Biomaterial Database

Dysplastic nevus syndrome with multiple primary amelanotic melanomas in oculocutaneous albinism. 1984

H Pehamberger, and H Hönigsmann, and K Wolff

Melanomas are rare in albinos, although the incidence of solar radiation-induced skin tumors is extremely high because of the absence of photoprotective melanin. This report describes a 40-year-old white woman with tyrosinase-negative oculocutaneous albinism who developed four primary amelanotic melanomas--three of the superficial spreading and one of the nodular type-and, in addition, displayed nevi that exhibited histologically the characteristic features of dysplastic nevi. The concomitant occurrence of multiple primary melanomas and several dysplastic nevi classifies the patient's condition as "dysplastic nevus syndrome," which to our knowledge has not been described in albinism so far.

UI	MeSH Term	Description	Entries
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
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
D009508	Nevus, Pigmented	A nevus containing melanin. The term is usually restricted to nevocytic nevi (round or oval collections of melanin-containing nevus cells occurring at the dermoepidermal junction of the skin or in the dermis proper) or moles, but may be applied to other pigmented nevi.	Nevus, Melanocytic,Nevi, Melanocytic,Nevi, Pigmented,Pigmented Moles,Melanocytic Nevi,Melanocytic Nevus,Pigmented Nevi,Pigmented Nevus
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D000417	Albinism	General term for a number of inherited defects of amino acid metabolism in which there is a deficiency or absence of pigment in the eyes, skin, or hair.
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
D013577	Syndrome	A characteristic symptom complex.	Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes