Malignant nerve sheath tumor containing endocrine cells.
A malignant nerve sheath tumor occurred in the thigh of a 29-year-old man who had the stigmata of von Recklinghausen's neurofibromatosis. Chondroid foci, rhabdomyoblasts, and mucus-containing acini were identified in the tumor. Argyrophil cells and somatostatin-immunoreactive cells were present in acinar epithelium. Endocrine type granules were found in epithelial cells on ultrastructural examination. This is an example of a so-called "glandular schwannoma" and is unique in that it contained somatostatin-immunoreactive cells. A neural crest derivation is suggested for this complex tumor.
D000328:Adult; D004848:Epithelium / Q000473:pathology; D006651:Histocytochemistry; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D009442:Neurilemmoma / Q000378:metabolism / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000378:metabolism / Q000473:pathology*; D013004:Somatostatin / Q000378:metabolism; D013848:Thigh
D013004:Somatostatin