Malignant triton tumor of the gluteal region in a patient unaffected by neurofibromatosis: A case report

Abstract

Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) made up of both malignant schwannoma cells and malignant rhabdomyoblasts.A 26-years-old male patient was admitted with an asymptomatic gluteal mass. Magnetic resonance imaging showed heterogeneous soft tissue mass and he underwent open biopsy. Malignant peripheral nerve sheath tumor was diagnosed. He was given adjuvant chemotherapy following the removal of the tumor with hip disarticulation. The tumor was diagnosed as “malignant triton tumor” based on pathological examination including immunohistochemical studies. There were no signs of metastasis but recurrence was observed at 9 months follow up.MTT is usually associated with Neurofibromatosis 1 and located in head, neck region. In this case sporadic involvement of gluteal region and aggressive behavior of the lesion despite radical surgery was demonstrated.

Orbital Involvement by Biphenotypic Sinonasal Sarcoma With a Literature Review

“…27,28 Another composite neoplasm is the so-called malignant triton tumor (MTT) of peripheral nerve sheath. [31][32][33][34][35][36][37] It is likely that the earlier characterization of BSNS was prevented by cases that were buried among MTT. The tumor bears this designation based on experiments on the triton salamander in which implantation of the sciatic nerve to its back produces striated muscle, nervous tissue, and bone representing a supernumerary limb.…”

A case report of giant malignant schwannoma of the sciatic nerve associated with neurofibromatosis-1: A CARE-compliant article

“…However, people with NF-1 have a 10% chance of developing malignant peripheral nerve sheath tumors (MPNST), which often grow within a neurofibroma. [4,5] The vast majority of MPNSTs are aggressive, high-grade sarcomas with a significant risk of local recurrence (40%-65%) and distant metastasis. The lung is the most common site of metastasis, followed by bone, brain, and liver.…”

“…However, people with NF-1 have a 10% chance of developing malignant peripheral nerve sheath tumors (MPNST), which often grow within a neurofibroma. [4,5]…”

Onkologische Sicherheit und Funktion im Fokus: Primäre Defektrekonstruktion eines malignen Triton-Tumors der Nervenscheide des N. ischiadicus durch freie myokutane Lappenplastik

“…Maligne Triton Tumoren (MTT) sind eine seltene Form von malignen Tumoren peripherer Nervenscheiden [1,2], die aus Schwann´schen Zellen entstehen und gleichzeitig Rhabdomyoblasten enthalten [2,3]. Erstmalig 1932 beschrieben sind weltweit seither ca.…”

“…MTT weisen eine schlechte Prognose mit einem 5-Jahres-Überleben von ca. 12,5 % auf [3]. Je nach Befund können MTT einzeitig mit Resektion des Tumors und Sofortrekonstruktion des Weichteildefektes oder zweizeitig mit sekundärer Rekonstruktion therapiert werden.…”