Published online Apr 16, 2015. doi: 10.12998/wjcc.v3.i4.389
Peer-review started: October 21, 2014
First decision: December 12, 2014
Revised: January 4, 2015
Accepted: February 4, 2015
Article in press: February 9, 2015
Published online: April 16, 2015
Malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation is called as malignant triton tumor (MTT). It is highly aggressive soft tissue tumor with higher local recurrence rate. MTT has poor prognosis than MPNST. MTT seems to be more aggressive in patients with neurofibromatosis (NF-1). We herein, reporting an interesting case of 55 years male with multiple neurofibromas all over the body since 30 years and multiple café-au-lait spots, diagnosed as NF-1. Since 6 years, he had an enlarged mass in left thigh. Wide excision of mass was done. On histopathological examination revealed the diagnosis of MTT and diagnosis of which was confirmed on immunohistochemistry.
Core tip: Meticulous histopathological examination along with immunohistochemistry is the mainstay to arrive at such rare histological diagnosis. The surgical pathologist should keep in mind such rare entity while dealing with such kind of patients.