Phyllodes Tumor: Review Article

Abstract

Introduction. Phyllodes tumours are rare fibroepithelial lesions. Accurate preoperative pathological diagnosis allows correct surgical planning and avoidance of reoperation. Treatment can be either wide local excision or mastectomy to achieve histologically clear margins. Discussion. The exact aetiology of phyllodes tumour and its relationship with fibroadenoma are unclear. Women aged between 35 and 55 years are commonly involved. The median tumour size is 4 cm but can grow even larger having dilated veins and a blue discoloration over skin. Palpable axillary lymphadenopathy can be identified in up to 10-15% of patients but <1% had pathological positive nodes. Mammography and ultrasonography are main imaging modalities. Cytologically the presence of both epithelial and stromal elements supports the diagnosis. The value of FNAC in diagnosis of phyllodes tumour remains controversial, but core needle biopsy has high sensitivity and negative predictive value. Surgical management is the mainstay and local recurrence in phyllodes tumours has been associated with inadequate local excision. The role of adjuvant radiotherapy and chemotherapy remains uncertain and use of hormonal therapy has not been fully investigated. Conclusion. The preoperative diagnosis and proper management are crucial in phyllodes tumours because of their tendency to recur and malignant potential in some of these tumours.

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http://dx.doi.org/.//
Review Article
Phyllodes Tumor of Breast: A Review Article
Shashi Prakash Mishra, Satyendra Kumar Tiwary,
Manjaree Mishra, and Ajay Kumar Khanna
Department of Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Ultar Pradesh 221005, India
Correspondence should be addressed to Ajay Kumar Khanna; akhannabhu@gmail.com
Received  January ; Accepted  February 
Academic Editors: M. G. Chiofalo, M. Frascio, H. Hirose, K.-E. Kahnberg, and M. Wronski
Copyright ©  Shashi Prakash Mishra et al. is is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.
Introduction. Phyllodes tumoursare rare broepithelial lesions. Accurate preoperative pathological diagnosis allows correct surgical
planning and avoidance of reoperation. Treatment can be either wide local excision or mastectomy to achieve histologically clear
margins. Discussion. e exact aetiology of phyllodes tumour and its relationship with broadenoma are unclear. Women aged
between  and  years are commonly involved. e median tumour size is cm but can grow even larger having dilated veins
and a blue discoloration over skin. Palpable axillary lymphadenopathy can be identied in up to –% of patients but <% had
pathological positive nodes. Mammography and ultrasonography are main imaging modalities. Cytologically the presence of both
epithelial and stromal elements supports the diagnosis. e value of FNAC in diagnosis of phyllodes tumour remains controversial,
but core needle biopsy has high sensitivity and negative predictive value. Surgical management is the mainstay and local recurrence
in phyllodes tumours has been associated with inadequate local excision. e role of adjuvant radiotherapy and chemotherapy
remains uncertain and use of hormonal therapy has not been fully investigated. Conclusion. e preoperative diagnosis and proper
management are crucial in phyllodes tumours because of their tendency to recur and malignant potential in some of these tumours.
1. Introduction
Phyllodes tumors are rare broepithelial lesions. ey make
up.to.%offemalebreasttumors[]andhavean
incidence of about . per million, the peak of which occurs in
womenagedtoyears[,]. e tumor is rarely found
in adolescents and the elderly. ey have been described as
early as , as a giant type of broadenoma []. Chelius
[] in  rst described this tumor. Johannes Muller (1838)
was the rst person to use the term cystosarcoma phyllodes.
It was believed to be benign until , when Cooper and
Ackerman reported on the malignant biological potential of
this tumor. In 1981 [6]theWorldHealthOrganizationadopted
the term phyllodes tumor and as described by Rosen []
subclassied them histologically as benign, borderline, or
malignant according to the features such as tumor margins,
stromal overgrowth, tumor necrosis, cellular atypia, and
number of mitosis per high power eld. e majority of
phyllodes tumors have been described as benign (% to
%), with the remainder divided between the borderline and
malignant subtypes. e term phyllodes tumor represents a
broad range of broepithelial diseases and presence of an
epithelial component with stromal components dierentiates
the phyllodes tumor from other stromal sarcomas.
