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Malignant triton tumor: a rare case.

Prasad K Shetty1, Sameer V Baliga1, Kopparthi Balaiah1

  • 1Department of Pathology and Surgical, Bhagwan Mahaveer Jain Hospital, Bangalore, Karnataka 560052 India.

The Indian Journal of Surgery
|January 16, 2014
PubMed
Summary

Malignant triton tumor (MTT), a rare peripheral nerve sheath tumor, was diagnosed in a 34-year-old man with neurofibromatosis 1. This aggressive cancer, characterized by rhabdomyoblastic differentiation, has a poor prognosis.

Keywords:
Malignant peripheral nerve sheath tumor (MPNST)Malignant triton tumor (MTT)Neurofibromatosis-1 (NF-1)S-100 protein

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Area of Science:

  • Oncology
  • Pathology
  • Genetics

Background:

  • Malignant triton tumor (MTT) is a rare peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation.
  • Fewer than 100 cases are reported, typically affecting adult patients with neurofibromatosis 1 (NF-1).
  • MTT exhibits aggressive biological behavior and a poor prognosis.

Purpose of the Study:

  • To report an extremely rare case of Malignant Triton Tumor in a young adult male with NF-1.
  • To highlight the diagnostic challenges and histopathological features of MTT.
  • To emphasize the aggressive nature and poor prognosis associated with this rare tumor.

Main Methods:

  • Case report of a 34-year-old male patient with NF-1 presenting with a medial arm mass.
  • Histopathological examination revealing rhabdomyoblasts and malignant Schwann cells.
  • Immunohistochemical analysis using S-100 protein and myogenin.

Main Results:

  • Diagnosis of Malignant Triton Tumor confirmed through histopathology and immunostaining.
  • Tumor arose from a peripheral nerve, consistent with MPNST.
  • Patient presented with a mass on the medial side of the arm.

Conclusions:

  • Malignant Triton Tumor is an extremely rare and aggressive neoplasm.
  • Early diagnosis and understanding of its behavior are crucial for patient management.
  • This case underscores the importance of recognizing MTT in patients with NF-1.