Malignant Peripheral Nerve Sheath Tumor With Rhabdomyosarcomatous and Glandular Elements: Rare Epithelial Differentiation in a Triton Tumor

We describe a case occurring in the mediastinum (between the ascending aorta and the main pulmonary artery) of a 40-year-old man with neurofibromatosis type-1. Despite treatment, the tumor metastasized to the right frontal lobe 16 months after diagnosis. Histologically, the primary neoplasm comprised cellular fascicles of atypical spindle cells, with discrete foci of well-formed or more primitive glandular epithelial structures, while the metastases comprised spindle cell sarcoma, including rhabdomyosarcoma, without epithelial elements. We review and discuss the literature on these little characterized neoplasms, including histology and clinical features, adding to the spectrum of documented cases of divergent epithelial differentiation in Triton tumor.
Source: International Journal of Surgical Pathology - Category: Pathology Authors: Tags: Case Reports Source Type: research