Gastric Stromal Sarcoma, Pulmonary Chondroma, and Extra-adrenal Paraganglioma (Carney Triad): Natural History, Adrenocortical Component, and Possible Familial Occurrence
Section snippets
Methods
Records of the Mayo Clinic, Rochester, Minn, the world literature, and the author's files were searched for patients with 2 or more of the following: (1) gastric leiomyoblastoma, gastric leiomyosarcoma, gastric epithelioid leiomyosarcoma, gastric stromal tumor, gastric plexosarcoma, or gastric autonomic nerve tumor; (2) pulmonary chondroma, pulmonary hamartoma, or pulmonary osteochondroma; and (3) extra-adrenal paraganglioma or pheochromocytoma (adrenal paraganglioma). Seventy-nine cases were
Patients
The patients were 67 women (85%) and 12 men (15%) from all continents except Africa. None was black (1 patient was erroneously reported as such43). None was related. Forty-one patients had 103 children (maximum, 9) after onset of the disorder.
Age
Age at onset (designated as the date of his to log ical diagnosis of the first component) ranged from 7 to 48 years (Figure 1). Onset was before the age of 30 years in 65 patients (82%) and after the age of 40 years in 5 (6%). Table 1 displays the
Discussion
This study provides insight into the natural history of an unusual disorder, the triad of gastric stromal sarcoma, pulmonary chondroma, and paraganglioma, and shows that it is chronic, persistent, and indolent. Thus far, only 10 patients (13%) have died of causes directly attributable to the disorder, but it contributed to the deaths of 2 others, and the number of deaths doubtless will increase because 28 surviving patients (35%) have inoperable or metastatic gastric stromal sarcoma or residual
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