalities, are
essentially identical to those of desmoid type fi-
bromatosis in other locations (Figs 12–14). These
lesions arise along and often involve the rectus
abdominis muscle (Figs 12, 14). The identifica-
tion of a predominantly low- to intermediate-
signal-intensity abdominal wall mass with linear
extensions (fascial tail sign) along the superficial
fascia at the margins and low-signal-intensity
bands that do not enhance following intravenous
contrast material administration is nearly pathog-
nomonic of an abdominal wall desmoid (Figs
12–14) (3,14). MR imaging is optimal for detect-
ing deep intraabdominal extension that (although
it is unusual) is important to guide complete sur-
gical resection (Fig 13).
Treatment and Prognosis.—The local man-
agement of extraabdominal desmoid type fibro-
matosis and abdominal wall desmoid tumors is
quite similar, with attempted wide-margin resec-
tion being the initial treatment of choice in the
majority of cases. These lesions also have a pro-
pensity to recur locally. The local recurrence rate
of abdominal wall lesions is 15%–30%, which
is lower than that of extraabdominal lesions
(17,79,80). Lesions most commonly recur within
the first 2 years after the initial excision or in con-
nection with subsequent pregnancies or deliveries
(53). Multiple recurrences have been reported
(3,5). There are also reports of abdominal wall le-
sions that regress with menopause.
Unfortunately, wide resection may not be
possible for abdominal wall desmoids without
compromising the integrity of the trunk wall, and
adjuvant radiation therapy may also be required
(Fig 14). Radiation therapy may cause central
necrosis of these lesions (Fig 14). Full-thickness
abdominal wall reconstruction may be necessary
via prosthetic fascial augmentation and local fas-
ciocutaneous advancement or regional flaps (80),
although remote free tissue transfer has been rec-
ommended to correct large defects (81). Lastly,
regimens of high-dose antiestrogen and proges-
terone agents (such as tamoxifen and raloxifene),
luteinizing-releasing hormone, and testosterone
have been reported in the successful management
of abdominal wall desmoids (82).
Teaching
Point

2162 November-December 2009 radiographics.rsna.org
subsequent development of muscle edema, de-
generation of fibers, and fibrosis (84). A genetic
component may also play a role, as 11% of pa-
tients have a positive family history (5).
Involvement of the sternocleidomastoid mus-
cle causes fusiform thickening and shortening
and can result in torticollis, which is reported in
14%–30% of cases (1–5,85). The tilting and rota-
tion of the head is toward the side of the lesion.
Torticollis may also develop later in life in these
patients because of the reduced growth of the
affected sternocleidomastoid muscle. Facial and
skull asymmetry (plagiocephaly) with decreased
Figure 15. Fibromatosis colli
in a 2-week-old girl with a new
left-sided neck mass and torti-
collis. (a) Transverse US image
demonstrates an enlarged left
lower sternocleidomastoid
muscle (between arrows). The
right sternocleidomastoid
muscle (between arrowheads)
is normal. A = airway, T = thy-
roid. (b) On an axial CT image,
the left sternocleidomastoid
muscle (arrows) is enlarged,
compared with the normal
right sternocleidomastoid mus-
cle (arrowhead). (c, d) Axial
T1-weighted (c, 700/15)
and coronal T2-weighted
(d, 1800/90) MR images also
show the enlarged left sterno-
cleidomastoid muscle (arrows)
and the normal right side (ar-
rowheads). The T2-weighted
MR image (d) reveals mildly
increased signal intensity in
the involved muscle (arrows).
(e) Photomicrograph (origi-
nal magnification, ×200; H-E
stain) reveals fibroblastic
proliferation, which infiltrates
muscle (arrows).
Childhood Fibromatoses
Fibromatosis Colli
Clinical Features.—Fibromatosis colli, also
known as sternocleidomastoid tumor of infancy,
pseudotumor of infancy, and congenital muscular
torticollis, is categorized as a benign fibroblastic
proliferation in the 2002 WHO classification of
soft-tissue tumors. Its etiology is not known, but
the process may represent a scarlike reaction to
injury of the sternocleidomastoid muscle in the
last trimester of intrauterine growth or during de-
livery (1–5). The lesion almost exclusively affects
the sternocleidomastoid muscle, although the tra-
pezius muscle is also involved in rare cases (Fig
15). Its prevalence is estimated at 0.4% of live
births (83). It is frequently (>60%–90% of cases),
but not exclusively, accompanied by a history of
birth trauma, difficult delivery (including use of
forceps), or breech delivery (84,85). One theory
contends that traumatic compression of the neck
during delivery could result in pressure necrosis
or occlusion of the venous outflow system with

RG ■ Volume 29 • Number 7 Murphey et al 2163
size on the involved side may also be seen (1–5).
Additional associated congenital abnormali-
ties include developmental dysplasia of the hip,
rib anomalies, talipes equinovarus (clubfoot),
thoracic scoliosis, metatarsus adductus, mental
retardation, and seizure disorders (1–5). The dis-
ease is almost invariably unilateral and has a mild
predilection for the right side (84,85). Bilateral
involvement is rare. Patients are typically unaf-
fected at birth, with an enlarging neck mass and
symptoms arising 10–14 days afterward (85). The
soft-tissue mass is often firm and rubbery and
may continue to enlarge for an additional 2–4
weeks with subsequent gradual resolution over
4–8 months (85).
Pathologic Features.—Although pathologic
specimens are rarely encountered today, at gross
examination fibromatosis colli is seen as a glisten-
ing gray-white to tan, gritty, soft-tissue mass that
averages 1–3 cm in size (1–5). The mass blends
imperceptibly with and infiltrates the surround-
ing sternocleidomastoid muscle. Typical changes
observed at histologic evaluation include alternat-
ing skeletal muscle fibers that have undergone
atrophy or degeneration and evolving scarlike
fibroblastic-myofibroblastic proliferation (Fig 15)
(1–5). This infiltration of the lesion with skeletal
muscle should not be mistaken for aggressive
growth. As with other types of fibromatosis, the
degree of cellularity is variable. Lesions early in
their development are more cellular, as opposed
to those of longer duration that contain increas-
ing amounts of collagen and reduced cellularity.
Imaging Features.—Radiography is often not
performed in this age group for an abnormality
at this site, but radiographic findings are typically
normal. Lytic lesions in the clavicular head at the
insertion of the sternocleidomastoid muscle have
been described (86). Associated skeletal abnormal-
ities include ipsilateral lateral mandibular asymme-
try, cervicothoracic scoliosis, facial deformity, ipsi-
lateral mastoid process hypertrophy, and ipsilateral
elevation of the clavicle or shoulder (1–5).
Cross-sectional imaging features of fibromato-
sis colli are diagnostic and should aid in avoiding
unnecessary biopsy. US, CT, and MR imag-
ing all show focal or diffuse enlargement of the
sternocleidomastoid muscle. US is the preferred
diagnostic tool because of its low cost and lack
of ionizing radiation. It typically demonstrates
fusiform thickening in the lower two-thirds of the
sternocleidomastoid muscle, although the echo-
genicity is variable and can range from hypoecho-
ic to hyperechoic (Fig 15) (14,84). Similarly, the
thickened muscle can be heterogeneous or homo-
geneous. Margins are usually well defined, and
a hypoechoic rim, which is thought to represent
normal peripheral muscle, may be seen (84). The
mass should move synchronously with the muscle
at real-time US (84,85).
CT shows isoattenuated enlargement of the
sternocleidomastoid muscle with normal sur-
rounding fascial planes (Fig 15) (84,85). CT
may also demonstrate associated mass effect and
mild deviation of adjacent structures, includ-
ing the trachea and carotid sheath, but there
should be no evidence of encasement or promi-
nent displacement (84,85). MR imaging reveals
similar features, including fusiform thickening,
well-defined margins, and mild mass effect on
adjacent structures (Fig 15). Variation in signal
intensity characteristics may be observed. In one
study, on T2-weighted MR images, the enlarged
muscle demonstrated diffuse abnormal high
signal intensity greater than that of fat, and no
focal discrete mass was seen (Fig 15) (3,84). MR
imaging also helps demonstrate lack of involve-
ment of surrounding structures and absence of
lymphadenopathy, airway compression, vascular
encasement, bone involvement, or other features
that would suggest a more aggressive process.
Treatment and Prognosis.—Treatment of fibro-
matosis colli is conservative, consisting of clinical
observation and serial stretching exercises, after
the diagnosis is established on a clinical, radio-
logic, or less frequently histologic basis (87,88). In
more than 70%–90% of cases, lesions spontane-
ously regress, and secondary contractures resolve
within 4–8 months (87,88). Surgical intervention
is required in 10%–15% of cases and is indicated
in patients with severe refractory disease after 1
year of age to prevent permanent contracture and
plagiocephaly (1–5,87,88). Surgical treatment
consists of proximal or distal release (tenotomy) of
the sternocleidomastoid muscle. Excision of the fi-
brous lesion is rarely, if ever, indicated or required.
More recently, use of Botulinum toxin type A has
shown promise for treatment of refractory cases
and may further prevent the need for surgical in-
tervention (89,90).
Lipofibromatosis
Clinical Features.—Lipofibromatosis has been
recently described as an entity distinct from
other forms of fibromatosis and fatty tumors
in children and was designated as such by the
WHO Committee for Classification of Soft Tissue
Tumors in 2002 (1–5,91,92). This lesion has also
been referred to as infantile fibromatosis of the

2164 November-December 2009 radiographics.rsna.org
Figure 16. Lipofibromatosis in a 6-month-old girl with an enlarging soft-tissue mass that diffusely involves the foot.
