Granular cell tumors are relatively uncommon neoplasms of unknown histogenesis which arise in different regions of the body. In the head and neck, these are found most frequently in the tongue. Although the reported incidence of local recurrence after excision or multicentricity is high, these are believed to be benign. Only a few malignant variations have been reported.
This presentation is a case of malignant granular cell tumor of the right side of the neck in a 25-year-old man. The tumor increased rapidly in size and showed local recurrence and metastases to the pancreas, skin, and lymphnodes of the mediastinum. In spite of extensive surgery and chemotherapy, the patient died 268 days after admission. The histology, multicentricity, malignant variation, and therapy of these tumors are discussed.