Pleomorphic Sarcoma of the Head and Neck Region

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Pleomorphic Sarcoma or Malignant Fibrous Histiocytoma is commonest soft tissue sarcoma but is rare in Head and neck. Commonest site in Head and neck being Nasal cavity and para nasal sinuses. It is more common in male. Tumor is diagnosed histologically consisting of both histiocytes and fibroblast cells. Pleomorphic Sarcoma is classified into primary and secondary types with mean age of presentation between 6th and 7th decade. The treatment of choice for this tumor is surgery with clear margins and adjuvant Radiotherapy. Prognosis remains poor even after surgery with local and distant metastasis seen commonly.

Soft Tissue Sarcoma encompasses (STS) a broad array of malignant tumors that are derived from cells of mesenchymal origin at any anatomical site. The originating tissue is diverse that includes bones, cartilage, muscular, fibrous, vascular, fatty and neural tissue.  Of all the soft tissue sarcomas only 5-20% occurs in the head and neck region. The most common STS of the head and neck region are Rhabdomyosarcoma followed by Malignant fibrous Histiocytoma, Fibrosarcoma and Neuro-fibrosarcoma. The incidence of MFH seems to be the highest among various types of adult malignant soft tissue sarcomas.  Pleomorphic Sarcoma or Malignant Fibrous Histiocytoma (MFH) is a rare primitive mesenchymal tumor showing both fibroblastic and histiocytic differentiation. We report a case of Pleomorphic Sarcoma/ MFH and the review of literature in relation to Pleomorphic Sarcoma.

A 40 year old male presented with the chief complaint of gradually progressive, painless, irregular swelling over the right side of the face for the past 6 months. On examination it was a 10 x 8 x 8 cm large firm mass of the right parotid region with no evidence of intra oral lesion. There were no palpable neck glands. On radiological evaluation, CT scan revealed a 98 x 113x 106 mm enhancing soft tissue lesion over the right parotid region. Lesion showed area of necrosis within. Lesion reaches up to the skin and involves the masseter muscle. Right submandibular gland is not seen separately from the lesion. Presence of few lymph nodes at level IA, II, III with the largest at IA measuring 20 x17 mm. No evidence of mandible erosion.