Maxillary Fibromatosis: A Rare Clinical Report and Its Review of Literature

Abstract

Fibromatosis is a rare locally aggressive benign tumour which arises from the musculoaponeurotic structures throughout the body. In the oral and maxillofacial region, It has been described under a variety of synonyms, including ‘extra articular desmoids’, ‘desmoids tumours’, ‘grade-1 fibrosarcomas’,‘non metastasizing fibrosarcoma’and ‘aggressive fibromatosis’. The pecularity of this entity in paranasal sinuses is that it is rare in this location and are locally aggressive with higher rates of recurrence in a relatively restricted area.The purpose of this study is to present a rare case report and reviewing the literature of this entity.

Introduction

Fibromatosis is a rare locally aggressive benign tumour which arises from the musculoaponeurotic structures throughout the body. In the oral and maxillofacial region, It has been also been named ‘extra articular desmoids’, ‘desmoids tumours’, ‘grade-1 fibrosarcomas’,‘non metastasizing fibrosarcoma’and ‘aggressive fibromatosis’. The pecularity of this entity in paranasal sinuses is that it is rare in this location and are locally aggressive with higher rates of recurrence in a relatively restricted area [1]. Fibromatosis remain a fibrous tissue proliferation with an intermediate biologic behavior between a benign fibroma and fibrosarcoma [2]. So it exhibits destructive infiltrative growth and frequently recurs but rarely metastasize [2]. Fibromatosis is very uncommon in head and neck region [2]. The supraclavicular fossa is the most common site for head and neck fibromatosis (40%–85%), followed by face (about 25%) [3]. Other sites affected are fibromatosis of mandible, tongue, all the paranasal sinuses, infratemporal space and para pharyngeal space [4–6]. The oral and maxillofacial locations (mostly the mandible, maxilla and mastoid area) show a younger peak age range (5–20 years compared to 35 years), more infiltrative and faster growth and a much greater propensity to invade underlying bone or to arise seemingly from bone [2]. In our case report, patient is 25 years old which is within the most prevalent age group. The purpose of this study is to present the rare case report and reviewing the literature of this entity.

Patient and Methods

A 36 year old male with good performance status of 0 presented to our OPD with complaints of left sided gradually progressing palatal swelling since last 6 months. There were no associated symptoms of difficulty in mouth opening/ear ache/proptosis/pain over the local site. On clinical examination, there was presence of 5 × 5 cm smooth lobulated swelling confined to left half of hard palate, with no extension to soft palate or retromolar trigone (Fig. 1). Overlying cheek skin was free.There were no clinical neck nodes.On radiological evaluation,Contrast enhanced computer tomography of face and neck revealed heterogenously enhancing mass of size 5.5 × 5 cm in size in left side of hard palate with posterior extension to pterygoid plates and involving both pterygoid muscles with possible extension to soft palate (Fig. 2). The inferior wall of maxillary sinus seemed to have tumor extension along with its mucosal thickening.Punch biopsy of the lesion revealed fibrinous material,areas of necrosis and proliferated spindle cells.Patient was counselled for surgery.Routine blood investigations were performed before surgical intervention.He underwent total maxillectomy utilizing Weber-Ferguson incision (Fig. 3). Intraoperative frozen section of the resected maxillectomy specimen was performed which revealed no evidence of malignancy. Finally, the residual maxillary defect was packed with betadine soaked rollgauze for obturator placement in postoperative period.

Fig. 1.

Clinical picture of palatal growth

Fig. 2.

CT neck showing tumor extent to pterygoid plates

Fig. 3.

Intraoperative pic of total maxillectomy

Results

He was extubated on the Postoperative day 0. He was started on Ryle’s tube feed on postoperative day 1.His recovery remained uneventful, wound was healthy and was discharged on postoperative day 5 after seeking consultation from Prosthodontist for his subsequent visit (Fig. 4). His final histopathology report revealed long sweeping fascicles with elongated, slender, spindled cells of uniform appearance and pale cytoplasm set in a collagenous stroma. There was no nuclear hyperchromasia, minimal cytologic atypia and low mitotic rate. Also there was presence of thin walled and prominent blood vessels with perivascular edema. On Immunohistochemical panel, tumor cells were found positive for Beta catenin while they were negative for CD34 and Cytokeratins. Final diagnosis of fibromatosis was reached at. He was provided interim prosthesis after 1 month postoperatively. He was started on adjuvant tablet Tamoxifen 20 mg once daily and is currently disease free with regular follow up from last 3 months.

