Gastric autonomic nerve (GAN) tumor and extra-adrenal paraganglioma in Carney's triad. A common origin.
Carney's Triad comprises a triad of neoplasms: gastric stromal tumor, extra-adrenal paraganglioma (usually functional), and pulmonary chondroma. At least two of these are needed for the presumptive diagnosis of the Triad. This report presents a patient who had resected a gastric tumor and nonfunctional extra-adrenal paraganglioma. The gastric tumor resembled a gastric leiomyosarcoma by light microscopy, but electron microscopy revealed it to be a gastric autonomic nerve (GAN) tumor. Based on this evidence it appears that both the gastric lesions and the paragangliomata of Carney's Triad are tumors of the autonomic nervous system. Thus, the Triad may be a disorder of the autonomic nervous system rather than a multiple endocrine neoplasia syndrome or multiple hamartoma syndrome.
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000601:surgery; D006801:Humans; D008297:Male; D009378:Neoplasms, Multiple Primary; D010235:Paraganglioma / Q000473:pathology / Q000601:surgery*; D010524:Peripheral Nervous System Neoplasms / Q000473:pathology / Q000601:surgery*; D012186:Retroperitoneal Neoplasms / Q000473:pathology / Q000601:surgery*; D013274:Stomach Neoplasms / Q000473:pathology / Q000601:surgery*; D013577:Syndrome