(a) Long-axis T1-weighted (400/15) MR image shows a soft-tissue mass (*) with predominantly lipomatous tissue in-
filtrating the foot with some interspersed low-signal-intensity fibrous strands. (b) Sagittal T2-weighted fat-suppressed
(3000/70) MR image reveals diffuse enlargement of the foot caused by the infiltrative soft-tissue mass with its large
adipose component, which has low signal intensity (arrows). (c) Photomicrograph (original magnification, ×175; H-E
stain) of lipofibromatosis shows the white lobular fat cells (arrowheads) with intermixed cellular fibrous tissue (*).
nondesmoid type and represents a rare fibrofatty
tumor of childhood. Lipofibromatosis occurs in a
wide anatomic distribution, although it has a pre-
dilection to affect the extremities, more specifical-
ly, the hands and feet (Fig 16). Other less common
locations of these lesions include the head or neck,
chest wall, abdominal wall, and jaw (92). Patient
age at the time of initial manifestation ranges from
11 days to 12 years, with a median age of 1 year
(92). Fetsch and co-workers (92) at the AFIP
noted that 18% of these lesions were present at
birth. There is a male predominance of approxi-
mately 2:1 (92). The lesions are generally described
as slow-growing painless masses, although rapid
growth has also been reported in rare cases. This
tumor appears as a poorly demarcated, soft-tissue
mass arising in the subcutaneous tissues or in the
deep soft tissues (92).
Pathologic Features.—At gross pathologic ex-
amination, lipofibromatosis usually appears as a
yellow fatty mass because of its dominant adipose
component, but interspersed gray-white streaks
of fibrous tissue are also present. The lesions vary
in size from a small 1–3-cm nodule to a large
10-cm mass (median size, 2 cm) that can involve
much of the distal extremity (92).
These lesions are composed of abundant
adipose tissue traversed by bundles of spindle-
shaped fibroblast-like cells (Fig 16) (92). The
strands of fibromatosis-like component within the
adipose tissue are moderately cellular, but mitotic
activity is inconspicuous.
Imaging Features.—Only limited information
(three separate case reports) has been published
on the imaging appearances of lipofibroma-
tosis (73,91,93). Radiography has a limited
role in evaluation of these soft-tissue tumors.
Radiographs may show a low-density mass if
substantial fat is present (91). In one of the case
reports, the lesions eroded the adjacent osseous
structures due to pressure effect (43,91).
The MR imaging appearance is suggestive of
but not entirely specific for the diagnosis (91). In
our experience, which is similar to that of Deepti
and colleagues (91), the MR imaging features of
lipofibromatosis correspond to its pathologic ap-
pearance. These lesions are largely composed of
lipomatous tissue with interspersed muscle and
fibrous bands; the former is isointense relative to
fat and the latter have low to intermediate signal
intensity with all pulse sequences (Fig 16) (91).
The fibrous components may enhance following
intravenous contrast administration. The lesions
are often infiltrative, involving subcutaneous and
the deeper soft tissues, and MR imaging is the
modality of choice for evaluating the extent of
soft-tissue and bone involvement.
Treatment and Prognosis.—Complete surgical
excision is the preferred treatment for lipofibro-
matosis. However, if the end result would entail
increased morbidity or significant functional
compromise, management should be individual-
ized because there are some cases with long-term
follow-up that have not progressed, even when
the lesions were incompletely excised (92). The

RG ■ Volume 29 • Number 7 Murphey et al 2165
Figure 17. Calcifying aponeurotic fibroma in a 1-year-old child with a slowly enlarging soft-tissue
mass in the foot. (a) Lateral radiograph of the foot shows a mass along the plantar aspect of the foot
with subtle associated calcification (arrows). (b) Short-axis T2-weighted (2900/85) MR image reveals a
lobular soft-tissue mass (arrows) with intermediate to high signal intensity along the plantar aponeurosis.
(c) Photograph of the gross specimen demonstrates the soft-tissue mass, which contains subtle white
areas (arrowheads) that represent calcifications. Scale is in centimeters. (d) Photomicrograph (original
magnification, ×200; H-E stain) shows the coarse calcifications (arrows) and surrounding areas of fibrous
tissue containing spindle-shaped cells (*).
high rate of local recurrence (72%) is likely re-
lated to the poorly defined, infiltrative margins of
these lesions, a characteristic that results in in-
complete resection (91). Additional features that
have been implicated as risk factors for local re-
currence include congenital onset, male sex, hand
or foot location, and increased mitotic activity in
the fibroblastic components of the lesion. In cases
with extensive involvement of the limb, debulk-
ing procedures have been performed. Amputation
may be required if the limb is nonviable or non-
functional after a debulking procedure (43). This
lesion has no metastatic potential.
