Synonyms
Malignant peripheral nerve sheath tumor with divergent differentiation; Malignant schwannoma with rhabdomyoblastic differentiation; MTT
Definition
Malignant triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor (MPNST) that shows rhabdomyoblastic differentiation. While MPNSTs are known to be capable of showing divergent differentiation in the form of bone, cartilage, skeletal muscle, or epithelial tissues, only those containing rhabdomyoblastic elements are designated as “malignant triton tumor.” The name stems from the observation that a certain type of salamander, the triton salamander, is able to regenerate limbs, including muscle components, from implanted nerve roots. MPNST as a group accounts for 5–10% of all soft tissue sarcomas and arise either in association with peripheral nerves or within a preexisting neurofibroma. A large percentage of MPNSTs occur in patients with neurofibromatosis type 1 (NF1) (40–50%) or after irradiation (8%), but...
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References and Further Reading
Brooks, J. S., Freeman, M., & Enterline, H. T. (1985). Malignant “Triton” tumors. Natural history and immunohistochemistry of nine new cases with literature review. Cancer, 55, 2543–2549.
Bura, M., Musani, V., Cretnik, M., Botica, I., & Levanat, S. (2008). Hedgehog-patched pathway aberrations in a malignant triton tumor case study. Oncology Reports, 2008(20), 347–352.
Ducatman, B. S., & Scheithauer, B. W. (1984). Malignant peripheral nerve sheath tumors with divergent differentiation. Cancer, 1984(54), 1049–1057.
McConnell, Y. J., & Giacomantonio, C. A. (2012). Malignant triton tumors – Complete surgical resection and adjuvant radiotherapy associated with improved survival. Journal of Surgical Oncology, 106, 51–56.
Ren, W., Xu, X., Yan, J., Qian, X., & Liu, B. (2014). Malignant triton tumor of the anterior mediastinum: A case report. Oncology Letters, 7, 807–810.
Stasik, C. J., & Tawfik, O. (2006). Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor). Archives of Pathology and Laboratory Medicine, 130, 1878–1881.
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Weissferdt, A., Moran, C.A. (2018). Mediastinal Malignant Triton Tumor. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_32
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DOI: https://doi.org/10.1007/978-3-319-66796-6_32
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