Accurate preoperative pathological diagnosis allows cor-
rect surgical planning and avoidance of reoperation, either to
achieve wider excision or for subsequent tumor recurrence
[–]. At one extreme, malignant phyllodes tumors, if
inadequately treated, have a propensity for rapid growth
and metastatic spread. In contrast, benign phyllodes tumors
on clinical, radiological, and cytological examination are
oen indistinguishable from broadenomas and can be cured
by local surgery. With the nonoperative management of
broadenomas widely adopted, the importance of phyllodes
tumors today lies in the need to dierentiate them from
other benign breast lesions. Treatment can be either wide
local excision or mastectomy provided histologically clear
specimen margins are ensured [,,].
2. Etiology
At present time, the exact etiology of phyllodes tumor and
its relationship with broadenoma are unclear. Noguchi

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T 
Criteria Benign Borderline Malignant
Stromal
cellularity
and atypia
Minimal Moderate Marked
Stromal
overgrowth Minimal Moderate Marked
Mitoses/
high power
elds – – ≥
Tumor
margins
Well
circumscribed
with pushing
tumor margins
Zone of
microscopic
invasion around
tumor margins
Inltrative
tumor margins
et al. [] showed that most broadenomas have polyclonal
elements and should be regarded as hyperplasic rather than
neoplastic lesions. It has been suggested that, in a proportion
of broadenomas, a somatic mutation can result in a mono-
clonal proliferation, histologically indistinguishable from the
polyclonal element, but with a propensity to local recurrence
and progression to a phyllodes tumor which has also been
supported by clonal analysis. It has also been postulated
that stromal induction of phyllodes tumors can occur as a
result of growth factors produced by the breast epithelium.
Trauma,lactation,pregnancy,andincreasedestrogenactivity
occasionally have been implicated as factors stimulating
tumor growth. e nature of these factors is unclear but
endothelin-, a stimulator of breast broblast growth, may be
important.
3. Pathogenesis
Unlike carcinoma breast, phyllodes tumors start outside of
theductsandlobules,inthebreast’sconnectivetissue,called
thestromawhichincludesthefattytissueandligamentsthat
surroundtheducts,lobules,andbloodandlymphvesselsin
the breast. In addition to stromal cells, phyllodes tumors can
also contain cells from the ducts and lobules.
4. Classification
4.1. WHO Criteria. World Health Organization divided
phyllodes tumor into benign, borderline, and malignant
categories based on the degree of stromal cellular atypia,
mitotic activity per  high power elds, degree of stromal
overgrowth (these three are main), tumor necrosis, and
margin appearance (see Tabl e  ).
4.2. Criteria Proposed by Azzopardi et al. [14]andSalvadoriet
al. [2]. See Table .
5. Diagnosis
5.1. Clinical Presentation. Most of the tumor arises in women
aged between  and  years (approximately  years
T 
Criteria Histological type
Benign Borderline Malignant
Tumor margins Pushing ↔Inltrative
Stroma cellularity Low Moderate High
Mitoticrate(perhpf) <– ≥
Pleomorphism Mild Moderate Severe
F : Giant phyllodes tumor.
later than broadenoma), , ,  more prevalent in the
LatinAmericanwhiteandAsianpopulations[]. Few cases
have been reported in men and these have invariably been
associated with the presence of gynaecomastia. It usually
presents as a rapidly growing but clinically benign breast
lump. In some patients a lesion may have been apparent
for several years, with clinical presentation precipitated by a
sudden increase in size [,].
(i) eskinoverlargetumorsmayhavedilatedveinsand
a blue discoloration but nipple retraction is rare.
(ii) Fixation to skin and pectoralis muscles has been
reported, but ulceration is uncommon.
(iii) More commonly found in upper outer quadrant with
anequalpropensitytooccurineitherbreast.
(iv) Rarely presentation may be bilateral.
(v)emediansizeofphyllodestumorsisaroundcm.
% of tumors grow larger than  cm (giant phyl-
lodes tumor). ese tumors can reach sizes up to
 cm in diameter (see Figure ).
(vi) A signicant proportion of patients have history of
broadenoma and in a minority these have been
multiple.
(vii) Palpable axillary lymphadenopathy can be identied
in up to –% of patients but <% had pathological
positive nodes.
5.2. Radiological Investigations. Mammography and ultra-
sonography are mainstay of routine imaging of breast lumps.
Wurdinger et al. [ ] show that round or lobulated shape,
well-dened margins, heterogeneous internal structure, and
nonenhancing internal septations are more common ndings
in phyllodes tumors than in broadenomas.

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F : Phyllodes tumor on mammography.
5.2.1. Ultrasonography [18,19]. Lobulated shape (in some
cases round or oval) well circumscribed with smooth mar-
gins, echogenic rim, and low level homogenous internal
echoes. Fluid-lled cles in a predominantly solid mass
(highly suggestive of phyllodes tumor) with good thorough
transmission and lack of microcalcication are seen.
5.2.2. Color Doppler Ultrasonography. Features suggesting
malignant behavior are
(i) marked hypoechogenicity,
(ii) posterior acoustic shadowing,
(iii) ill-dened tumor margins.
(iv) higher values of RI (resistance index),
(v) increased PI (pulsatility index),
(vi) increased Vmax (systolic peak ow velocity).
5.2.3. Mammography [18,20,21](seeFigure 2)
(i) It shows well circumscribed oval or lobulated mass
with rounded borders.
(ii) A radiolucent halo may be seen around the lesion due
to compression of the surroundings.
(iii) Coarse calcication (but malignant microcalcica-
tion is rare) may be present.
5.2.4. Magnetic Resonance Imaging (MRI) [21–28]. e fol-
lowing features are mainly found in phyllodes tumor on MRI:
(i) round or lobulated shape and well-dened margins,
(ii) heterogeneous internal structure/nonenhancing sep-
tations,
(iii) exhibits hypointense signals on T-weighted images,
(iv) exhibits hyper/isointense signals on T-weighted
images,
(v) contrast enhancement pattern:
(a) benign lesion:
() slow initial enhancement with persistent
delayed phase;
(b) malignant lesion:
() fast initial enhancement with plateau
phage,
() fast initial enhancement with wash-out
phenomenon.
5.3. Pathological/Histological Assessment. As both phyllodes
tumors and broadenomas belong to a spectrum of broep-
ithelial lesions, accurate cytological diagnosis of phyllodes
tumors by ne needle aspiration can be dicult.
Cytologically, it is oen easier to dierentiate benign
from malignant phyllodes tumors than to separate benign
phyllodes tumors from broadenomas. In the correct clinical
setting, the presence of both epithelial and stromal elements
within the cytological smear supports the diagnosis. Epithe-
lial cells may, however, be absent from specimens taken from
malignant lesions. e presence of cohesive stromal cells
(phyllodes fragments), isolated mesenchymal cells, clusters of
hyperplastic duct cells, foreign body giant cells, blood vessels
crossing the stromal fragments, and bipolar naked nuclei
and the absence of apocrine metaplasia are highly suggestive
of a phy l lode s tumor. However, the va lue o f FNAC in t he
diagnosis of phyllodes tumor remains controversial, with an
overallaccuracyofabout%[,]. Core tissue biopsy
is an attractive alternative to FNAC because of the extra
architectural information provided by histology compared
with cytology. Komenaka et al. []foundthesensitivityof
core needle biopsy to be % and negative predictive value
and positive predictive value % and %, respectively, for
the diagnosis.
5.3.1. Macroscopic Appearance. Macroscopically most small
tumorshaveauniformwhiteconsistencywithalobulated
surface,similartothatofabroadenoma.Largetumorson
cut section oen have a red or grey “meaty” consistency with
brogelatinous, hemorrhagic, and necrotic areas with leaf like
protrusions into the cystic spaces.
5.3.2. Microscopic Appearance (see Figures 3–6). Fine Needle
Aspiration Cytology. e cytological diagnosis of phyllodes
tumors is mainly suggested by the presence of hypercellular
stroma and the stromal elements on the smears being more
numerous than the epithelial ones. e cells on the smears
were classied by Deen et al. [] in , and Jayaram
and Sthaneshwar in  [], by comparison with small
lymphocytes, in
() short, round/oval cells, two-size smaller than the size
of a lymphocyte: considered to be epithelial cells;
() long, spindle cells, three-size larger than the size of a
lymphocyte: considered to be stromal cells.
Many authors considered that the following aspects
shouldalsobetakenintoconsiderationinthecaseof
cytological diagnosis of phyllodes tumors:
(a)thepresenceofhypercellularstromalfragments;
(b) the cellular composition of the stromal fragments;
(c) the amount of naked nuclei on the background of the
smears;

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F : Stained slide showing microphotograph of phyllodes
tumor.
F : Stained slide showing microphotograph of phyllodes
tumor.
(d) the morphology of the naked nuclei (especially the
bipolar ones).
See Tab le  .
Core Needle Biopsy. Fibroepithelial lesions with cellular
stroma in breast core needle biopsy (CNB) specimens may
result in either broadenoma or phyllodes tumor at exci-
sion. Assessment of stromal cellularity, stromal cell atypia,
mitoses, and relative proportion of stroma to epithelium are
mainly helpful to reach the diagnosis. Phyllodes tumors are
usually dierentiated histologically from broadenoma by its
increased stromal cellularity and mitotic activity. However,
benign phyllodes tumor by denition lacks marked atypia
F : Stained slide showing microphotograph of phyllodes
tumor.
F : Stained slide showing microphotograph of phyllodes
tumor.
and excess mitotic activity in its stromal component, and
juvenile broadenoma may also have cellular stroma, pre-
senting a source of increased diagnostic diculty. Diagnosis
relies on the recognition of the exaggerated intracanalicular
growth pattern in phyllodes tumor. In addition, the stromal
proliferation in juvenile broadenoma tends to be relatively
uniform, whereas in phyllodes tumor it is oen (though not
always) more prominent in the periductal areas. e stromal
cellularity in phyllodes tumor may be heterogeneous. Con-
sequently, surgical excision is recommended for complete
evaluation of the lesion.
Jacobs et al. [] found that  stromal features in
CNB specimens (i.e., cellularity, nuclear atypia, mitoses, and
amount of stroma relative to epithelium) diered signicantly
between cases that were broadenoma at excision compared
with those that were phyllodes tumor. However, only cases
that had mildly or markedly increased stromal cellularity in
CNB specimens were absolutely predictive of broadenoma
or phyllodes tumor, respectively. Among the subset of cases
with moderate stromal cellularity in the CNB specimens, the

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T 
Benign phyllodes tumors Borderline phyllodes tumors Malignant phyllodes tumors
e stromal compound, represented by
stromal fragments, isolated stromal cells,
and naked stromal nuclei are found to be
more numerous than the epithelial one in
most of the cases.
(i) ere is predominance of the stromal
component as compared to the epithelial
one.
(ii) Frequent hypercellular stromal
fragments, an average of  in each
microscopical eld.
(iii) Frequent large spindle cells and
monomorphic naked stromal nuclei.
(i) Stromal fragments of variable
dimensions, with moderate cellularity,
made of discohesive spindle cells, with
atypical nuclei.
(ii) Minimal/no epithelial elements found
on the smears.
(iii) Presence of atypical multinucleated
giant cells.
Clinical ndings
(i) Sudden increase in size in a longstanding breast lesion
(ii) Apparent broadenoma >cmdiameterorinpatient> years
Imaging ndings
(i) Rounded borders/lobulated appearance at mammography
(ii) Attenuation or cystic areas within a solid mass on Ultrasonography
FNAC ndings
(i) Presence of hypercellular stromal fragments
(ii) Indeterminate features
ANY  features mandate core biopsy
B 
presence of stromal mitoses remained the single histological
feature signicantly dierent between the phyllodes tumor
and broadenoma groups.
Sarcomatous stromal elements, including angiosarcoma,
chondrosarcoma, leiomyosarcoma, osteosarcoma, liposar-
coma, and rhabdomyosarcoma, are rarely encountered in
malignant phyllodes tumors.
Paddington Clinicopathological Suspicion Score.isoutlines
criteria to assist in the selection of patients for core biopsy,
for use in conjunction with existing local protocols. e aim
of developing the score is to improve the rates of preoperative
diagnosis (see Box ).
5.3.3. Dierential Diagnosis. It includes the following:
(i) broadenoma,
(ii) adenoma,
(iii) hamartoma,
(iv) lipoma,
(v) juvenile papillomatosis,
(vi) carcinoma,
(vii) sarcomas,
(viii) metastatic tumor.
Management. Surgical management is the mainstay but the
type of surgery has been a source of debate over the years.
Studies have shown no dierences between breast conserving
surgery versus mastectomy in terms of metastasis-free sur-
vival or overall survival, despite the higher incidence of local
recurrence that comes with breast conserving surgery [].
If diagnosed preoperatively, tumor should be resected
with at least  cm margins particularly in the borderline and
malignant phyllodes tumors. is can be accomplished by
either lumpectomy or mastectomy, depending upon the size
of the tumor relative to the breast. For benign phyllodes
tumors diagnosed aer local excision of what appeared to
be a broadenoma, a “watch and wait” policy does appear
to be safe. With such an approach, local recurrence and ve
year survival rates of % and % respectively have been
reported for benign phyllodes tumors. Whether patients with
benign phyllodes tumors who have undergone local excision
and have histologically positive specimen margins should
undergo further surgery or be entered in a surveillance pro-
gram is controversial. Reexcision of borderline and malignant
phyllodes tumors identied aer local excision should be
considered.
Twenty percent of tumors grow larger than cm, the
arbitrary cuto point for the designation as giant phyllodes
tumor, an entity that presents the surgeon with several
unique management problems. ese tumors can reach sizes
up to  cm in diameter [].Sinceanexcisionwiththe
required margins is oen impossible in giant phyllodes
tumors, mastectomy should be reserved for larger tumors
and should be considered in recurrent tumors, especially
of the malignant histotype [,–]. Local recurrence in
phyllodes tumors has been associated with inadequate local
excision and various histological characteristics, including
mitotic activity, tumor margin, and stromal cellular atypia.
Depending on the size of the breast and the location of
the phyllodes tumor, mastectomy may also be required
for tumors that are between  and  cm in diameter
[]. While managing a giant phyllodes tumor, emphasis
should be on complete extirpation of all visible tumors and

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breast tissue during mastectomy to minimize the chances of
recurrence.
As malignant phyllodes tumors undergo mainly hema-
togenous spread, the proportion of patients with lymph
node metastases are <% (lymph node enlargement in about
%) and routine axillary clearance is not recommended.
Axillary dissection is required, when histologically positive
for malignant cells.
Chest wall invasion appears to be an uncommon event
with phyllodes tumors [], extended excision of involved
pectoralis muscle, followed by reconstruction of the chest
wall with marlex mesh or latissimus dorsi muscular/myocu-
taneous ap been recommended if the fascia or muscle is
inltrated. Some have recommended the consideration of
postoperative radiation for cases of chest wall inltration.
Foreknowledge of the location of the tumor’s blood
supply can be vital informationwhen removing large tumors.
Jonsson and Libshitz documented the angiographic pattern
of a  cm phyllodes tumor via one large and several smaller
perforating anterior branches of the internal mammary,
lateral thoracic, acromiothoracic arteries, and branches of the
axillary artery []. Liang et al. [] found that the giant
tumors in the present report derived the majority of their
blood supply from skin collaterals. us, the surgeon can
expect the majority of blood loss during resection to come
from the creation of the skin aps. In this situation, the
surgeon need not routinely obtain an angiogram.
In general, immediate breast reconstruction can be per-
formed at the time of mastectomy for phyllodes tumors [].
Mendel et al. [] reported a case in which subcutaneous mas-
tectomy was performed for a large phyllodes tumor, followed
by immediate implantation of a breast prosthesis. ey cite
minimal interference with the detection of recurrent lesions
and the minimization of emotional distress as advantages to
the procedure. Orenstein and Tsur described a similar case in
an adolescent female in which a silicon implant was placed
under the pectoralis major, where it would not impair the
recognition of recurrent disease []. Local recurrence rates
forphyllodestumorsareto%andarecorrelatedwith
positive excision margins, rather than with tumor grade or
size [,,,]. Other studies have shown a higher risk
of local recurrence in borderline and malignant tumors. In
a series of  patients by Salvadori et al.,  patients were
treated with breast conserving surgery (enucleations, wide
excisions), and  of the tumors recurred locally. In contrast,
the  patients treated with mastectomy (subcutaneous,
modiedradical,orradical)showednoevidenceoflocal
recurrence []. Importantly, there is no contraindication to
immediate reconstruction aer mastectomy in cases of giant
phyllodes tumor, and this decision can be made solely based
upon patient preference [,].
NCCN Guidelines for the Management of Phyllodes Tumor.
According to NCCN guidelines wide excision means excision
with the intention of obtaining surgical margins ≥cm.
Narrow surgical margins are associated with high local recur-
rence risk, but are not an absolute indication for mastectomy
when partial mastectomy fails to achieve margin width ≥cm
(see Figure ).
Role of Adjuvant erapy. e role of adjuvant radiother-
apy and chemotherapy remains uncertain, but encouraging
results using radiotherapy and chemotherapy for so-tissue
sarcomas suggest that consideration be given for their use in
cases of malignant phyllodes tumors [–].
Chaney et al. [] found adjuvant radiotherapy to be ben-
ecial in patients with adverse features (e.g., bulky tumors,
close or positive surgical margins, hypercellular stroma,
high nuclear pleomorphism, high mitotic rate, presence of
necrosis, and increased vascularity within the tumor and
tumor recurrence) but the use is controversial. A study done
by Richard J. Barth Jr demonstrated that margin-negative
resectioncombinedwithadjuvantradiotherapyisaneective
therapy for local control of borderline and malignant phyl-
lodes tumors. In MD Anderson Cancer Center, radiotherapy
is recommended only for cases with positive or near-positive
surgical margins and selected cases for whom further surgical
procedures cannot be performed.
Chemotherapy, including anthracyclines, ifosfamide, cis-
platin, and etoposide, has been mentioned in various studies
but with no survival advantage.
e use of hormonal therapy, such as tamoxifen, has not
been fully investigated in cystosarcoma phyllodes. Estrogen
and progesterone receptor expression has been shown in %
and %, respectively, of the epithelium and less than % of
the stromal cells. Still, the use of endocrine therapy in either
the adjuvant or palliative setting has not been extensively
studied.
Prognostic Factors. No reliable clinical prognostic factors have
been identied that predict for local recurrence or metastasis.
Patient age does not appear to be important but tumors
presenting in adolescence do seem to be less aggressive
irrespective of their histological type. e size of the tumor
not as such but in relation to the breast appears important as
this usually determines the extent of surgery and the resulting
specimen resection margins.
Most distant metastases develop from borderline or
malignant tumors. Unlike local recurrence, tumor size does
appear to be an important factor in predicting for metastatic
spread. Many histological prognostic factors have been eval-
uated. Dierent studies have regarded stromal overgrowth,
tumor necrosis, inltrating margins, mixed mesenchymal
components, high mitotic rate, and stromal atypia as impor-
tantbutinisolationeachappearstohavealowpredictive
value.
5.3.4. Role of Tumor Markers in Phyllodes Tumor. Increased
p protein and Ki- antigen expression has been detected
in malignant phyllodes tumors and they may be valuable
in dierentiating broadenomas from phyllodes tumors.
Furthermore, in phyllodes tumors, p and Ki- expression
has been shown to correlate with negative prognostic factors.
Philip C. W. et al. showed the role of angiogenesis and
found that the higher the microvessel density, the higher the
degree of malignancy for the phyllodes tumor.
Gary M. K. Tse et al. found that CD protein expression
by stromal cells in phyllodes tumors is correlated with histo-
logical parameters such as grade, implying a possible role of

ISRN Surgery 
Clinical presentation Clinical suspicion of phyllodes tumor
Palpable mass
Rapid growth
Imaging with ultrasound suggestive of
broadenoma except for size and/or
history of growth
Workup History and physical examination
Ultrasound
Findings Treatment
Observe
FibroadenomaExcisional biopsy
Phyllodes tumors
including benign, borderline,
and malignant axillary staging
Core needle biopsy Fibroadenoma or indeterminate Excisional biopsy
Phyllodes tumor axillary staging
∙
∙
∙
∙
∙
∙
H
Mammogram for women ≥30 yrs
Wide excision (≥1 cm) without
Wide excision (≥1 cm) without
Large size (>2 cm)
F 
their being used as adjunctive markers of malignancy in these
tumors. In malignant phyllodes tumors, the rate of expression
is up to %. is provides additional strong evidence
that c-kit receptor-mediated tyrosine kinase activity may be
involved early on in the pathogenesis of phyllodes tumors,
and the new therapeutic agent, STI, Glivec, may be a
useful drug therapy for this disease, particularly in the tumor
recurrences and advanced-stage disease. Marick Lae et al.
showed that chromosomal changes detected by comparative
genomic hybridization (CGH) could be helpful in grading
phyllodes tumors. In ow cytometric studies, correlations
between DNA content, cell proliferation, and histological
grade have been demonstrated. Some studies have identied
a correlation between these markers of cellular proliferation
and clinical outcome, however, most have not. Recent small
studies have suggested that telomerase, a ribonucleoprotein
enzyme that generates telomeres (DNA sequences important
in determining cell immortality), may be a useful prognostic
factor in phyllodes tumors.
Recurrence. To date, local recurrence rates ranging from %
to % have been reported with most series averaging about
%. Local recurrence appears to be related to the extent
of the initial surgery and should be regarded as a failure of
primary surgical treatment. Whether malignant tumors have
an increased risk of recurrence is unclear but when it does
occur it is invariably seen earlier than with benign tumors.
In multivariate analysis, the surgical margin is found to be
the only independent predictive factor for local recurrence.
In most patients, local recurrence is isolated and is not
associated with the development of distant metastases.
In a minority of patients repeated local recurrence occurs.
isisoenseenirrespectiveofeitherthehistological
type of the tumor or the extent of the specimen margins.
Localrecurrencecanusuallybecontrolledbyfurtherwide
excision (with  cm margins) and mastectomy is not invari-
ably required. Mastectomy should, however, be considered
for local recurrence aer local surgery for borderline or
malignant tumors. Occasionally aggressive local recurrence
can result in widespread chest wall disease with direct
invasion of the underlying lung parenchyma. Isolated reports
of good palliation in this situation with radiotherapy have
been published.
NCCN Guidelines for the Management of Recurrence. See
Figure .
Metastasis. Overall, % of patients with phyllodes tumors
develop distant metastases and these eventually occur in
approximately % of patients with histologically malignant

ISRN Surgery
Locally recurrent breast mass following
excision of phyllodes tumor
History and physical examination
Ultrasound
Mammogram
Tissue sampling
Consider chest imaging
No metastatic disease Metastatic disease
Reexcision with wide margins without Metastatic disease management following
axillary staging principles of so tissue sarcoma
∘
∘
∘
∘
∘
Consider postoperative radiotherapy∗∗
∗∗ere is no prospective randomized data supporting the use of radiation treatment with phyllodes
tumors. However, in the setting where additional recurrence would create signicant morbidity, e.g.,
chest wall recurrence following salvage mastectomy, radiation therapy may be considered, following
the same principles that are applied to the treatment of so tissue sarcoma.
F 
tumors. Most distant metastases develop without evidence of
local recurrence. e commonest sites for distant metastases
are the lungs (%), bones (%), and brain (%) and in rare
instances,theliverandheart.eriskofmetastaticdisease
does not appear to be inuenced by the extent of the initial
surgery and appears to be predetermined by tumor biology.
Metastatic phyllodes tumors have a poor prognosis and no
long-term survival.
Followup. Since phyllodes tumors are locally recurrent tumor
especially when not excised with a clear margins and very
unpredictableingrowthandmetastaticactivity,itisverynec-
essary to follow up the patient regularly at -month interval
for the rst two years (chances of recurrence are maximum
in the rst two years) and then on yearly basis. Patients
must be instructed to self examine her breast regularly and
consult her doctor, if any abnormality detected. In followup,
patient should be examined and, if any abnormality detected,
it should be investigated with USG, mammogram, MRI, or
tissue biopsy.
6. Conclusion
Phyllodes tumor bears specic clinical characteristic and
can be considered as a dierential diagnosis for the breast
lumps. e preoperative diagnosis and proper management
are crucial in phyllodes tumor because of their tendency to
recur and malignant potential in some of these tumors.
Conflict of Interests
e authors declared that they have no conict of interests.
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