# Carney complex

Source: https://onco.cc/terms/carney-complex/  
OnCo record `carney-complex` (Term). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Summary

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.

## Fields

- Kind: Term
- Last checked: 2026-11-04
- Also known as: PRKAR1A Carney complex
- Tags: gap-fill; hereditary

## Notes

- NHS England genomic test directory: R156 (PRKAR1A). Orphanet ORPHA:1359.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Carney_complex
- Stratakis, J Clin Endocrinol Metab 2001: Carney complex, diagnostic criteria and recommendations for patient evaluation: https://doi.org/10.1210/jcem.86.9.7903
- Wasserman, Clin Cancer Res 2025: paediatric surveillance in hereditary endocrine neoplasia syndromes including Carney complex (AACR update): https://doi.org/10.1158/1078-0432.ccr-24-3860
- Orphanet ORPHA:1359: https://www.orpha.net/en/disease/detail/1359
- NHS England National Genomic Test Directory, rare and inherited disease, version 9 (spreadsheet): https://www.england.nhs.uk/wp-content/uploads/2018/08/rare-and-inherited-disease-national-genomics-test-directory-v9.xlsx

## Connected records

- cancers: [Sertoli cell tumour of the testis](https://onco.cc/cancers/sertoli-cell-tumour/)
- technologies: [Germline (hereditary) testing](https://onco.cc/technologies/germline-testing/)
- targets: [PRKAR1A](https://onco.cc/targets/prkar1a/)
- terms: [Hereditary cancer syndromes](https://onco.cc/terms/hereditary-cancer-syndromes/)
- drugs: [Invitae Multi-Cancer Panel](https://onco.cc/drugs/invitae-multi-cancer-panel/)

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