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Carney Complex



Carney first described patients with the association of myxomas, spotty pigmentation of skin and mucous membranes, and endocrine overactivity. This syndrome belongs to a group of genetic disorders, the lentiginoses, which include Peutz-Jeghers, LEOPARD, and Laugier-Hunziker syndromes. The most serious manifestation of the Carney complex are cardiac myxomas, which can occur in approximately one third of patients

Carney complex, a familial multiple neoplasia and lentiginosis syndrome: analysis of 11 kindreds and linkage to the short arm of chromosome 2 J Clin Invest 1996;97:699-705
Circulation 1998;98:2560-6.
Identification of a novel genetic locus for familial cardiac myxomas and Carney complex Circulation 1998;98:2560-6.

Long arm chromosome 17


Cutaneous myxomas One third of patients
Pink shiny papules which may be larger and pedunculated
Cutaneous lentigines Majority of patients

Most commonly seen on the face, especially in the periocular and the perioral regions
May also occur on the ears, trunk, neck, lips (commonly on the vermilion border), conjunctiva, sclera, vulva, or glans penis


Osteochondromyxoma of Bone Am J Surg Pathol 2001;25:164-176

Four bone tumors
Three were congenital; the fourth, detected at age 1 year, was probably of considerable duration

Three boys and one girl, each presented with a painless mass
Two had the Carney complex
Another probably had this disorder
Fourth did not show it, but his mother did

Tumors occurred in the nasal region (n = 2) and the diaphysis of the tibia and radius (n = 1 each)

Roentgenographically, three had benign characteristics; the fourth, malignant features

Grossly, the tumors were gelatinous, cartilaginous, and bony

Benign-appearing polymorphic cells with few division figures arranged in sheets and lobules set in a myxomatous, cartilaginous, osseous, and hyaline fibrous matrix
Cellularity was low to moderate
Tumors eroded bone, one infiltrated between bony trabeculae, and three had soft tissue extension

Complete resection of one tumor was curative; incomplete excision of two tumors resulted in local recurrence (intracranial and fatal) in one and persistence in the other; the fourth tumor remains under observation after biopsy

No tumor metastasized

Epithelioid blue nevi Am J Surg Pathol 1996;19:259–72

Larger, somewhat raised lesions that are consistent with blue nevi

Psammomatous melanotic schwannoma Am J Surg Pathol 1990;14:206-22

Up to 10% of patients they are malignant and can metastasize

Pigmented lesions of the conjunctiva in Carney's complex J Am Acad Dermatol 2000;42:145.
Breast imaging findings Radiology 1997;205:221-7.

Myxoid mammary tumors or ductal adenomas, may be seen in about one fourth of patients

Endocrine abnormalities Adrenal or testicular tumors (or both) are present in approximately one third of patients, with pituitary tumors being present in about one tenth of patients

Medicine (Baltimore) 1985;64:270-83.
Semin Dermatol 1995;14:90-8.

New Support Group for Carney Complex.
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