Malignant Triton Tumour: Case report

Sandeep V Sandeep V,Rajat Kumar,Smita K Smita K,S S Hiremath S S Hiremath

doi:10.21276/apalm.2445

Abstract

Malignant triton tumours (MTTs) are a rare subtype of malignant peripheral nerve sheath tumour (MPNST) showing rhabdomyoblastic differentiation, which have no treatment consensus and a poor prognosis. This case report presents case of a 71yrs/ male with swelling in Right Hand over one month. Patient had same swelling 2yrs back which was diagnosed as Peripheral Nerve Sheath Tumour on histopathology following which he underwent surgery followed by radiotherapy. Swelling reappeared at same site after 3 months. FNAC of swelling shows large spindle cells having elongated buckled nuclei, pointed end, with varying nucleus size and coarse nuclear chromatin. Few cells are showing Rabdomyoblastic differentiation.

Highlights

Malignant peripheral nerve sheath tumour (MPNST) accounts for about 5–10 % of all soft tissue sarcomas 1
Malignant triton tumour (MTT) is a subtype of MPNST characterized by the presence of rhabdomyosarcomatous elements in a background of schwannoma cells and constitutes about 5 % of all MPNSTs and follow a aggressive course 2
MTTs are identified by focal evidence of skeletal muscle differentiation within the MPNSTs

Summary

Introduction

Malignant peripheral nerve sheath tumour (MPNST) accounts for about 5–10 % of all soft tissue sarcomas 1. Malignant peripheral nerve sheath tumours (MPNSTs) are believed to arise from Schwann cells or nearby cells with perineural differentiation. Patient 71yrs/ Male having two (2) swelling in Right Hand over last 2 month. He had same swelling 2yrs back for which biopsy was done and it was diagnosed as Peripheral Nerve Sheath Tumour on histopathology. FNAC of swelling shows large spindle cells having elongated buckled nuclei, pointed end, with varying nucleus size and coarse nuclear chromatin. Immunohistochemical stain showed malignant cells are strongly positive for S-100 protein (Fig:[5]).

Discussion

Conclusion

McConnell YJ and Giacomantonio CA

Findings

Masson P