Carney complex is a rare inherited condition that causes spotty pigmentation of the skin and lining of the mouth, heart tumours called myxomas, and tumours of the hormone glands, skin and nerves. Children with it are offered regular checks under recommendations updated in 2025.
Autosomal dominant multiple neoplasia syndrome with cardiac, endocrine, cutaneous and neural tumours and pigmented lesions of the skin and mucosae, sharing mucosal lentiginosis and large-cell calcifying Sertoli cell tumours with Peutz-Jeghers syndrome (Stratakis, J Clin Endocrinol Metab 2001). PRKAR1A, the regulatory subunit 1-alpha of protein kinase A, was found mutated in about half of the kindreds known in 2001 and behaves as a tumour suppressor; a second locus maps to 2p16.
The AACR updated its paediatric surveillance recommendations for Carney complex, alongside the multiple endocrine neoplasias and hyperparathyroidism-jaw tumour syndrome, in 2025. Only the abstracts were read in this round, so no intervals are recorded here.
In plain words · PRKAR1A (cAMP-dependent protein kinase type I-alpha regulatory subunit) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as an oncogene driver, a tumour suppressor and a biomarker, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Adrenocortical carcinoma, Thyroid cancer, Renal cell carcinoma and 2 more.
Showing the target this term concerns: PRKAR1A.
Shares Invitae Multi-Cancer Panel, Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Invitae Multi-Cancer Panel, Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Sertoli cell tumour of the testis, Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Every source this page cites, grouped by who published it. Grouping is read from the web address, not assigned by hand.
A source that has moved or gone is reported by the weekly link check. /audit/