Fig. 4.

Pic showing well healed wound

Discussion

Fibromatosis is a rare locally aggressive benign tumour which arises from the musculoaponeurotic structures throughout the body.Fibromatosis remain a rare clinical entity in oral and maxillofacial region.In head and neck region,the reported incidence in literature ranged from 11% to 12% [7, 8]. Most cases are prevelant between third and fourth decades. A study had shown that males are more affected with the male to female ratio of 3:2 [9]. The exact aetiology of fibromatosis is still unknown. However, trauma, endocrine or genetic factors awere found to be probable aetiological factors, but still no satisfactory explanations present behind them [2]. Fibromatosis in the head and neck region recur from 20 to 70% of cases,whether it is due to a more aggressive nature of the tumour or to a technically more difficult excision in this region are unclear. But fibromatosis almost never or in very few cases turn into fibrosarcoma [10, 11].

The usual presentation of fibromatosis is slowly growing asymptomatic swelling which is often fixed with the underlying deep muscles or bony structures but not to the skin [12]. This tendency often results trismus. These findings were consistent with our case report too. Other features may be nasal obstruction, epistaxis, facial pain, dyspnoea, and proptosis depending upon the anatomical sites of the lesion [13]. It was shown that on CT scan face it showed poorly demarcated, irregular bone destruction and if the lesion was central, it usually showed a destructive pattern in all directions [2]. Extensive bone destruction of all the walls of the maxillary antrum, involvement of the all muscles of mastication on the left side suggested us an aggressive lesion. The diagnosis required a deep incisional biopsy in the centre of the mass. The biopsy should extend to the bone and include periosteum to assess the infiltrative growth pattern. A biopsy at the edge of the tumour will induce scar tissue that is histologically similar to the tumour, thereby confusing the margin at the time of excision [6]. In our case,punch biopsy was taken intraorally.The main treatment modality remained the wide surgical excision of the lesion and any involved bone with 1–1.5 cm of healthy margin.Treatment of the lymph nodes is not necessary [2]. But in the head and region, preservation of the vital structures and their function may impede these objectives [14]. Therefore, a multi-modality treatment strategy is usually employed to control residual disease. Surgery combined with radiotherapy or and chemotherapy are being reported. Besides, anti estrogen (Tamoxifen) therapy, even NSAIDS (Sulindac) are being used. But their efficacy is not established.

Histopathological features showed grayish white, firm or rubbery in consistency with its long axis usually oriented in the direction of muscle bundles in which it was found. It remained unencapsulated with ill defined borders. In our case report, per operatively, it was evident that the lesion had a special affinity to the muscles. The lesion was poorly localized and not encapsulated. The muscles were dark grayish on their cut surface. In our case, post operative histopathology showed proliferation of fibrous tissue with abundant collagen along with dense infiltration of acute and chronic inflammatory cells. The extensive fibrosis of the muscles in per operative findings, no anaesthesia or paraesthesia, no lymph nodes involvements, no evidence of metastasis, absence of any malignant features and post operative histopathology with IHC positive for Beta catenin confirmed us the lesion to be fibromatosis. In several reports, Radiotherapy alone (50–60 Gy), or combined with surgery in patients with positive margin gave long term control in 70%–80% of patients with desmoids [15, 16].

Conclusions

Fibromatosis is rare in maxillofacial region. But it is locally aggressive as it recurs more frequently. In most cases, the lesion is asymptomatic and the patients seek treatment when the lesion becomes a significant size and when the lesion involves the surrounding structures which make the surgery more difficult. Surgery is the treatment of choice with 1.5 cm healthy margin. Adjuvant therapy by radiotherapy is advised in patients with residual disease. Other modalitieds with Tablet tamoxifen is being used as it has shown efficacy for local control. Long term follow up is necessary because re-growth or recurrence could occur after a long period of stabilization.

Author Contributions

A, B, C made and reviewed the manuscript and D, E reviewed the manuscript.

Funding

No funds, grants, or other support was received.

Declarations

Conflict of interest

The authors have no relevant financial or non-financial interests to disclose.

Ethical Approval

Ethical approval was waived by the local Ethics Committee as it is a part of routine care.

Informed Consent

Taken from the patient.