Calcifying Aponeurotic Fibroma
Clinical Features.—Calcifying aponeurotic
fibroma is a rare, locally aggressive fibroblastic le-
sion that most commonly affects the palms of the
hand and soles of the feet in young children. It is
one of the few fibrous lesions that can calcify. It
was originally described and referred to as juvenile
aponeurotic fibroma by Keasbey (94) in 1953 and
represented only 0.4% of all benign soft-tissue
tumors in the large soft-tissue tumor registry over
a 10-year period from the AFIP (3). These lesions
typically occur in patients during the first 2 de-
cades of life (peak incidence, age 8–14 years), al-
though more recently a wider age distribution has
been described (1–5). There is a 2:1 male predilec-
tion (1–5). The lesions most commonly involve
the deep volar fascia, tendons, and aponeuroses
of the hands (67%–75% of cases), followed by
the feet (Fig 17) (1–5,94). Additional, less fre-
quently involved sites include the neck, thigh, fore-
arm, popliteal fossa, and lumbosacral region. The

2166 November-December 2009 radiographics.rsna.org
Figure 18. Inclusion body fibromatosis in a 16-month-old girl with lateral growth of her
left second toe and superficial ulceration. (a) Anteroposterior radiograph of the foot shows
a soft-tissue mass (arrows) in the distal lateral second toe. (b) Photomicrograph (original
magnification, ×300; H-E stain) demonstrates a largely collagenized (pinkish tissue) lesion
with fibroblastic cellularity and multiple small round eosinophilic inclusions (arrowheads).
typical clinical manifestation is that of a slow-
growing, poorly circumscribed, asymptomatic soft-
tissue mass that does not limit joint motion.
Pathologic Features.—This rare fibroblastic
proliferation of childhood is currently considered
more related to fibromas than a true fibromatosis,
although it has somewhat similar potential for
repeated local recurrences. At gross pathologic
examination, calcifying aponeurotic fibroma has
irregular, ill-defined contours and is usually a
relatively small (<3 cm in size), rubbery to firm,
gray-white mass. Lesions typically contain two
components: (a) fibromatosis-like spindle-shaped
cell elements and (b) distinctive, round or oval,
fibrocartilaginous, often calcified foci composed
of epithelioid cells (Fig 17). Calcified foci are
often more prominent centrally and are seen in
older children and young adults.
Imaging Features.—There are only limited
reports in the radiology literature describ-
ing the imaging appearances of these lesions.
Radiography may show a nonspecific soft-tissue
mass with a variable extent of fine, stippled
calcifications (Fig 17). Extrinsic erosion of the
adjacent bone is rarely seen (1–5). CT is optimal
for depicting the calcified areas of the lesion,
with other regions demonstrating nonspecific
soft-tissue attenuation. In our experience, the
MR imaging findings are also nonspecific. The
lesion appears as a heterogeneous, round to oval
superficial soft-tissue mass, features that are simi-
lar to those of other superficial fibromatoses (Fig
17) (95). Prominent areas of globular low signal
intensity that correspond to areas of calcification
may be seen with all MR pulse sequences.
Treatment and Prognosis.—Biopsy is impor-
tant in the evaluation of these lesions to allow
differentiation from synovial sarcoma, which can
have similar imaging features and which may affect
the hands and feet of younger patients. Resection
is typically the initial treatment of choice, and
function-preserving surgery is advocated because
of the low biologic potential of this tumor. Local
recurrence is common (52% of all cases) and de-
velops more frequently in patients younger than 5
years of age, typically within the first 3 postopera-
tive years (1–5). Reexcision of local recurrence is
usually performed, rather than more aggressive
measures, to preserve function (1–5). There are
exceedingly rare cases of purported malignant
transformation and subsequent metastases.

RG ■ Volume 29 • Number 7 Murphey et al 2167
Inclusion Body Fibromatosis
Clinical Features.—Inclusion body fibromatosis
was initially described by Jensen and co-workers
(96) in 1957 and referred to as digital neurofibro-
sarcoma. Additional designations for this lesion
have included infantile digital fibromatosis, infan-
tile dermal fibromatosis, infantile digital fibroma,
infantile dermal fibroma, infantile digital myofi-
bromatosis, Reye tumor, and digital fibrous tu-
mor of infancy and childhood (1–5). As many of
these names suggest, inclusion body fibromatosis
occurs in the fingers and toes of infants (Fig 18).
The majority of cases (80%) are diagnosed in the
first year of life, and approximately 33% of cases
are congenital (1–5,97). No familial tendency has
been reported. There is a slight predilection for
these lesions to occur in girls. The fingers (60%
of cases) are more frequently involved than the
toes (40%), a distribution that is similar to that of
calcifying aponeurotic fibroma (97). Extradigital
lesions occur in the breast and arm in rare cases.
Inclusion body fibromatosis usually manifests
clinically as single or multiple (with an equal
distribution) dome-shaped nodules that involve
the extensor surfaces, most commonly the dorsal
and lateral aspect of the distal or middle phalanx
(particularly the third, fourth and fifth fingers)
(Fig 18). Involvement of the thumb is rare, and,
to the best of our knowledge, involvement of the
great toe has not been reported. Lesions are typi-
cally nontender, and the overlying skin may be
pale red (Fig 18); accompanying ulceration is rare.
Associated joint deformity (angular and contrac-
ture) may be seen and persist after treatment.
