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42 (6), e13635 (2018) B
Definition / general Rare melanocytic tumors that originate from leptomeningeal melanocytes Classified according to their growth pattern (circumscribed or diffuse) and histological features (benign versus malignant) Essential features Circumscribed forms include benign lesions (melanocytomas), intermediate grade lesions (intermediate grade melanocytomas) and their malignant counterpart (melanomas) Diffuse forms, often occurring in the setting of neurocutaneous melanosis, are classified as meningeal melanocytosis (benign) or meningeal melanomatosis (malignant) according to histological features Histological and molecular similarities of primary leptomeningeal melanomas to blue nevus-like melanoma and uveal melanoma Immunohistochemical positivity for melanocytic markers (S100, SOX10, MART1, HMB45) Mutations in GNAQ , GNA11 , PLCB4 and CYSLTR
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