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Primary melanocytic neoplasms of the central nervous system
Authors:Simon V. Liubinas  Nicholas Maartens  Katharine J. Drummond
Affiliation:1. Department of Pathology, Canisius Wilhelmina Hospital, P.O. Box 9015, 6500 GS Nijmegen, The Netherlands;2. Department of Medical Oncology, Ghent University Hospital, De Pintelaan 185, 9000 Ghent, Belgium;3. Center for Medical Genetics, Ghent University Hospital, De Pintelaan 185, 9000 Ghent, Belgium;4. Department of Radiation Oncology, Ghent University Hospital, De Pintelaan 185, 9000 Ghent, Belgium;5. Department of Human Genetics, Radboud University Medical Center, P.O. Box 9100, 6500 HB Nijmegen, The Netherlands;6. Department of Pathology, Ghent University Hospital, De Pintelaan 185, 9000 Ghent, Belgium.;7. Department of Neuropathology, Heidelberg University Hospital, INF 224, 69120 Heidelberg, Germany;8. Department of Pathology, Radboud University Medical Center, P.O. Box 9100, 6500 HB Nijmegen, The Netherlands;9. Department of Pathology, Maastricht University Medical Center, P.O. Box 5800, 6202 AZ Maastricht, The Netherlands;10. Department of Pathology, VU University Medical Center, P.O. Box 7057, 1007 MB Amsterdam, The Netherlands;11. Department of Pathology, Princess Máxima Center for Pediatric Oncology and University Medical Center Utrecht, P.O. Box 85500, 3508 GA Utrecht, The Netherlands
Abstract:Primary melanocytic neoplasms of the central nervous system (CNS) are rare lesions arising from melanocytes of the leptomeninges. They include diffuse leptomeningeal melanocytosis or melanomatosis, melanocytoma and primary malignant melanoma. We have reviewed the English literature regarding these lesions, which consists of case reports and a small number of larger case series. The presenting features, radiological, surgical and histological findings are reviewed, as are current management options and prognosis. We also present illustrative case reports of diffuse leptomeningeal melanocytosis and primary melanoma of the CNS.
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