Pathologic Features.—At gross pathologic ex-
amination, inclusion body fibromatosis is usually
1–2 cm in diameter and appears tan to white (Fig
18). Lesions are typically poorly circumscribed
and extend from the epidermis to involve the
dermis and subcutaneous tissue. The tumor is
composed of fibroblasts and myofibroblasts that
entrap skin adnexal structures. A unique fea-
ture to this tumor is occurrence of cytoplasmic
eosinophilic inclusions in the myofibroblasts
that histologically resemble erythrocytes (97).
However, these inclusions are believed to con-
tain cytoskeletal filaments, particularly actins.
Immunohistochemical staining of this lesion is
positive for desmin, whereas nuclear β-catenin
does not occur in this tumor (97).
Imaging Features.—Radiographs of inclusion
body fibromatosis show a nonspecific soft-tissue
mass (Fig 18). Underlying bone involvement
with either extrinsic erosion or invasion is seen
in rare cases (3). Reports in the radiology litera-
ture describing the cross-sectional imaging ap-
pearance of these lesions are limited. In our ex-
perience, US, CT, and MR imaging findings of
inclusion body fibromatosis—a heterogeneous,
round to oval, subcutaneous soft-tissue mass—
are nonspecific and similar to those of other su-
perficial fibromatoses (98). Lesion margins are
typically poorly defined.
Treatment and Prognosis.—Spontaneous re-
gression and complete resolution of inclusion body
fibromatosis occur in at least 8% of cases (1–5).
Because of this possibility, low inherent biologic
potential, and lack of metastatic potential, con-
servative treatment is often advocated. However,
in many cases, surgical excision is performed with
marginal or wide excision. Similar to the other su-
perficial fibromatoses, inclusion body fibromatosis
often locally recurs (50%–60% of cases), particu-
larly when lesions are incompletely resected (1–5).
Myobroma and Myobromatosis
Clinical Features.—Myofibromatosis was
initially described by Williams and Schrum
(99) in 1951 and was referred to as congenital
fibrosarcoma. The term congenital generalized
bromatosis was coined by Stout (100) in 1954.
In a review of 61 cases at the AFIP from 1955
through 1979, Chung and Enzinger (101) sug-
gested that the term infantile bromatosis better
characterized the condition, since it occurred in
both newborns and infants and because of the
cellular myofibroblastic features.
The WHO, in its 2002 classification of soft-
tissue tumors, recognized myofibromatosis under
the benign category of fibroblastic-myofibroblastic
lesions (1). The terms myobroma and myobroma-
tosis are used to represent the solitary and multi-
centric forms of the disease process, respectively.
Myofibromatosis is the most common fibroblastic-
myofibroblastic soft-tissue tumor of childhood and
adolescents (1) and accounted for 22% of lesions
in the series by Coffin and Dehner (102).

2168 November-December 2009 radiographics.rsna.org
In the review by Chung and Enzinger (101),
the solitary form predominated (approximate
ratio, 3:1), but, in a review of 170 cases in the
literature, Wiswell and colleagues (103) reported
a slight predominance of multicentric disease
(90 vs 80 cases). The overall prevalence of mul-
ticentric to solitary lesions has been variably
reported in the literature, ranging from 2:1 to
1:4 (101,103). The solitary form (myofibroma)
most frequently affects the head and neck region,
which accounts for approximately 50% of lesions;
these typically appear in the scalp, forehead, or-
bit, oral cavity, and parotid region (Fig 19) (1–5).
The trunk is the next most commonly involved
site, followed by the extremities. Myofibromas
usually develop in skin, muscle, and subcutane-
ous tissue (86% of cases) (Fig 19), but they also
occur as solitary bone lesions (9%) (101,103).
Figure 19. Myofibroma in-
volving the upper neck and oc-
cipital region in a 7-day-old girl
with a large protuberant scalp
mass. (a) Clinical photograph
shows the large protuberant
scalp mass (arrows); its reddish
color suggests a possible vascu-
lar lesion. (b) Axial CT image
reveals the soft-tissue mass (*),
which has nonspecific features
and attenuation similar to that of
muscle. (c) Sagittal T2-weighted
(4500/99) MR image also dem-
onstrates the heterogeneous
nonspecific soft-tissue mass (*).

RG ■ Volume 29 • Number 7 Murphey et al 2169
Myofibromatosis, the multicentric form of this
disease, is typically found in skin, muscle, and
subcutaneous tissue (98% of cases) and bone
(57%), but it can also involve viscera (Figs 20,
21) (101,103). In 90 multicentric cases reviewed
by Wiswell and co-workers (103), 37% demon-
strated visceral involvement (most commonly, the
lung, heart, gastrointestinal tract, and pancreas).
There is a male predominance (1.6–1.7:1) in
both forms of the disease (103).
