Primary Leptomeningeal Gliomatosis is a rare neoplastic disease of the central nervous system. This tumor arises solely in the leptomeninges without a dominant intraparenchymal brain or spinal cord lesion. The diagnosis is very challenging during life, with fewer than 100 reported cases in the medical literature. Initially thought to present as either a nodular or diffuse subtype (Primary Diffuse Leptomeningeal Gliomatosis: PDLG), the nodular forms are currently felt to be more appropriately termed Primary Leptomeningeal Glioma. This tumor is too rare to have been assigned a WHO classification. We propose that the term PDLG be replaced with Gliomatosis Meningi to make the relationship to the other form of Gliomatosis—Gliomatosis Cerebri, clearer. We report the case of a 63 year-old man who first presented with a grand mal seizure followed by progressive neurological decline, hydrocephalus, and spinal radiculopathy. He had an initial tentative diagnosis of neurosarcoidoisis radiographically. He went on to have progressive leptomeningeal enhancement on MRI involving the brain and the spinal cord with a negative initial temporal lobe biopsy followed by a subsequent biopsy of the leptomeninges, which revealed Leptomeningeal Gliomatosis. He continued to decline clinically and died 9 months after symptom onset despite treatment with craniospinal radiation therapy and chemotherapy with oral Temozolamide. We present a comprehensive review of the cases published in the literature to date. We restricted our search to the cases that described diffuse meningeal disease and excluded solitary tumors, non glial tumors as well as secondary leptomeningeal spread. A comprehensive review of the natural history of this disease is provided in the hope that this diagnosis can be made earlier in the future so that treatment can be offered before the tumor is too advanced.
展开▼
