TY - JOUR
T1 - Primary Leptomeningeal Gliomatosis in Children and Adults
T2 - A Morphological and Molecular Comparative Study with Literature Review
AU - Tauziede-Espariat, Arnault
AU - Maues De Paula, Andre
AU - Pages, Melanie
AU - Laquerriere, Annie
AU - Caietta, Emilie
AU - Delpont, Benoit
AU - Viennet, Gabriel
AU - Medeiros De Bustos, Elisabeth
AU - Moulin, Thierry
AU - Barnerias, Christine
AU - Vauleon, Elodie
AU - Grill, Jacques
AU - Chiforeanu, Dan
AU - Vasiljevic, Alexandre
AU - Varlet, Pascale
N1 - Publisher Copyright:
© 2015 by the Congress of Neurological Surgeons.
PY - 2016/3/1
Y1 - 2016/3/1
N2 - BACKGROUND: Primary leptomeningeal gliomatosis (PLG) is a poorly recognized tumor of the central nervous system. OBJECTIVE: To describe the histopathological, immunohistochemical, and molecular features of PLG. METHODS: Results of our multicentric retrospective study of 6 PLG cases (3 pediatric and 3 adult) were compared with literature data. RESULTS: The 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. CONCLUSION: This 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.
AB - BACKGROUND: Primary leptomeningeal gliomatosis (PLG) is a poorly recognized tumor of the central nervous system. OBJECTIVE: To describe the histopathological, immunohistochemical, and molecular features of PLG. METHODS: Results of our multicentric retrospective study of 6 PLG cases (3 pediatric and 3 adult) were compared with literature data. RESULTS: The 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. CONCLUSION: This 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.
KW - Histones
KW - Isocitrate dehydrogenase
KW - Primary leptomeningeal gliomatosis
UR - http://www.scopus.com/inward/record.url?scp=84959075104&partnerID=8YFLogxK
U2 - 10.1227/NEU.0000000000001028
DO - 10.1227/NEU.0000000000001028
M3 - Article
C2 - 26397750
AN - SCOPUS:84959075104
SN - 0148-396X
VL - 78
SP - 343
EP - 352
JO - Neurosurgery
JF - Neurosurgery
IS - 3
ER -