Figure 20. Myofibromatosis in a
2-day-old full-term boy with a con-
genital soft-tissue mass protruding
from his left shoulder. (a) Clinical
photograph reveals a large purple
protuberant soft-tissue mass over-
lying the left shoulder (arrow); its
appearance resembles a vascular
lesion. (b) Coronal CT recon-
structed image shows that the large
infiltrating lesion (arrows) extends
into the mediastinum and upper
abdomen. (c) Coronal T2-weighted
(5400/23) MR image also demon-
strates the extension (arrows) and
intermediate signal intensity of the
lesion. (d) Sagittal postcontrast
T1-weighted (733/14) MR image
shows that the mass (arrows) also
extends into the spinal canal (ar-
rowheads) and causes mass effect on
the spinal cord. There is mild diffuse
enhancement of the lesion. The le-
sion largely resolved after 6 weeks of
chemotherapy.

2170 November-December 2009 radiographics.rsna.org
Figure 21. Myofibromatosis with bone involvement in a 7-month-old boy with gluteal
swelling and a soft-tissue mass lateral to the left hip. (a) Radiograph of the pelvis shows bilat-
eral proximal femoral metaphyseal lytic lesions with sclerotic margins (arrows). There is a soft-
tissue mass lateral to the left hip with a small focus of calcification (arrowhead). (b) US image
reveals the hypoechoic solid soft-tissue mass lateral to the left hip (arrows); it also shows the
calcification, which appears as a small hyperechoic focus (arrowhead) with posterior acoustic
shadowing. (c) Radiograph of the left lower extremity shows multiple metaphyseal lytic
lesions (arrows) that are predominantly eccentric in the medial and lateral distal femur, tib-
ia, and fibula. Similar lesions were seen on radiographs of the upper extremities (not shown).
(d) Photomicrograph (original magnification, ×175; H-E stain) shows cellular myofibroblastic
regions (M) and vascular hemangiopericytoma areas (arrowheads), which are typical of this
lesion. Results from excisional biopsy of the soft-tissue mass confirmed the diagnosis; bone le-
sions spontaneously resolved and no further treatment was required.

RG ■ Volume 29 • Number 7 Murphey et al 2171
Although myofibromatosis is considered rare,
some believe that the disease could be under-
reported, because lesions may not be clinically
discernible and most spontaneously resolve
(101). Myofibromatosis affects infants and young
children almost exclusively, with 88% of cases
reported by Chung and Enzinger occurring in
patients less than 2 years of age (Figs 19–21)
(101). Only 11% of cases reviewed by Wiswell
and colleagues (103) were in children older than
12 months of age, and cases rarely have been de-
scribed in adults (101).
Patients usually present with palpable, rubbery,
firm to hard nodules or masses in the skin, muscle,
or subcutaneous tissue that generally are nonten-
der and painless (101,103). Skin lesions can appear
as a purplish macule, mimicking a hemangioma
(101) (Figs 19, 20). Subcutaneous lesions are
freely mobile, whereas more deeply seated lesions
may be fixed. The number of lesions in myofibro-
matosis can vary from 2 to 100 (103). If a patient
with multicentric disease has a single tumor that
predominates in size among other scattered small-
er tumors, this entity can occasionally be mis-
taken for a primary malignancy with metastases
(101,103). Visceral lesions may cause symptoms
referable to the organ system affected. Rare fa-
milial occurrences of these lesions have suggested
both a potential autosomal dominant and recessive
pattern of inheritance, although the prevalence is
too low to allow firm conclusions (1–5).
Pathologic Features.—At gross pathologic
examination, the nodules of myofibroma and
myofibromatosis vary from 0.5 to 7 cm in size
(usually less than several centimeters) (1–5). On
cut section, these lesions appear grayish-white to
light tan-brown and have a firm scarlike fibrous
consistency. Central yellow areas are common
and represent necrotic regions or cystic spaces
filled with a caseous-like material or hemorrhage.
Superficial lesions are sharply demarcated but
unencapsulated, whereas deep-seated lesions are
often more poorly defined.
Myofibroma has two distinctive microscopic
components: (a) a central, highly vascular and
cellular, hemangiopericytoma-like component,
and (b) coalescent clusters and bundles of
spindle-shaped myofibroblasts (Fig 21). The
hemangiopericytoma-like component may pre-
dominate histologically, a characteristic that has
led to the suggestion that most cases initially
diagnosed as infantile hemangiopericytoma may
represent myofibromas (Figs 19, 20) (1–5). The
prominent vascular component may also be sug-
gestive of a diagnosis of hemangioma in superfi-
cial lesions. In many cases, the central immature
part of the lesion has extensive, typically sharply
demarcated areas of coagulative necrosis, some-
times containing calcification, hyalinization, and
cystic change. Mitotic activity is usually minimal
but may be prominent in a minority of cases.
The differentiated myofibroblastic component
is immunohistochemically positive for α-smooth
muscle actin but not desmin (1–5).
Imaging Features.—At radiography, involve-
ment of bones by myofibromatosis typically
manifests as well-circumscribed, multifocal,
geographic lytic lesions, with or without sclerotic
borders (Fig 21) (14,104,105). Sclerosis may
develop within the osseous lesions and may rep-
resent the initiation of healing. These lesions are
often eccentric and metaphyseal in location and
are frequently bilateral and symmetric (Fig 21).
