Unusual histological variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation

Abstract

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.

Intradural extramedullary granular cell tumour in a cat

“…Our positivity may, once again, confirm the uncertainty about the origin of GCTs, or reflect a rhabdoid differentiation of the tumour. This latter type of differentiation has already been reported in human peripheral nerve sheath tumours (Li et al 2015, Shete et al 2015.…”

Malignant Peripheral Nerve Sheath Tumor With Osseous Heterologous Differentiation in Uncommon Locations (Heart and Retropharynx)

“…Divergent differentiation is a well-known phenomenon in MPNSTs, and heterologous elements, such as epithelial (glandular) or mesenchymal (skeletal muscle, bone, cartilage, and blood vessels), are present in approximately 15% of tumors. 1,2,5,6,11,13,17-19 The most common divergent line of differentiation is toward skeletal muscle (Triton tumor). 1,2,5,6,17-19 Malignant Triton tumor has a worse prognosis than classical MPNST, in contrast to the other forms of heterologous differentiation (including epithelial), which are not thought to change prognosis.…”

“…1,2,5,6,11,13,17-19 The most common divergent line of differentiation is toward skeletal muscle (Triton tumor). 1,2,5,6,17-19 Malignant Triton tumor has a worse prognosis than classical MPNST, in contrast to the other forms of heterologous differentiation (including epithelial), which are not thought to change prognosis. 1,2,5,6,17-19…”

“…1,2,5,6,17-19 Malignant Triton tumor has a worse prognosis than classical MPNST, in contrast to the other forms of heterologous differentiation (including epithelial), which are not thought to change prognosis. 1,2,5,6,17-19…”

“…This divergent differentiation makes it difficult to distinguish MPNSTs from other soft tissue or bone sarcomas. 1,2,6,12-25…”