Gastric Stromal Sarcoma, Pulmonary Chondroma, and Extra-adrenal Paraganglioma (Carney Triad): Natural History, Adrenocortical Component, and Possible Familial Occurrence

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Objective

To investigate the natural history of the triad of gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma, a rare syndrome of unknown cause primarily affecting young women.

Methods

Mayo Clinic records, the world literature, and the author's files were searched for patients with all or 2 of the 3 tumors.

Results

Seventy-nine patients, 67 women and 12 men, were identified, 17 (22%) with the 3 tumors and 62 (78%) with 2 tumors. Forty-two (53%) had gastric and pulmonary tumors, the most common combination. The longest interval between detection of the first and second components was 26 years (mean, 8.4 years; median, 6 years). Follow-up ranged from 1 year to 49 years (mean, 20.6 years; median, 20 years). Sixty-four patients (81%) were alive, 19 (24%) apparently free of disease and 45 (57%) with residual or metastatic tumors. Thirty-two patients (41%) had 1 or more local recurrences of the gastric sarcoma; the longest interval to first recurrence was 36 years. Twenty-one survivors (27%) had hepatic metastatic gastric sarcoma with follow-up of 1 year to 25 years (mean, 9.3 years; median, 7 years). Fifteen patients (19%) were dead, 10 (13%) of whom died of the disorder. Ten patients (13%) had nonfunctioning adrenocortical tumors. Two patients each had a sibling with 1 component of the triad.

Conclusions

The triad is a chronic, persistent, and indolent disease. Benign adrenocortical tumors are a component of the condition. The disorder may be familial.

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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