Biomaterial Database

B-K mole syndrome. Cutaneous and ocular malignant melanoma. 1980

D H Abramson, and R S Rodriguez-Sains, and R Rubman

A 51-year-old white man had the B-K mole syndrome (multiple large atypical cutaneous nevi of the upper part of the trunk and extremities, inherited as an autosomal dominant trait, and thought to be more susceptible to malignant transformation), so named after two patients in whom the syndrome was first observed. Two cutaneous malignant melanomas (thigh and back) and an ocular malignant melanoma (ciliary body and iris) simultaneously developed. Patients with B-K mole syndrome have been known to have a very high risk for the development of cutaneous melanoma (including multiple primary cutaneous melanomas) and multiple primary malignancies. There may be a propensity in these patients for development of ocular melanomas.

UI	MeSH Term	Description	Entries
D007498	Iris	The most anterior portion of the uveal layer, separating the anterior chamber from the posterior. It consists of two layers - the stroma and the pigmented epithelium. Color of the iris depends on the amount of melanin in the stroma on reflection from the pigmented epithelium.
D008297	Male		Males
D008545	Melanoma	A malignant neoplasm derived from cells that are capable of forming melanin, which may occur in the skin of any part of the body, in the eye, or, rarely, in the mucous membranes of the genitalia, anus, oral cavity, or other sites. It occurs mostly in adults and may originate de novo or from a pigmented nevus or malignant lentigo. Melanomas frequently metastasize widely, and the regional lymph nodes, liver, lungs, and brain are likely to be involved. The incidence of malignant skin melanomas is rising rapidly in all parts of the world. (Stedman, 25th ed; from Rook et al., Textbook of Dermatology, 4th ed, p2445)	Malignant Melanoma,Malignant Melanomas,Melanoma, Malignant,Melanomas,Melanomas, Malignant
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D009378	Neoplasms, Multiple Primary	Two or more abnormal growths of tissue occurring simultaneously and presumed to be of separate origin. The neoplasms may be histologically the same or different, and may be found in the same or different sites.	Neoplasms, Synchronous,Neoplasms, Synchronous Multiple Primary,Multiple Primary Neoplasms,Multiple Primary Neoplasms, Synchronous,Synchronous Multiple Primary Neoplasms,Synchronous Neoplasms,Multiple Primary Neoplasm,Neoplasm, Multiple Primary,Neoplasm, Synchronous,Primary Neoplasm, Multiple,Primary Neoplasms, Multiple,Synchronous Neoplasm
D009506	Nevus	A circumscribed stable malformation of the skin and occasionally of the oral mucosa, which is not due to external causes and therefore presumed to be of hereditary origin.	Mole, Skin,Moles, Skin,Skin Mole,Nevi,Skin Moles
D002924	Ciliary Body	A ring of tissue extending from the scleral spur to the ora serrata of the RETINA. It consists of the uveal portion and the epithelial portion. The ciliary muscle is in the uveal portion and the ciliary processes are in the epithelial portion.	Corpus Ciliare,Corpus Ciliaris,Bodies, Ciliary,Body, Ciliary,Ciliare, Corpus,Ciliares, Corpus,Ciliari, Corpus,Ciliaris, Corpus,Ciliary Bodies,Corpus Ciliares,Corpus Ciliari
D005134	Eye Neoplasms	Tumors or cancer of the EYE.	Cancer of Eye,Eye Cancer,Cancer of the Eye,Neoplasms, Eye,Cancer, Eye,Cancers, Eye,Eye Cancers,Eye Neoplasm,Neoplasm, Eye
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012878	Skin Neoplasms	Tumors or cancer of the SKIN.	Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm