Primary Diffuse Leptomeningeal Gliomatosis Coincident With a Melanocytic Disorder

Abstract

Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplasm of the leptomeninges associated with limited survival time. Around 60 cases of PDLG have been reported in the literature and most of them were diagnosed at post mortem examination. We describe the longest survival in a PDLG case, a 24 year old man who survived for 30 months with an aggressive treatment approach that included chemoradiation and repeated surgical excisions of tumor. Our patient also had multiple dysplastic nevi and dysmorphic facial features raising the suspicion of an underlying genetic disorder.




J Med Cases. 2013;4(8):526-529
doi: http://dx.doi.org/10.4021/jmc1361w