Anila Chughtai, Mohammad Fayyaz Anwar, Saira Rathore, Samina Zaman, Omar Chughtai, Akhtar Sohail Chughtai.
Malignant triton tumor of thigh: a case report and review of literature.
Pak J Pathol Jan ;28(3):141-4.

Malignant triton tumor (MTT) is a tumor arising from Schwann cells with divergent rhabdomyoblastic differentiation. It is a relatively rare and aggressive variant of malignant peripheral nerve sheath tumor (MPNST). We present a case of a 33 years old male with thigh swelling. Surgical excision was done followed by histopathological and immunohistochemical (IHC) workup which revealed a tumor showing two types of cell populations including pleomorphic spindle cells and large cells with pleomorphic eccentric nuclei. Spindle cells showed positivity for S-100 IHC stain confirming their neural origin while large cells with eccentric nuclei showed positivity for desmin & myogenin IHC stains confirming their rhabdomyoblastic origin. Hence, the diagnosis of MTT was made. A 5 years survival rate for MTT is 5-15% compared to 50-60% for MPNST. Considering it is a rare entity with aggressive clinical behavior and poor prognosis, correct diagnosis is essential that can be achieved by careful histological and IHC evaluation.

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