MALIGNANT TRITON TUMOUR OF BRACHIAL PLEXUS

Malignant peripheral nerve sheath tumour with rhabdomyoblastic differentiation is referred to as malignant Triton tumour. Malignant peripheral nerve sheath tumours arise from Schwann cells or within existing neurofibromas and have a strong association with type 1 neurofibromatosis. Tumours associated with NF1 have a particularly poor prognosis compared with those occurring on a sporadic basis. Malignant Triton tumour has a worse prognosis than classic malignant peripheral nerve sheath tumour and the correct diagnosis requires attention to the clinical history, histological features including immunohistochemistry in some cases extensive sampling. We report a case of malignant Triton tumour arising in the brachial plexus of a 47-year-old woman with neurofibromatosis type 1. The patient underwent complete excision of the tumour. During follow up patient had recurrence of the tumor after 5 months and died in 1 year. In this report, we discuss the clinical, histopathological, immunohistochemical and prognostic features of this rare neoplasm.