Archives of International Surgery Desmoid tumor in the maxillary sinus

Abstract

Desmoid tumor or desmoid-type fibromatosis is a benign but aggressive tumor that usually arises from the musculoaponeurotic structures in the body. It is very rare accounting for less than 3% of all soft-tissue tumors. Intra-abdominal and extra abdominal forms of this disease have been identified in the literature. Mandible, tongue, mastoid process, and maxilla are the most common subsites affected by the tumor in the head and neck region. Occurrence of desmoid tumors in the paranasal sinus is extremely rare, and only very few cases were reported in the literature especially in Nigeria. We present a case of desmoid tumor occurring in the maxillary sinus of a 29-year-old lady from Nigeria. The clinical presentation, diagnosis, and successful treatment of desmoid tumor are discussed. Desmoid-type fibromatosis is an aggressive benign tumor that rarely occurs in the maxilla. It can progressively increase in size, leading to facial disfigurement. Meticulous surgery is necessary to ensure adequate resection of the tumor and at the same time avoiding damage to vital surrounding structures.

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© 2020 Archives of International Surgery | Published by Wolters Kluwer - Medknow 39
Desmoid tumor in the maxillary sinus
ABSTRACT
Desmoid tumor or desmoid-type fibromatosis is a benign but aggressive tumor that usually arises from the musculoaponeurotic
structures in the body. It is very rare accounting for less than 3% of all soft-tissue tumors. Intra-abdominal and extra abdominal
forms of this disease have been identified in the literature. Mandible, tongue, mastoid process, and maxilla are the most common
subsites affected by the tumor in the head and neck region. Occurrence of desmoid tumors in the paranasal sinus is extremely
rare, and only very few cases were reported in the literature especially in Nigeria. We present a case of desmoid tumor occurring
in the maxillary sinus of a 29-year-old lady from Nigeria. The clinical presentation, diagnosis, and successful treatment of desmoid
tumor are discussed. Desmoid-type fibromatosis is an aggressive benign tumor that rarely occurs in the maxilla. It can progressively
increase in size, leading to facial disfigurement. Meticulous surgery is necessary to ensure adequate resection of the tumor and at the
same time avoiding damage to vital surrounding structures.
Key words: Desmoid fibromatosis, desmoid tumor, maxillary sinus
Iliyasu Y. Shuaibu, Mohammed A. Usman, Nurudeen A. Shofoluwe, Abdulrazak Ajiya1,
Dotiro Chitumu, Mohammed I. Babatunde
Division of Otorhinolaryngology, Department of Surgery, Ahmadu Bello University Teaching Hospital, Ahmadu Bello University,
Zaria, 1Department of Otorhinolaryngology, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria
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DOI:
10.4103/ais.ais_41_19
form is the most common and may carry poor prognosis
especially when it is associated with familial adenomatous
polyposis and Gardner syndrome because of their diffusely
infiltrative and unresectable nature.[2] Extraabdominal
desmoid tumors are mostly sporadic and are amenable
to local surgical resection.[2] However, systemic treatment
is reserved for refractory disease.[3] Despite the cases of
desmoid tumor reported in the head and neck region, its
occurrence in the paranasal sinus is extremely rare and
only very few cases have been reported in the literature.
To the best of our knowledge, no case of maxillary sinus
desmoid tumor has been reported in Nigeria. We report a
case of desmoid fibromatoses of the maxillary sinus in a
Nigerian lady.
Introduction
Desmoid tumor or desmoid‑type fibromatosis is a
benign but aggressive tumor that usually arises from
the musculoaponeurotic structures in the body.[1] It is
very rare, accounting for less than 3% of all soft‑tissue
tumors.[2] Low‑grade fibroblastic proliferation, infiltration
of surrounding structures, and tendency to recur after
excision are the three main characteristics of this tumor.[3]
Intra‑abdominal and extra‑abdominal forms of this disease
have been identified in the literature.[3] The intra‑abdominal
How to cite this article: Shuaibu IY, Usman MA, Shofoluwe NA, Ajiya A,
Chitumu D, Babatunde MI. Desmoid tumor in the maxillary sinus. Arch
Int Surg 2019;9:39-42.
This is an open access journal, and articles are distributed under the
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4.0 License, which allows others to remix, tweak, and build upon the
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For reprints contact: WKHLRPMedknow_reprints@wolterskluwer.com
Address for correspondence: Dr. Abdulrazak Ajiya,
Department of Otorhinolaryngology, Aminu Kano Teaching Hospital,
Bayero University, Kano, Nigeria.
E‑mail: ajiyaabdulrazak@yahoo.com
Received: 25 December 2019 Revised: 31 March 2020
Accepted: 05 April 2020 Published: 08 August 2020
Case Report
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Shuaibu, et al.: Desmoid tumor in the maxillary sinus
40 Archives of International Surgery / Volume 9 / Issue 2 / April-June 2019
Case Report
The patient was a 29‑year‑old Muslim housewife. She
presented to our clinic with a 2‑year history of left‑sided
facial swelling, which was insidious in onset and gradually
increased in size to involve the left eye with associated
proptosis. She also had history of left‑sided persistent
nasal blockage, rhinorrhea, and anosmia. No exposure to
irradiation, noxious chemicals, or allergic reaction. She
does not smoke cigarette nor ingest alcohol.
Examination revealed left facial mass extending from the
upper part of the left eyebrow to the upper part of the lip
measuring 12 cm by 8 cm [Figure 1] with a discharging sinus
in the lower part of the cheek. It was hard and attached
to underlying structures but the skin over it was mobile.
She had left‑sided non‑axial proptosis and purulent eye
discharge. However, her vision was intact. The external nasal
pyramid was distorted with associated hypertelorism and
deviation of the nasal septum to the right. The right nasal
cavity was patent. No mass in both nasal cavities. The hard
palate appeared normal with no erosion, bulge, or ulceration.
An impression of left maxillary mass with differential
diagnosis of left fibro‑osseous lesion to rule out osteoma
was made.
Computed tomographic (CT) scan of the paranasal sinuses
showed an expansile homogenous huge soft‑tissue mass
arising from the left maxillary antrum and extending to the
left orbit causing proptosis and distortion of the ipsilateral
nasal cavity. There was no intracranial extension.
Absence of an intranasal mass prompted a sublabial
approach for an incisional biopsy and histology revealed
features of desmoid tumor. Preoperative baseline
investigations such as electrolyte, urea, and creatinine
and full blood count and differentials were normal.
A preoperative obturator fabrication was made in
preparation for a possible total maxillectomy. Following
informed consent, patient had excisional biopsy via Weber
Ferguson incision [Figure 2a and b]. Intraoperative finding
was that of a huge grayish firm to hard mass occupying
left maxillary sinus with associated destruction of the
anterior and roof of the maxillary antrum [Figure 3]. The
mass was completely resected leaving intact hard palate,
and an obturator was not fitted. No injury to vital structures
was observed. Patient was transfused 2 units of blood
postoperatively.
Following removal of stitches on the 8th day postsurgery,
patient was discharged on the 9th postoperative day.
Histopathological findings from the tissues revealed spindle
cell tumor growing in diffuse whorls and stariform patterns
with scanty collagenous stroma and infrequent mitosis
in keeping with desmoid‑type fibromatosis [Figure 4].
The patient is currently being followed up with 6 monthly
appointments and no recurrence was observed. On every
appointment, Patient is usually subjected to thorough
history taking and physical examination together with
nasal endoscopic assessment. A repeat CT scan was not
done because of financial constraints.
Figure 3: Grayish hard mass after excision
Figure 1: Left-sided hard facial mass (a) (anterior view) and (b) (lateral
view)
b
a
Figure 2: (a) Weber–Ferguson incision during tumor excision. (b) After
wound closure
b
a
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Shuaibu, et al.: Desmoid tumor in the maxillary sinus
Archives of International Surgery / Volume 9 / Issue 2 / April-June 2019 41
Discussion
Desmoid tumor is a rare benign, locally aggressive
soft‑tissue tumor accounting for approximately 0.03% of
all neoplasms, and less than 3% of all soft‑tissue tumors.[2]
The incidence of Desmoid tumour (DT) has been estimated
to be approximately 2–4/1,000,000/year of the general
population.[4] Up to 900 new cases annually are reported
in the USA.[4] There is no known etiology of Desmoid
tumour but trauma, endocrine or genetic factors have been
speculated as the possible etiologic factors.[5] Dysregulation
of wound healing has been suggested as the possible
pathogenesis of the disease.[6] It usually occurs as large
bulky tumors that locally infiltrate adjacent structures.
Desmoid tumor involving the head and neck region is
rare and constitutes 11%–12% of all cases in the body.[7]
Mandible, tongue, mastoid process, and maxilla are the
most common subsites affected by the tumor in the head
and neck region.[8]
Desmoid tumor can occur in all age groups but has been
observed to be more common between the third and
fourth decade of life.[9] The age of our patient (29 years)
is fairly in agreement with the finding in the literature.
Female preponderance was also observed by Islam
et al
.[2]
However, Min
et al
.[10] reported male preponderance in his
report. Overall, female‑to‑male ratio was between 3:2 and
2:1[7] and no ethnic or racial preference was observed.
Patient with Desmoid tumour may present with nasal
blockage, noisy breathing, epistaxis, facial pain, and
slowly growing mass that may be fixed to the underlying
structures. Other features such as proptosis, epistaxis,
stridor, and trismus were also reported.[7] Our patient
presented with facial swelling, proptosis, persistent nasal
blockage, rhinorrhea, anosmia, and discharging sinus from
the mouth with no epistaxis and trismus.
Radiological investigation such as CT scan may reveal the
site and extent of the tumor. It may appear as a homogenous
lesion filling the antrum with associated erosion of the
walls.[1] Our patient presented with homogenous expansile
huge soft‑tissue mass arising from the left maxillary
antrum and extending to the left orbit causing proptosis
with associated erosion of the anterior wall and roof of the
maxillary antrum. The hard palate was intact.
Biopsy is necessary to establish a histological diagnosis. It
is recommended that the biopsy should be deep enough to
include periosteum[1] to ascertain its infiltrative nature. There
are controversies on whether the biopsy should be taken from
the edge or central part of the tumor. The proponents of biopsy
from the edge argued that taking a biopsy from the center
may be mistaken for scar tissue during the main surgical
resection.[11] Other workers opined that taking the biopsy at
the edge will induce scar tissue that is histologically similar
to the tumor which can lead to confusion about surgical
margin.[5] As observed in our patients, Desmoid tumour is
large, bulky with a tendency of infiltrating locally adjacent
structures. The possible drawback with our sublabial
approach for biopsy is difficulty with proper establishment
of surgical margin and the point of incision being a possible
avenue for residual disease. This informs why our patient is
still on follow‑up. The histological characteristic of the tumor
is the presence of spindle cells, collagenous stroma and
infrequent mitoses.[2] The histopathological findings of our
patient also revealed spindle cells tumor growing in diffuse
whorly and stariform patterns with scanty collagenous
stroma and infrequent mitosis.
Complete surgical excision is the recommended modality of
treatment. This is usually achieved by wide local resection
with a 1.5 cm free margin while at the same time preserving
the vital structures to avoid significant morbidity.[1] The
possibility of residual disease or recurrence is common
due to the infiltrative nature of the tumor. Recurrence of up
to 20%–70% of cases usually within 2 months to 11 years
has been reported.[12]
Thus, multi‑modality treatment options including surgery,
chemotherapy, and radiotherapy have been suggested
Figure 4: Desmoid tumor of the maxilla (H and E). (a) (original
magnication, ×100) Low power view showing both storiform and
whorling spindle cell patterns. (b) (original magnication, ×400) higher
magnication of same tumor showing taper‑ended plump broblastic
cells laying down collagenous matrix. (c): (original magnication,
×40) Tumor margin showing inltration of surrounding bone (green
arrow). (d): (original magnication, ×40) calcic remnants of destroyed
bone (black arrows)
d
c
b
a
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Shuaibu, et al.: Desmoid tumor in the maxillary sinus
42 Archives of International Surgery / Volume 9 / Issue 2 / April-June 2019
in the literature.[13] Moreover, the use of nonsteroidal
anti‑inflammatory drugs and tamoxifen, though not yet
validated, have also been suggested as the treatment for
the recurrent desmoid tumors.[14] Various studies have
shown that the main factors associated with recurrence
among patients were incomplete resection of the tumor
or positive surgical margins.[2,15] In contrast to this, other
authors opined that recurrence is independent of surgical
margins status.[16,17] Transformation of Desmoid tumour
to malignant disease is very rare. Only very few series
reported transformation to fibrosarcoma.[10] Our patient
is being followed up every 6 months and no recurrence
observed after 20 months.
Conclusion
Desmoid‑type fibromatosis is an aggressive benign tumor
that rarely occurs in the maxilla. It can progressively
increase in size, leading to facial disfigurement. Meticulous
surgery is necessary to ensure adequate resection of
the tumor and at the same time avoiding damage to
vital surrounding structures. Long‑term follow‑up is
recommended in patients with. This will help in early
detection of recurrence and institution of appropriate
management of the affected patients.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient (s) has/have
given his/her/their consent for his/her/their images and
other clinical information to be reported in the journal. The
patients understand that their names and initials will not
be published and due efforts will be made to conceal their
identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conicts of interest
There are no conflicts of interest.
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