Carney complex (CNC, also known as Carney syndrome, NAME syndrome [Nevi, Atrial Myxoma, Myxoid neurofibromas, and Ephelides], LAMB syndrome [Lentigines, Atrial Myxomas, and Blue nevi]) is defined by the manifestation of multiple entities, including primary pigmented nodular adrenal dysplasia causing Cushing syndrome, abnormal pigmentation of the skin (blue nevi, lentigines) and tumors such as cardiac, skin and breast myxomas, pituitary adenomas, melanotic schwannomas, myxoid neurofibromas, and testicular tumors [1]. It is an autosomal dominant disease caused by mutations in the CNC1 (PRKAR1A on chromosome 17) and CNC2 (chromosome 2) genes [2,3]. Although genetic testing is currently only available for CNC1 in most institutions, the publication of familiar cases with CNC remains important for both pathologists and clinicians [4,5].

In 1992, a 6-year-old girl (Patient 1) presented with left-sided weakness caused by a cerebral vascular accident (CVA) secondary to the embolization of a cardiac myxoma located in the left atrium. The initial diagnosis was NAME syndrome. Ten years later, in 2002, she was found to have a second left atrial myxoma, which prompted an investigation of the rest of her family. Left atrial myxomas were identified in the younger sister (Patient 2), and the mother (Patient 3); the brother (Patient 5) was also known to have developed testicular Sertoli cell tumor at age 6. The maternal grandmother (Patient 4) was diagnosed with a left atrial myxoma in 2005 (Figures 1-2).

All cardiac myxomas were surgically treated at the time of diagnosis. Patient 1 subsequently underwent a bilateral mastectomy, which revealed breast myxomas with no evidence of malignancy; Patient 2 was diagnosed with recurrent left atrial myxomas in 2006 and 2008; Patient 3 was found to have two right and left atrial myxomas in 2005, as well as spotty skin pigmentation.