A rare case of intracranial malignant triton tumor arising in the middle cranial fossa: a case report and review of the literature

Abstract

We describe a rare case of intracranial malignant triton tumor (MTT) arising in the middle cranial fossa in a 74-yearold female patient who had previously been exposed to radiation in the Chernobyl disaster. The patient underwent a surgical subtotal removal of the mass and radiation therapy, but the progression-free survival was only 2.5 months and death occurred four months after the onset of symptoms. MTTs are rare aggressive tumors arising from the nerve sheath showing rhabdomyosarcomatous differentiation and associated with a poor prognosis. The intracranial location is very rare, and only 10 cases, including the present report, have been described so far. Among intracranial MTTs, the cerebellopontine angle is the most common location. Neurofibromatosis type 1 (NF-1) and radiation exposure are risk factors as for MTTs located in other sites. The gold standard therapy is surgical excision followed by radiation therapy, but the prognosis is usually very poor.

Neurofibromatosis in Children: Actually and Perspectives

“…MTTs represent a rare subtype of MPNSTs that upon inspection are sturdy, large, grayish tan neoplasms with intrinsic zones of hemorrhage and necrosis [ 8 , 9 , 10 , 11 ]. They are very rare, since MPNSTs comprise between 5% and 10% of all soft tissue sarcomas, while MTTs represent less than 10% of all MPSNTs .…”

“…Chronic lymphocytic leukemia and diffuse B cell lymphomas may also occur in these patients, although these associations are exceptionally rare [ 6 , 7 ]. Other cancers more commonly encountered in patients with NF1 include the malignant triton tumors (MTTs), which are a rare variant of MPNSTs [ 8 , 9 , 10 , 11 ]. The malignant transformation of spinal low-grade astrocytomas and the appearance of cerebral glioblastomas associated with NF-1 have also been documented [ 12 , 13 ].…”

Elbow flexion reconstruction after arm-sparing excision for high-grade triton sarcoma: a case report

“…We believe that when surgical reconstruction is possible downstream, neo-chemotherapy and neo-radiotherapy are justified and optimal in the conservative treatment of these high-grade STSs. The authors of the few publications concerning MTT have different recommendations for radiochemotherapy and no optimal strategy has been determined [41][42][43]. In fact, given the rarity of MTTs, no large-scale trials have been conducted to assess the appropriateness of adjuvant therapy.…”

Retroperitoneal malignant triton tumor in an adolescent with Neurofibromatosis type 1

“…Up to 8% of reported cases have been related to previous radiation (4)(5)(6). Other unusual locations such as intracranial fossa, spinal cord, mediastinum, uterus or epididymis have been described in the literature (17,(21)(22)(23)(24)(25)(26)(27)(28)(29). Our patient presented with a huge retroperitoneal mass, which is an uncommon finding with less than 20 similar cases reported to date (30)(31)(32)(33)(34)(35).…”