Nevi and melanoma: Lessons from Turner's syndrome

Females with Turner's syndrome (TS) have a markedly increased number of cut aneous nevi. While this is a well-recognized risk factor for cutaneous mela noma (CM), the incidence of this tumor in TS and the implications for our u nderstanding of nevi and melanoma have not previously been considered. Here we report a case of an anorectal melanoma in a woman with TS and a review of the literature. Overall, there appears to be a lower than expected incid ence of CM, Possible explanations are discussed and in particular the possi ble relationship between sex hormones and melanoma development as these gir ls fail to undergo normal pubertal development. Further study of this syndr ome may provide important insights into the genetic factors involved in nor mal melanocyte and nevus development, the potential influence of sex hormon es on melanoma development and the relationship between the presence of nev i and the risk of developing CM. Copyright (C) 2001 S. Karger AG, Basel.