malignant Triton tumor
Definition
a malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differenciation
Localization (Examples)
rectum (#15827834#)
Synopsis
sporadic or in the setting of neurofibromatosis 1 (NF1)
scattered rhabdomyoblasts in a stroma identical to ordinary MPNST :
- usually rather mature, with abundant eosinophilic cytoplasm
- cross-striations
- immunohistochemistry : desmin, MyoD1 and myogenin positive
See also
malignant peripheral nerve sheath tumor (MPNST)
References
Daimaru Y, Hashimoto H, Enjoji M. Malignant "triton" tumors: a clinicopathologic and immunohistochemical study of nine cases. Hum Pathol. 1984 Aug;15(8):768-78. PMID: #6235165#