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Gastric Autonomic Nerve (GAN) Tumor and Extra-adrenal Paraganglioma in Carneyʼs Triad

1987; Lippincott Williams & Wilkins; Volume: 205; Issue: 3 Linguagem: Inglês

10.1097/00000658-198703000-00001

1528-1140

Bartholomew J. Tortella, Jeffrey B. Matthews, Donald A. Antonioli, Ann M. Dvořàk, William Silen,

Neuroendocrine Tumor Research Advances

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 leiomyo- sarcoma 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.