Leptomeningeal melanoma in childhood

BACKGROUND, Malignant melanoma (MM) is one of the least common types of chi ldhood cancer, accounting for less than 1% of all pediatric malignancies. N eurocutaneous melanosis (NCM) is a rare phakomatosis consisting of congenit al abnormal pigmentation of the skin and meninges. The meningeal lesions ar e particularly prone to malignant change. METHODS, The authors describe 5 patients with NCM and I with primary leptom eningeal melanoma (LMM) seen at 2 treatment centers in the north of England over a 13-year period (1984-1997). RESULTS. The clinical features, progress, radiological findings, and treatm ent of these patients are discussed. AU six died within eight months of the ir diagnosis, illustrating the difficulties faced in treating patients with these conditions. The authors reviewed the published literature on NCM, co ncentrating on the various therapeutic strategies that have been tried. Ver y little consistency in approach was found. Malignant skin lesions in NCM m ay be less responsive than primary malignant melanoma, but the small number of patients with primary LMM or brain metastases of MM make comparisons wi th NCM difficult. The authors' own series illustrates well the piecemeal na ture of therapy for patients with these rare conditions. CONCLUSIONS. The rate of incidence of MM melanoma in the U.K. is increasing , and it will represent an increasing proportion of the pediatric oncologis t's workload. A consistent approach to the therapy of patients with metasta tic NM and NCM is needed if we are to have any hope of offering more than p alliative therapy to these children in the future. (C) 1999 American Cancer Society.