Comments:
Myxoma Syndrome: About 5-10% of cardiac myxomas (CM) are familial (autosomal dominant) and occur as a component of Carney Complex which consists of:
- Primary pigmented nodular adrenocortical hyperplasia causing Cushing syndrome
- Lentigines, ephelides, and blue nevi of the skin & mucosae
- Myxomas of skin, heart, breast (myxoid mammary fibroadenomas) & other locations
- Psammomatous melanotic schwannoma, large cell calcifying Sertoli cell tumor, growth hormone-producing pituitary adenoma
Most patients do not have all of the manifestations. About
20% of familial cardiac myxomas are associated with
extra-cardiac manifestations. Patients with familial CM are often
younger than sporadic CM and show
no female predilection. The CM in these patients may not necessarily occur near fossa ovalis, and are more prone to embolization and recurrence.
About this image: Cutaneous and mucocutaneous pigmentation in a
9 y/o girl with
Carney Complex. Multiple non-elevated brown-black spots are present on the face, the vermilion borders of lips, and the upper eyelids.
Image Source: Courcoutsakis NA et al. The complex of myxomas, spotty skin pigmentation and endocrine overactivity (Carney complex): imaging findings with clinical and pathological correlation. Insights Imaging. 2013 Feb; 4(1): 119-133; image cropped from the original and used under Creative Common Attribution License.
