title: A case of von Recklinghausen's disease with bilateral pheochromocytoma-malignant peripheral nerve sheath tumors of the adrenal and gastrointestinal autonomic nerve tumors.
abstract: A 48-year-old man with neurofibromatosis type 1 presented with chest pain, paroxysmal hypertension, tachycardia, and progressive respiratory insufficiency. Clinical investigation displayed calcified tumors in the anterior mediastinum and pararenal region. Histological examination at autopsy revealed composite tumors consisting of pheochromocytoma and malignant peripheral nerve sheath tumor (MPNST) at two sites: the left adrenal gland and the region surrounding the inferior vena cava, probably corresponding to the right adrenal gland. The MPNST component showed a varied histological appearance, including hyalinized bands with polygonal cells, a cartilaginous and myxoid stroma, a hemangiopericytomatous architecture, and a fibrosarcomatous structure, which suggested osteosarcoma, chondrosarcoma, angiosarcoma, and fibrosarcoma, respectively. In addition, based on the ultrastructural findings, the gastrointestinal tract was involved with mesenchymal tumors showing neurogenic differentiation. These lesions suggest the divergent cellular differentiation of neural crest-derived cells to mesenchymal elements as well as neuroectodermal neoplasms.
authors.affiliation: Department of Pathology, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Sakaguchi
authors.forename: K
authors.initials: K
authors.lastname: Sano
authors.forename: M
authors.initials: M
authors.lastname: Ito
authors.forename: T
authors.initials: T
authors.lastname: Baba
authors.forename: M
authors.initials: M
authors.lastname: Fukuzawa
authors.forename: M
authors.initials: M
authors.lastname: Hotchi
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000473:pathology* / Q000648:ultrastructure; D000311:Adrenal Glands / Q000294:innervation*; D001342:Autonomic Nervous System Diseases / Q000473:pathology; D004064:Digestive System / Q000294:innervation*; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D018317:Nerve Sheath Neoplasms / Q000473:pathology* / Q000648:ultrastructure; D009456:Neurofibromatosis 1 / Q000473:pathology*; D010524:Peripheral Nervous System Neoplasms / Q000473:pathology* / Q000648:ultrastructure; D010673:Pheochromocytoma / Q000473:pathology* / Q000648:ultrastructure