1. ^*MGC-Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands
2. ^†Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands

Correspondence: Dr N.A. Gruis, MGC-Department of Human Genetics, Leiden University Medical Center, PO Box 9503, 2300 RA Leiden, The Netherlands. E-mail: gruis@ruly46.medfac.Leidenuniv.nl

Received 10 December 1998; Revised 2 March 1999; Accepted 9 March 1999.

Abstract

The most common hereditary melanoma susceptibility disorder is the familial atypical multiple mole-melanoma (FAMMM) syndrome. FAMMM is regarded as an ideal natural model to study the very complex pathologic mechanism of melanoma. In 1994, cloning of the melanoma susceptibility gene CDKN2A was thought to give answers to many questions on genotype-phenotype correlations in familial melanoma. Today, 4 y later, germline mutations cosegregate with melanoma in only 40%–50% of the families predisposed to this disease. The hunt for genes and modifying genes is on again. Through the years the very well-characterized Dutch FAMMM families have proven to be valuable study subjects in melanoma research. This paper describes over 10 y of melanoma research illustrated by research performed in the Dutch FAMMM families.