Malignant peripheral nerve sheath tumour of parapharyngeal space

ABSTRACT

Sarcomas account for only 1% of all head and neck primary cancers. Of all soft tissue sarcomas only 5% are neurogenic sarcomas [malignant peripheral nerve sheath tumour] and upto 20% of these lesions occur in the head and neck region.[1,2] About 50% of these tumours are associated with neurofibromatosis type I. Sporadic cases usually occur during the 4[th], 5[th] or 6[th] decades of life with equal sex predisposition[1]. Painless enlarging neck mass with or without neuropathy is the usual presenting feature [3]. Differentiating neurofibrosarcoma from other spindle cell sarcomas, e.g., malignant fibrous histiocytoma, fibrosarcoma or leiomyosarcoma can be very difficult without immunohistochemistry. Wide local excision is the treatment of choice [3, 4] but prognosis is poor for high grade tumours, tumours larger than 5 cm in diameter, if surgical margins are not free from tumour and in patients with neurofibromatosis[1, 4]