|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 3 | Page : 289-290
N Gangwar1, R Balakrishnan1, P Pujary1, L Rao2, R Kudva2
1 Department of ENT, Kasturba Medical College, Manipal University, Karnataka, India
2 Department of Pathology, Kasturba Medical College, Manipal University, Karnataka, India
|Date of Web Publication||18-Feb-2016|
Department of ENT, Kasturba Medical College, Manipal University, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gangwar N, Balakrishnan R, Pujary P, Rao L, Kudva R. Myofibrosarcoma-Maxilla. Indian J Cancer 2015;52:289-90
Myofibrosarcoma is a rare malignant soft tissue tumor with neoplastic cells differentiating into myofibroblasts., Eyden et al. reported this entity in 1992. It affects the deep soft tissues of the oral cavity commonly tongue and is also reported in limbs, trunk, abdominal and pelvic cavities. we present a 27 year old male who presented with complaints of progressively increasing painless swelling on right side of face, loosening of right upper molar teeth and right sided nasal obstruction and painless swelling over right side of palate. On examination there was 6 × 5 cm smooth diffuse swelling over right molar region extending 6 cm downwards from right infra orbital margin, and from right nasolabial angle to 5 cm lateral to zygoma. It was firm, non-tender and overlying skin was free. Mouth opening was restricted to two fingers. In the oral cavity there was a dome shaped swelling extending from right upper alveolus involving the hard palate, falling short of midline and covered with slough. There was fullness of right gingivobuccal sulcus and upper pre molar and molar teeth were missing. Nasal cavity revealed a smooth bulge on the right lateral wall pushing the structures medially. In the preliminary work up, X ray of the paranasal sinuses showed haziness of right maxillary sinus with irregular destruction of floor of maxillary sinus. An intraoral orthopantomogram (IOPA) showed a honeycomb appearance of the growth involving the right alveolus. Due to suspicion of malignancy, CT scan with contrast study of the paranasal sinuses was done. There was a heterogeneously enhancing soft tissue density mass lesion measuring 6.1 × 4 × 5.4 cms in the right maxillary sinus with associated erosion of anterior, posterior, superior and inferior walls of maxilla [Figure 1]. A total maxillectomy with complete excision of its soft tissue extension was done. The buccal pad of fat and jugulodigastric lymph nodes was also excised. All the specimens were sent for histopathological evaluation. The pathological diagnosis was confirmed as myofibrosarcoma of intermediate grade [Figure 2]. The right jugulodigastric lymph node and buccal pad of fat was free of tumour. Immunohistochemistry of the specimen was strong and diffusely positive for Vimentin, focally positive for smooth muscle actin (SMA) and negative for cytokeratin, desmin, EMA and CD 34. These are characterisitic features of myofibrosarcoma.,,, Patient underwent post op radiotherapy. 56 Grays of radiotherapy was given over six weeks. Regular follow up two years after completion of radiotherapy revealed well healed operated site with no recurrence [Figure 3]. There are only three cases of myofibrosarcoma primarily involving the maxilla reported in the literature., Treatment is aggressive wide local excision alone or a combination of excision plus radiotherapy and or chemotherapy., Review of pediatric case reports of myofibrosarcoma of head and neck reveals that chemotherapy and radiotherapy alone is ineffective, and aggressive surgical resection is needed., Out of three adult reported cases of myofobrosarcoma of this region- one had local spread in nine months, second had local recurrence in 24 months  and the third one had 14 months of symptom free period. We are reporting this case to bring out the importance of considering these malignancies as part of differential diagnosis for planning treatment and anticipating therapeutic outcome.
|Figure 1: Axial and Coronal CT images of heterogeneously enhancing soft tissue mass in right maxillary sinus|
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|Figure 2: Histopathological images, a (×20), b (×40) of highly cellular tumour of spindle cells. Immunohistochemistry slides c (×100) positive for vimentin and d (×100) positive for SMA|
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|Figure 3: Post radiotherapy picture showing well healed cavity with no recurrence|
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[Figure 1], [Figure 2], [Figure 3]