TUMOR SPECTRUM IN THE FAMMM SYNDROME

The familial atypical multiple mole-melanoma syndrome (FAMMM) is characterized by an autosomal dominantly inherited susceptibility to multiple atypical nevi. Patients with this hereditary phenotype show a strong susceptibility to cutaneous malignant melanoma (CMM). An investigation of an extended Dutch kindred showing the FAMMM phenotype revealed a proband with bilateral intraocular malignant melanoma (IOM) and multiple CMM. The family revealed an array of tumors which included carcinoma of the lung, skin, larynx and breast in addition to CMM and IOM, which were transmitted vertically through 3 generations. There was male-to-male transmission and the number of affected males and females was about the same which was consistent with an autosomal dominant inheritance. The FAMMM syndrome indicates a potential for CMM and a susceptibility to other systemic cancers. These observations merit a painstaking evaluation of cancer of all anatomical sites in other kindreds showing the FAMMM syndrome. Such studies could yield clues to cancer etiology, pathogenesis and control.