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Case Reports
. 2022 Jun 23;2022(6):omac063.
doi: 10.1093/omcr/omac063. eCollection 2022 Jun.

Carney complex with multiple breast tumours including breast cancer: a case report

Akihiro Fujimoto  1 , Ayaka Sakakibara  1 , Yoshiki Numajiri  2 , Kazuo Matsuura  1 , Tomonori Kawasaki  3 , Akihiko Osaki  1 , Toshiaki Saeki  1
Affiliations
Case Reports

Carney complex with multiple breast tumours including breast cancer: a case report

Akihiro Fujimoto et al. Oxf Med Case Reports. .

Abstract

Carney complex (CNC) is a rare multiple tumour syndrome characterized by cutaneous pigmented lesions, myxoma and endocrine tumours, among others, and is inherited as an autosomal dominant trait. Protein kinase cAMP-dependent type I regulatory subunit alpha (PRKAR1A) is known to be the responsible gene. Breast myxomatosis and ductal adenoma, which are regarded as benign, are well-known mammary lesions of CNC and are included in the main diagnostic criteria. In this case, a 59-year-old woman with repeated cardiac myxoma was diagnosed with CNC with PRKAR1A mutation. She also had three multiple breast tumours bilaterally: breast cancer, adenomyoepithelioma and intraductal papilloma. In mammary lesions of CNC, attention should be paid to benign lesions, such as breast myxomatosis or ductal adenomas, and the development of breast cancer or breast tumours with malignant potential. Mammary lesions should be aggressively scrutinized and considered for resection, as required.

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Figures

Figure 1
Figure 1
Important timelines.
Figure 2
Figure 2
Physical findings in 2020. Many brown spotty skin pigmentations on the anterior chest. Palpable breast mass in the right breast (red arrow). Surgical scars on the bilateral breasts (blue arrows).
Figure 3
Figure 3
Clinical images of the right breast tumour in 2020. (a) Mammography showing a high-density mass with circumscribed margins (arrow). (b) Ultrasonography showing a 38-mm complicated cyst.
Figure 4
Figure 4
Macroscopic findings of the specimen. (a) Tumour location is in the red circle. (b) Cut surface of the excised specimen.
Figure 5
Figure 5
Histopathological findings of AME. (a) The tumour comprises a mosaic admixture of eosinophilic epithelial cell components and clear myoepithelial cell elements (haematoxyin and eosin, ×200). (b) Immunohistochemically, p63 is positive in myoepithelial cells (×200). (c) Cytokeratin 14 is antithetically reactive in epithelial cells (×200). (d) Oestrogen receptor shows diffuse and relatively weak immuno-expressions in tumour cells (×200).
See this image and copyright information in PMC

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