And It's About Time There Was Some Support For Cushing's!
From http://www.thedoctorsdoctor.com/diseases/carney_complex.htm
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
| PATHOGENESIS | CHARACTERIZATION |
| 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 |
| GROSS APPEARANCE/CLINICAL VARIANTS | CHARACTERIZATION |
| General | |
| 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 |
| HISTOLOGICAL TYPES | CHARACTERIZATION |
| General | |
| VARIANTS | |
| Osteochondromyxoma of Bone | Am J Surg Pathol
2001;25:164-176 Four bone tumors Three boys and one girl, each presented with a
painless mass 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 Microscopic: 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.
