Skin manifestations include dark pigmented skin
lesions (lentigines). Although they can be found anywhere on the body, they
typically are located in the center of the face (i.e., periorally,
perinasally, periorbitally, and in the sclerae). The finding of lentigines
on the lips in association with a myxoma or an endocrine disorder/tumor
should prompt further examinations to rule out Carney complex. Urogenital
(vulvar) lentigines also are considered characteristic for this disorder.
Cardiac myxomas may be solitary or multiple and can occur in any cardiac
chamber, although almost 90% occur in one of the atria. The left atrium
accounts for 80% of all atrial myxomas. After resection they may recur
(approximately in 10-20% of cases) at the same or different intracardiac and/or
extracardiac sites. Cardiac myxomas account for 25% of deaths in
patients with Carney complex. A history of neurologic deficits or transient
ischemic attacks may be present if tumor embolization has occurred. Systemic
manifestations such as fever, arthralgia, elevated erythrocyte sedimentation
rate, and lupus-like rashes may accompany some myxomas. Extracardiac myxomas
(of breast, testis, thyroid, brain, adrenal gland) and nonmyxomatous tumors
(e.g., Sertoli cell and Leydig cell tumor of the testis, pituitary adenomas,
and psammomatous melanotic schwannomas [mainly esophagus and stomach, but
also along the paraspinal sympathetic ganglia]) have been described.
Pituitary adenomas may result in hyperprolactinemia and acromegaly.