19. CARNEY COMPLEX CASE REPORT AND LITERATURE REVIEW

Van Hung Dung1,2, Nguyen Van Phuoc1, Dang Huu Danh1
1 Ho Chi Minh City Heart Institute
2 Pham Ngoc Thach University of Medicine

Main Article Content

Abstract

Carney complex is a dominant genetic disease associated with many neoplasm in multiple endocrine glands such as the thyroid, pituitary, and adrenal glands and tumors outside the endocrine glands. Typically, patients have many brown pigmented spots on the face and many tumors in the heart, breast, adrenal glands... The most common heart tumor is atrial myxoma and can recur many times. Patients also have symptoms of Cushing's syndrome in many cases. Confirmed diagnosis is often missed. We report a typical case and review the literature on this disease.

Article Details

References

[1] KM Lowe, WF Young, C Lyssikatos C, CA Stratakis, JA Carney. Cushing Syndrome in Carney Complex: Clinical, Pathologic, and Molecular Genetic Findings in the 17 Affected Mayo Clinic Patients. Am J Surg Pathol. 2017 Feb;41(2):171-181
[2] C.A Stratakis, L.S. Kirschner, and J. Aidan Carney. Clinical and Molecular Features of the Carney Complex:Diagnostic Criteria and Recommendations for Patient Evaluation. J Clin Endocrinol Metab 2001; 86(9):4041–4046
[3] MR. Vindhyal, G Elshimy, N Haq, G Elhomsy. Carney Complex. StatPearls-NIH, Mar 2024.
[4] J Bertherat, A Horvath, L Groussin, S Grabar, S Boikos, et al. Mutations in regulatory subunit type 1A of cyclic adenosine 5′-monophosphate-dependent protein kinase (PRKAR1A): phenotype analysis in 353 patients and 80 different genotypes. J Clin Endocrinol Metab 2009 Jun; 94(6):2085–91.
[5] B. Harbeck, J. Flitsch, I. Kreitschmann-Andermah. Carney complex- why thorough medical history taking is so important - report of three cases and review of the literature. Endocrine 2023; 80:20–28. https://doi.org/10.1007/s12020-022-03209-2.
[6] J Vargas-Barrón, G Vargas-Alarcón, F-J Roldán, C Vázquez-Antona, Z.V Ortiz, et al. Cardiac Myxomas and the Carney Complex. Rev Esp Cardiol 2008;61(11):1205-9
[7] S Yokoyama, K Nagao, A Higashida, M Aoki, S Yamashita, et al. Diagnosis of Carney complex following multiple recurrent cardiac myxomas. General Thoracic and Cardiovascular Surgery 2022; 70:87–91. https://doi.org/10.1007/s11748-021-01719-w
[8] C Bireta, AF Popov, H Schotola, B Trethowan, M Friedrich, M El-Mehsen et al. Carney-Complex: Multiple resections of recurrent cardiac myxoma. J Cardiothorac Surg 2011, 6:12
[9] A. Nagesh Shetty Roy, M Radin, D Sarabi, E Shaoulian. Familial Recurrent Atrial Myxoma: Carney’s Complex. Clin. Cardiol. 2011; 34, 2, 83–86