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MALIGNANT TRITON TUMOUR OF BRACHIAL PLEXUS

YOGAMBAL YOGAMBAL

Abstract


Abstract : 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.
Keyword :Malignant peripheral nerve sheath tumour, Malignant
triton tumour(MTT), Neurofibromatosis 1, Brachial plexus


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References


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An Initiative of The Tamil Nadu Dr M.G.R. Medical University