• Arnault Tauziede-Espariat, Andre Maues de Paula, Melanie Pages, Annie Laquerriere, Emilie Caietta, Benoit Delpont, Gabriel Viennet, Elisabeth Medeiros de Bustos, Thierry Moulin, Christine Barnerias, Elodie Vauleon, Jacques Grill, Dan Chiforeanu, Alexandre Vasiljevic, and Pascale Varlet.
• *Department of Pathology, Lariboisière Hospital, Paris, France; ‡Department of Pathology, La Timone, Marseille, France; §Department of Neuropathology, Sainte-Anne's Hospital, Paris, France; ¶Department of Pathology, Charles Nicolle Hospital, Rouen, France; ‖Department of Neuropediatry, La Timone, Marseille, France; #Department of Neurology, Jean Minjoz Hospital, Besançon, France; **Department of Pathology, Jean Minjoz Hospital, Besançon, France; ‡‡Department of Neuropediatry, Necker Hospital, Paris, France; §§Department of Oncology, Rennes Hospital, Rennes, France; ¶¶Department of Oncology, Gustave Roussy Institute, Paris, France; ‖‖Department of Pathology, Rennes Hospital, Rennes, France; ##Department of Pathology, CHU Est Lyon, Bron, France.
• Neurosurgery. 2016 Mar 1; 78 (3): 343-52.

BackgroundPrimary leptomeningeal gliomatosis (PLG) is a poorly recognized tumor of the central nervous system.ObjectiveTo describe the histopathological, immunohistochemical, and molecular features of PLG.MethodsResults of our multicentric retrospective study of 6 PLG cases (3 pediatric and 3 adult) were compared with literature data.ResultsThe mean age was 54.7 years for adults and 8.7 years for children, with 3 males and 3 females. Clinical symptoms were nonspecific. Cerebrospinal fluid analyses showed a high protein level often associated with pleocytosis but without neoplastic cells. On neuroimaging, diffuse leptomeningeal enhancement and hydrocephalus were observed, except in 1 case. PLG was mostly misinterpreted as infectious or tumoral meningitis. The first biopsy was negative in 50% of cases. Histopathologically, PLG cases corresponded to 1 oligodendroglioma without 1p19q codeletion and 5 astrocytomas without expression of p53. No immunostaining for IDH1R132H and no mutations of IDH1/2 and H3F3A genes were found. Overall survival was highly variable (2-82 months) but seems to be increased in children treated with chemotherapy.ConclusionThis study shows the difficulties of PLG diagnosis. The challenge is to achieve an early biopsy to establish a diagnosis and to begin a treatment, but the prognosis remains poor. PLG seems to have a different molecular and immunohistochemical pattern compared with intraparenchymal malignant gliomas.

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