Published in:
01-06-2013 | Case Report
Malignant Triton Tumor: A Rare Case
Authors:
Prasad K. Shetty, Sameer V. Baliga, Kopparthi Balaiah
Published in:
Indian Journal of Surgery
|
Special Issue 1/2013
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Abstract
Malignant triton tumor (MTT) is a malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation. This rare tumor, with fewer than 100 cases reported in the literature, generally affects adult patients with neurofibromatosis 1 (NF-1). We report such a case in a 34-year-old man with NF-1 who presented with a mass over the medial side of the arm. Histopathologically finding of rhabdomyoblasts among malignant Schwann cells in a tumor arising from a peripheral nerve supported by immunostaining with S-100 protein and myogenin confirmed the diagnosis. MTT has a poor prognosis owing to its aggressive biological behavior. The fact that this tumor is extremely rare has prompted us to report this case.