Leptomeningeal melanoma in childhood

• Published in: Cancer 1999-09, Vol.86 (5), p.878-886
• Main Authors: Makin, Guy W. J., Eden, Osborn B., Lashford, Linda S., Moppett, John, Gerrard, Mary P., Davies, Helena A., Powell, Colin V. E., Campbell, Alastair N., Child, H. Frances
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; brain; Central Nervous System Neoplasms - diagnosis; Central Nervous System Neoplasms - secondary; chemotherapy; Child; Child, Preschool; Dermatology; Fatal Outcome; Female; Hereditary diseases of the skin. Congenital diseases of the skin. Haemangioma of the skin, of mucosae and of soft tissue; Humans; Male; Medical sciences; melanoma; Melanoma - congenital; Melanoma - diagnosis; Melanoma - pathology; Melanoma - secondary; Neurology; Nevus, Pigmented - congenital; Nevus, Pigmented - diagnosis; Nevus, Pigmented - pathology; pediatric oncology; Skin Neoplasms - congenital; Skin Neoplasms - diagnosis; Skin Neoplasms - pathology; Tumors of the nervous system. Phacomatoses
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/(SICI)1097-0142(19990901)86:5<878::AID-CNCR26>3.0.CO;2-Y

BACKGROUND Malignant melanoma (MM) is one of the least common types of childhood cancer, accounting for less than 1% of all pediatric malignancies. Neurocutaneous melanosis (NCM) is a rare phakomatosis consisting of congenital abnormal pigmentation of the skin and meninges. The meningeal lesions are particularly prone to malignant change. METHODS The authors describe 5 patients with NCM and 1 with primary leptomeningeal 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 treatment of these patients are discussed. All six died within eight months of their diagnosis, illustrating the difficulties faced in treating patients with these conditions. The authors reviewed the published literature on NCM, concentrating on the various therapeutic strategies that have been tried. Very little consistency in approach was found. Malignant skin lesions in NCM may be less responsive than primary malignant melanoma, but the small number of patients with primary LMM or brain metastases of MM make comparisons with NCM difficult. The authors' own series illustrates well the piecemeal nature 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 oncologist's workload. A consistent approach to the therapy of patients with metastatic MM and NCM is needed if we are to have any hope of offering more than palliative therapy to these children in the future. Cancer 1999;86:878–86. © 1999 American Cancer Society. This article describes a series of patients with leptomeningeal melanoma and reviews the literature concentrating on therapy.