The diagnostic dilemma of primary central nervous system melanoma |
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| Authors: | Tabassum Wadasadawala Swati Trivedi Tejpal Gupta Sreedhar Epari Rakesh Jalali |
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| Institution: | 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;1. UCLA School of Nursing, University of California at Los Angeles, Los Angeles, CA 90095, USA;2. Department of Anesthesiology, University of California at Los Angeles, Los Angeles, CA 90095, USA;3. The Brain Research Institute, University of California at Los Angeles, Los Angeles, CA 90095, USA;4. Division of Cardiology, University of California at Los Angeles, Los Angeles, CA 90095, USA;5. Department of Neurobiology, University of California at Los Angeles, Los Angeles, CA 90095, USA;6. Department of Radiological Sciences, University of California at Los Angeles, Los Angeles, CA 90095, USA;7. Department of Bioengineering, University of California at Los Angeles, Los Angeles, CA 90095, USA;1. Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing 100050, China;2. Department of Neuro-pathology, Beijing Neurosurgical Institute, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing 100050, China;3. Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, No. 6 Tiantan Xili, Dongcheng District, Beijing 100050, China |
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| Abstract: | Melanomas are malignant neoplasms of melanocytes developing predominantly in the skin, but occasionally arising from eyes, mucous membranes, and the central nervous system (CNS). The CNS can be affected by a spectrum of melanocytic lesions ranging from diffuse neurocutaneous melanosis, to a focal and benign neoplasm (melanocytoma), and to an overtly malignant tumor (melanoma). Primary melanocytic lesions involving the CNS are typically concentrated in the perimedullary and high cervical region. Primary CNS melanoma cannot be reliably distinguished from metastatic melanoma on neuroimaging and histopathological characteristics alone: its diagnosis is established only after exclusion of secondary metastatic disease from a cutaneous, mucosal or retinal primary. We present two patients with primary CNS melanoma and discuss relevant issues, available treatment options, and expected outcomes. Awareness of disease spectrum and clinico-biological differences may be used to guide therapeutic decision-making for a patient with a proven or suspected primary CNS melanoma. |
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