Carney triad

Classification according to ICD-10
D44.8	Involvement of several endocrine glands
ICD-10 online (WHO version 2019)

The Carney triad ( English Carney's syndrome ) is a very rare association of gastrointestinal stromal tumor (GIST), paragangliomas and chondromas of the lungs and is classified as multiple endocrine neoplasms .

The name refers to the first author of the first description from 1977 by the American pathologist J. Aiden Carney .

The disease is not to be confused with

• the Carney Stratakis syndrome , a congenital disease with gastrointestinal stromal tumors (GIST) and paragangliomas .
• the Carney complex (or Carney syndrome ), a complex of myxomas, skin spots, and overactive hormones.

distribution

The frequency is given as less than 1 in 1,000,000, a hereditary component is suspected, but so far nothing is known about the cause. The disease mainly affects women.

Clinical manifestations

In addition to the diseases of the triad, pheochromocytomas , leiomyomas of the esophagus and adenomas of the adrenal cortex are common.

diagnosis

The diagnosis is based on the findings of the clinical examination and medical imaging .

Differential diagnosis

The most important differential diagnosis is Carney-Stratakis syndrome.

therapy

The treatment is directed against the respective tumors, as described in their articles.

Individual evidence

1. ↑ ^{a b} Carney Trias. In: Orphanet (Rare Disease Database).
2. ^ Who named it Carney's syndrome
3. ^ Who named it Carney
4. ↑ JA Carney, SG Sheps, VL Go, H. Gordon: The triad of gastric leiomyosarcoma, functioning extra-adrenal paraganglioma and pulmonary chondroma. In: The New England Journal of Medicine . Vol. 296, No. 26, June 1977, pp. 1517-1518, doi: 10.1056 / NEJM197706302962609 , PMID 865533 .
5. ^ Carney Triassic. In: Orphanet (Rare Disease Database).
6. ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
7. ↑ SA Boikos, P. Xekouki, E. Fumagalli, FR Faucz, M. Raygada, E. Szarek, E. Ball, SY Kim, M. Miettinen, LJ Helman, JA Carney, K. Pacak, CA Stratakis: Carney triad can be (rarely) associated with germline succinate dehydrogenase defects. In: European Journal of Human Genetics  : EJHG. [Electronic publication before printing] July 2015, doi: 10.1038 / ejhg.2015.142 , PMID 26173966 .
8. ↑ Carney Triad.  In: Online Mendelian Inheritance in Man . (English)
9. ↑ CA Stratakis, JA Carney: The triad of paragangliomas, gastric stromal tumors and pulmonary chondromas (Carney triad), and the dyad of paragangliomas and gastric stromal sarcomas (Carney-Stratakis syndrome): molecular genetics and clinical implications. In: Journal of internal medicine. Vol. 266, No. 1, July 2009, pp. 43-52, doi: 10.1111 / j.1365-2796.2009.02110.x , PMID 19522824 , PMC 3129547 (free full text) (review).
10. ↑ GZ Papadakis, NJ Patronas, CC Chen, JA Carney, CA Stratakis: Combined PET / CT by 18F-FDOPA, 18F-FDA, 18F-FDG, and MRI correlation on a patient with Carney triad. In: Clinical Nuclear Medicine. Vol. 40, No. 1, January 2015, pp. 70-72, doi: 10.1097 / RLU.0000000000000616 , PMID 25423347 , PMC 4260990 (free full text).

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !