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Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence.
- Source :
-
Mayo Clinic proceedings [Mayo Clin Proc] 1999 Jun; Vol. 74 (6), pp. 543-52. - Publication Year :
- 1999
-
Abstract
- 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.<br />Methods: Mayo Clinic records, the world literature, and the author's files were searched for patients with all or 2 of the 3 tumors.<br />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 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.<br />Conclusions: The triad is a chronic, persistent, and indolent disease. Benign adrenocortical tumors are a component of the condition. The disorder may be familial.
- Subjects :
- Adolescent
Adult
Cause of Death
Child
Female
Follow-Up Studies
Humans
Male
Middle Aged
Adrenal Cortex Neoplasms diagnosis
Adrenal Cortex Neoplasms genetics
Adrenal Cortex Neoplasms therapy
Chondroma diagnosis
Chondroma genetics
Chondroma therapy
Lung Neoplasms diagnosis
Lung Neoplasms genetics
Lung Neoplasms therapy
Neoplasms, Multiple Primary diagnosis
Neoplasms, Multiple Primary genetics
Neoplasms, Multiple Primary therapy
Paraganglioma diagnosis
Paraganglioma genetics
Paraganglioma therapy
Sarcoma diagnosis
Sarcoma genetics
Sarcoma therapy
Stomach Neoplasms diagnosis
Stomach Neoplasms genetics
Stomach Neoplasms therapy
Subjects
Details
- Language :
- English
- ISSN :
- 0025-6196
- Volume :
- 74
- Issue :
- 6
- Database :
- MEDLINE
- Journal :
- Mayo Clinic proceedings
- Publication Type :
- Academic Journal
- Accession number :
- 10377927
- Full Text :
- https://doi.org/10.4065/74.6.543