MTT is a subtype of malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation. Histologically, it is characterized by the presence of rhabdomyoblasts in a stromal background identical to that of an ordinary MPNST. This variant was first recognized as a cervical mass in a patient with neurofibromatosis 1 (NF1) by Masson1in 1932. The term Triton, introduced in 1973 by Woodruff et al,2refers to the Triton salamanders that grew supernumerary limbs with both bone and muscle after the implantation of severed sciatic nerves in their backs in Locatelli’s3experiment. The MTT is an uncommon and highly aggressive soft-tissue sarcoma, with fewer than 100 cases in the literature. Approximately one-third of the MTTs were found in the head and neck area, with 10 previously reported cases involving the sinonasal tract as the primary site.4,5
Pathology Quiz Case 1: Diagnosis. Arch Otolaryngol Head Neck Surg. 2010;136(9):931. doi:10.1001/archoto.2010.137-b
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