A rare case of malignant triton tumor in the cerebellopontine angle

Li Gong,Xiao-Yan Liu,Xiu-Juan Han,Li Yao,Shao-Jun Zhu,Wei Zhang,Miao Lan,Wen-Dong Zhang,Yan-Hong Li

doi:10.1186/1746-1596-7-43

Li Gong, Xiao-Yan Liu + Show 7 more

Open Access

https://doi.org/10.1186/1746-1596-7-43

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Journal: Diagnostic Pathology	Publication Date: Apr 19, 2012
Citations: 26	License type: cc-by

Affiliation: Air Force Medical University

Abstract

Malignant triton tumor (MTT) is defined as malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. Intracranial MTT is extremely rare, and only four cases have been reported in the literature. Here, we report a case of MTT occurring in the cerebellopontine angle, and describe its histopathological characteristics, immunohistochemical features, and prognosis.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1336227313684480

Highlights

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon type of sarcoma that arises from peripheral nerve sheaths
About 50 % of MPNSTs are known to develop into cases of neurofibromatosis type 1 [1]
Malignant triton tumor (MTT), which is a subtype of MPNST in which malignant Schwann cells coexist with malignant rhabdomyoblasts, was firstly reported by Masson et al in 1932 [2]

Summary

Introduction

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon type of sarcoma that arises from peripheral nerve sheaths. Both cell types showed negative staining for glial fibrillary acidic protein (GFAP), neuron specific enolase (NSE), HMB45, melanoma-pan, cytokeratin (CK), epithelial membrane antigen (EMA), alpha-fetoprotein (AFP), CD30, human chorion gonadotropin (HCG), SM-action, CD68, CD163, and CD34 We diagnosed this case as malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation, namely MTT. Woodruff et al [17] provided three diagnostic criteria for MTT: 1) the tumor originates from the surrounding nerve or develops into NF-1; 2) spindle cells are the main cell type in MPNST; 3) there are true neoplastic rhabdomyoblasts, but they are not due to involvement or metastasis of rhabdomyosarcoma in other positions. These studies will be important for the understanding of the pathophysiology of MTT

Conclusions

Brooks JSJ

Findings

16. Best PV