The bone lesions may not be present at birth
but develop in early infancy. The skull, femur,
tibia, spine, and rib are most commonly affected
(101,105). Periosteal reaction, although unusual,
has been reported in these lesions and may be
extensive. Pathologic fractures have also been de-
scribed. Bone scintigraphy may demonstrate nor-
mal findings or may reveal increased radiotracer
uptake that corresponds to the bone involvement.
Extraskeletal lesions may be seen on radiographs
as a soft-tissue mass, often with associated foci of
calcification (Fig 21) (101).
At US, soft-tissue lesions often have the non-
specific appearance of a hypoechoic to isoechoic
mass (Fig 21). Koujok and co-workers (105)
reported that a majority of tumors demonstrated
an anechoic or partially anechoic center and
thick peripheral walls or septa. Echogenic foci
with posterior acoustic shadowing, findings that
represent calcification, may also be seen (Fig 21).
Doppler US features have varied widely (105).
At CT, soft-tissue lesions of myofibroma or
myofibromatosis often appear with attenuation
that is similar to or mildly higher than that of skel-
etal muscle (Figs 19, 20). A central area of low
attenuation (decreased relative to adjacent muscle)
and peripheral enhancement may also be seen.
Central calcification may be identified at CT, and
bone involvement can also be assessed (14,105).

2172 November-December 2009 radiographics.rsna.org
MR imaging features of this disease also
vary, with soft-tissue lesions most often showing
low to intermediate signal intensity with T1-
weighted pulse sequences and central high sig-
nal intensity with T2-weighted pulse sequences
(Figs 19, 20) (14). Foci of low signal intensity
seen with all MR pulse sequences may represent
calcification. Lesions typically reveal peripheral
enhancement following intravenous contrast
material administration. The nonenhancing cen-
tral portion correlates with pathologic findings
of central necrosis (105).
Radiologic workup of a patient with suspected
myofibromatosis involvement of the viscera in-
cludes chest radiography, CT, skeletal survey,
and MR imaging (105). Pulmonary involve-
ment may manifest with an interstitial pattern,
reticulonodular infiltrates, or an appearance of
generalized bronchopneumonia. Gastrointestinal
involvement may appear as diffuse narrowing of
the bowel or multiple filling defects (observed at
barium study) (3,14).
Treatment and Prognosis.—The prognosis is
generally excellent for patients who have solitary
or multicentric disease confined to soft tissue
and bone without visceral involvement; in such
cases, myofibromatosis is frequently self-limiting.
However, it is important to note that frequently
there is a transient increase in size and number
of tumors before the disease spontaneously re-
gresses. Bone lesions are particularly innocuous
and need no further treatment after the diagnosis
is confirmed, usually after biopsy of a soft-tissue
focus (Fig 21). Overall spontaneous regression is
reported in 30%–61% of these lesions (1–5).
Treatment of solitary or multicentric disease
without visceral involvement therefore primarily
consists of observation. In patients with signifi-
cant symptoms related to a soft-tissue lesion or to
confirm the diagnosis, local resection or excisional
biopsy may be performed. Recurrence of soft-
tissue lesions following resection is uncommon
(particularly in comparison with other forms of
fibromatosis) and was seen in only 7% of 43 cases
in the review by Chung and Enzinger (101) and all
were cured with repeated excision. Similarly, in the
review by Wiswell and co-workers (103), five of 54
patients (9%) who were monitored after excision
of a solitary tumor had a single recurrence. In the
setting of multicentric disease without visceral
involvement, virtually all tumor nodules under-
went spontaneous remission in 56% of the cases
(five of nine) in the study by Chung and Enzinger
(101) and 61% of the cases (11/18) reviewed by
Wiswell and colleagues (103).
Unfortunately, patients with multicentric dis-
ease with visceral involvement typically have a
poor prognosis, and the disease process frequently
results in death. Only rare instances of sponta-
neous regression have been reported in these
patients. In the reviews by Chung and Enzinger
(101) and Wiswell and co-workers (103), 75% and
73% of patients with visceral involvement, respec-
tively, died of the disease, compared with an over-
all mortality rate of less than 15%–20% in patients
with multicentric musculoskeletal fibromatosis
(3,14). However, if visceral involvement is limited
to only one lesion, is small in extent, and is not
pulmonary, the patient may still have an excellent
prognosis (101). Pulmonary involvement contin-
ues to be associated with a poor prognosis.
Because of the high mortality associated with
multicentric myofibromatosis with visceral involve-
ment, chemotherapy has been used in an attempt
to improve patient outcome. Recently, case reports
have described a good response to a combination
of vinblastine and methotrexate, which is also used
in treatment of desmoid tumors in other locations
and retroperitoneal fibromatosis (106,107).
Summary
The musculoskeletal fibromatoses represent a
diverse group of fibroblastic proliferations that
demonstrate a similar histologic appearance. The
biologic behavior of these tumors is intermedi-
ate between that of benign and malignant fibrous
lesions, as these tumors commonly demonstrate
infiltrative growth that leads to frequent local
recurrence but lack metastatic potential. The
WHO has classified these lesions based on their
anatomic location as superficial or deep. The
superficial lesions include palmar and plantar
fibromatosis. The deep lesions affecting the mus-
culoskeletal system include desmoid type and
abdominal wall fibromatosis. Several additional
types of fibromatosis predominantly affect the
musculoskeletal system during childhood. These
childhood musculoskeletal fibromatoses include
fibromatosis colli, lipofibromatosis, calcifying
aponeurotic fibroma, inclusion body fibromatosis,
and myofibroma and myofibromatosis.
The diagnosis of superficial fibromatosis is
typically suggested clinically by the presence of
a superficial soft-tissue mass and overlying skin
deformity. Palmar lesions frequently cause associ-

RG ■ Volume 29 • Number 7 Murphey et al 2173
ated flexion contracture of the ulnar sided digits
(Dupuytren contracture). Findings from cross-
sectional imaging, particularly MR imaging, of
the superficial fibromatoses are typically sugges-
tive of the diagnosis with lesion location related
to the flexor tendons or plantar aponeurosis.
Associated prominent low signal intensity on T2-
weighted MR images is also frequently seen. Low
signal intensity on T1-weighted MR images may
be useful to guide the optimal timing of surgery
to reduce local recurrence.
The deep fibromatoses, particularly in an ex-
traabdominal location, may manifest clinically
with a nonspecific soft-tissue mass. For these le-
sions, findings from cross-sectional imaging, par-
ticularly MR imaging, are frequently suggestive
of the diagnosis. The deep fibromatoses are often
heterogeneous with prominent low signal intensity
on long repetition time MR images. The detection
of extension along the fascia (fascial tail sign) and
low-signal-intensity, nonenhancing, collagenized
bands adds specificity for diagnosis at MR imag-
ing. Lesions involving the abdominal wall are often
related to pregnancy and have similar intrinsic
appearances on MR images compared with those
found in extraabdominal sites. This diagnosis is
also suggested by the tumor location.
The childhood fibromatoses represent a broad
spectrum of lesions, and the diagnosis is often
suggested by the young patient age and tumor
location. Lesion location includes the sterno-
cleidomastoid muscle in the neck (fibromatosis
colli), hands and feet (lipofibromatosis, calcifying
aponeurosis fibroma, and inclusion body fibro-
matosis), or multiple sites (myofibromatosis). The
intrinsic appearance of the childhood fibroma-
toses is often nonspecific, although identification
of calcification (calcifying aponeurotic fibroma)
or an adipose component (lipofibromatosis) aids
in diagnostic specificity.
The treatment of the majority of musculo-
skeletal fibromatoses may be either surgical
resection (superficial and deep fibromatosis and
lipofibromatosis) or conservative in lesions that
typically undergo spontaneous regression (fibro-
matosis colli, calcifying aponeurotic fibroma, in-
clusion body fibromatosis, and myofibromatosis
without visceral involvement). Local recurrence
is common after surgical resection owing to the
infiltrative margins of the lesions. MR imaging is
the optimal technique to stage lesion extent, de-
termine involvement of surrounding structures,
and detect local recurrence. Understanding and
recognizing the spectrum of radiologic appear-
ances and their pathologic basis allow improved
patient assessment and are important to opti-
mize clinical management.
Acknowledgments: The authors gratefully acknowl-
edge the support of Janice Danqing Liu for manuscript
preparation and Mark J. Kransdorf, MD, and the
residents who attend the AFIP radiologic pathology
courses (past, present, and future) for their contribu-
tion to our series of patients and without whom this
project would not have been possible.
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This article meets the criteria for 1.0 credit hour in category 1 of the AMA Physician’s Recognition Award. To obtain
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RG Volume 29 Number November-December 2009 Murphey et al
Musculoskeletal Fibromatoses: Radiologic-Pathologic
Correlation
Mark D. Murphey, MD, et al
Page 2144
These nodules may progress slowly (over months to years) to fibrous cords or bands that attach to
and cause traction on the underlying flexor tendons, resulting in flexion contractures of the digits
(Dupuytren contractures).
Page 2146
At MR imaging, palmar fibromatosis appears as multiple nodular (including knuckle pad
fibromatosis) or cordlike, superficial soft-tissue masses that arise from the proximal palmar
aponeurosis and extend superficially in parallel with the flexor tendons.
Page 2149
The typical MR imaging appearance of plantar fibromatosis is a well- or ill-defined superficial soft-
tissue mass occurring along the deep plantar aponeurosis.
Page 2156
These low-signal-intensity bands are common (62%--91% of cases) in desmoid type fibromatosis,
compared with other neoplastic lesions, and are related to the collagenized, hypocellular bands seen at
gross pathologic examination.
Page 2161
Abdominal wall desmoids arise from the musculoaponeurotic structures of the abdominal wall, most
frequently the rectus abdominis and internal oblique muscles and their fascial coverings.
7
RadioGraphics 2009; 29:2143–2176 • Published online 10.1148/rg.297095138 • Content Code: