Familial cutaneous malignant melanoma and tumors of the nervous system. A hereditary cancer syndrome.
BACKGROUND: Excessive risk of cutaneous melanoma as a second cancer has been associated with benign or malignant tumors of the nervous system. Cutaneous melanoma and nervous system tumors may independently aggregate in families. There are, however, no previous reports of increased likelihood of tumors of the nervous system in families of patients with cutaneous melanoma or--of cutaneous melanoma in families with tumors of the nervous system. METHODS: The occurrence of nervous system tumors as second cancers was examined in a series of 904 patients with cutaneous melanoma and/or their family members. RESULTS: Fifteen families with 17 members with cutaneous melanoma from this series had one or more additional relatives with tumors of the nervous system, including astrocytoma, medulloblastoma, glioblastoma multiforme, ependymoma, glioma, meningioma, and acoustic neurilemmoma. Another subgroup of 10 patients with cutaneous melanoma had either a meningioma (n = 9) or acoustic neurilemmoma (n = 1) as a second tumor. The pattern of atypical melanocytic nevi occurring in the majority (20/25) of cutaneous melanoma patients in our series and in additional first degree relatives of 9 of 11 of the affected families, has been previously associated with the Familial Atypical Multiple Mole-Melanoma syndrome. CONCLUSIONS: This unusual familial and personal occurrence of tumors, showing differentiation toward tissues of the neural crest, neuroepithelium, and/or mesenchymal derivation, supports a putative association with a hereditary cancer susceptibility trait.