|Year : 2019 | Volume
| Issue : 2 | Page : 39-42
Desmoid tumor in the maxillary sinus
Iliyasu Y Shuaibu1, Mohammed A Usman1, Nurudeen A Shofoluwe1, Abdulrazak Ajiya2, Dotiro Chitumu1, Mohammed I Babatunde1
1 Division of Otorhinolaryngology, Department of Surgery, Ahmadu Bello University Teaching Hospital, Ahmadu Bello University, Zaria, Nigeria
2 Department of Otorhinolaryngology, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria
|Date of Submission||25-Dec-2019|
|Date of Acceptance||05-Apr-2020|
|Date of Web Publication||08-Aug-2020|
Dr. Abdulrazak Ajiya
Department of Otorhinolaryngology, Aminu Kano Teaching Hospital, Bayero University, Kano
Source of Support: None, Conflict of Interest: None
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.
Keywords: Desmoid fibromatosis, desmoid tumor, maxillary sinus
|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
| Introduction|| |
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. Low-grade fibroblastic proliferation, infiltration of surrounding structures, and tendency to recur after excision are the three main characteristics of this tumor. Intra-abdominal and extra-abdominal forms of this disease have been identified in the literature. The intra-abdominal 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. Extraabdominal desmoid tumors are mostly sporadic and are amenable to local surgical resection. However, systemic treatment is reserved for refractory disease. 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.
| 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.
|Figure 1: Left-sided hard facial mass (a) (anterior view) and (b) (lateral view)|
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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 2]a and [Figure 2]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.
|Figure 2: (a) Weber–Ferguson incision during tumor excision. (b) After wound closure|
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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 4: Desmoid tumor of the maxilla (H and E). (a) (original magnification, ×100) Low power view showing both storiform and whorling spindle cell patterns. (b) (original magnification, ×400) higher magnification of same tumor showing taper-ended plump fibroblastic cells laying down collagenous matrix. (c): (original magnification, ×40) Tumor margin showing infiltration of surrounding bone (green arrow). (d): (original magnification, ×40) calcific remnants of destroyed bone (black arrows)|
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| 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. The incidence of Desmoid tumour (DT) has been estimated to be approximately 2–4/1,000,000/year of the general population. Up to 900 new cases annually are reported in the USA. There is no known etiology of Desmoid tumour but trauma, endocrine or genetic factors have been speculated as the possible etiologic factors. Dysregulation of wound healing has been suggested as the possible pathogenesis of the disease. 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. Mandible, tongue, mastoid process, and maxilla are the most common subsites affected by the tumor in the head and neck region.
Desmoid tumor can occur in all age groups but has been observed to be more common between the third and fourth decade of life. 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. However, Min et al. reported male preponderance in his report. Overall, female-to-male ratio was between 3:2 and 2:1 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. 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. 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 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. 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. 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. 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. 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.
Thus, multi-modality treatment options including surgery, chemotherapy, and radiotherapy have been suggested in the literature. 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. Various studies have shown that the main factors associated with recurrence among patients were incomplete resection of the tumor or positive surgical margins., In contrast to this, other authors opined that recurrence is independent of surgical margins status., Transformation of Desmoid tumour to malignant disease is very rare. Only very few series reported transformation to fibrosarcoma. 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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]