Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor).
Malignant peripheral nerve sheath tumors arise from Schwann cells or within existing neurofibromas and have a strong association with type 1 neurofibromatosis. These tumors are histologically diverse and may contain malignant areas of divergent mesenchymal differentiation, the most common of which is skeletal muscle (rhabdomyosarcoma). Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation is also known as malignant triton tumor. Malignant triton tumor has a worse prognosis than classic malignant peripheral nerve sheath tumor does, and the correct diagnosis requires attention to the clinical history and knowledge of the complexities regarding its differential diagnosis. In this review we discuss the clinical, histopathological, immunohistochemical, and prognostic features of this rare neoplasm.
Stasik
Christopher J
CJ
Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, 3901 Rainbow Blvd, Kansas City, KS 66160-7410, USA. cstasik@kumc.edu
Tawfik
Ossama
O
D014408:Biomarkers, Tumor / Q000032:analysis; D018959:CD57 Antigens / Q000032:analysis; D006801:Humans; D007150:Immunohistochemistry; D004676:Myelin Basic Protein / Q000032:analysis; D018317:Nerve Sheath Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D012208:Rhabdomyosarcoma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D009418:S100 Proteins / Q000032:analysis; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery
D014408:Biomarkers, Tumor; D018959:CD57 Antigens; D004676:Myelin Basic Protein; D009418:S100 Proteins