TY - JOUR T1 - Pearls & Oy-sters: Primary Diffuse Leptomeningeal Melanocytosis JF - Neurology JO - Neurology SP - e576 LP - e580 DO - 10.1212/WNL.0000000000207195 VL - 101 IS - 5 AU - Jeevi Mona Priyadharshni Selvarajan AU - Sridhar Epari AU - Arpita Sahu AU - Archya Dasgupta AU - Abhishek Chatterjee AU - Tejpal Gupta Y1 - 2023/08/01 UR - //www.ez-admanager.com/content/101/5/e576.abstract N2 - Primary diffuse leptomeningeal melanocytosis (PDLM) is an extremely rare CNS tumor with nonspecific clinicoradiologic features that overlap considerably with aseptic meningitis posing significant diagnostic and therapeutic challenges. We present one such case report of a patient treated empirically at first presentation as aseptic viral meningitis based on MRI and CSF analysis. Diagnosis of PDLM was established subsequently through meningeal biopsy that demonstrated a melanocytic tumor with fine granular melanin pigment without significant mitoses. Her systemic and ocular examination was unremarkable. Whole-body 18F-fluorodeoxyglucose PET/CT (FDG-PET/CT) did not identify any other primary site. Following ventriculoperitoneal shunt to relieve hydrocephalus, she was treated with definitive craniospinal irradiation plus whole-brain boost and remains stable on periodic clinicoradiologic surveillance. Optimal management of PDLM lacks consensus with role of radiotherapy, chemotherapy, targeted therapy and immunotherapy being controversial. ER -