Neurogenic sarcoma of the head and neck

We discuss our experience in the diagnosis and management of seven cases of neurogenic sarcomas of the head and neck. These uncommon tumors, which affect all ages, arise most frequently from the brachial plexus, sympathetic chain, and the cranial nerves or their branches. An enlarging mass is the most common initially appearing symptom. Diagnosis may be difficult and rests heavily on gross evidence of a relationship between the tumor and its nerve of origin. Histologically, the lesions are composed of spindle cells with varying degrees of pleomorphism in a pattern that is frequently undistinguishable from fibrosarcomas. Ultrastructural studies may be helpful in establishing the neurogenic origin of these tumors. Neurogenic sarcoma of the head and neck has an extremely poor prognosis. Surgery remains the cornerstone of treatment, although radiotherapy is important for palliation.