15 Year Old With Multiple Cutaneous Carcinomas

Kevin E. Snyder, M.D.

July 07, 2004

Discussion

Basal cell nevus syndrome, also known as Gorlin syndrome, is an autosomal dominantly inherited condition. Multiple basal cell carcinomas, jaw cysts, ectopic calcifications and skeletal anomalies characterize this syndrome. Presentation in these patients tends to be due to the cutaneous basal cell carcinomas, with presentation at a mean age of 19. Following puberty, these skin lesions demonstrate aggressive features, and may metastasize. The jaw cysts present are odontogenic in etiology. Abnormalities occur in the upper five ribs, and may demonstrate a fused, bifid or other dysplastic appearance. Calcification in the falx or tentorium may occur, as may other ectopic calcifications involving the subcutaneous tissues, ovaries or mesentery. Other possible findings, not demonstrated in the current case, include scoliosis, vertebral anomalies, Sprengel deformity and brachydactyly. There is a high association of childhood medulloblastomas with this syndrome.

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