Primary Leptomeningeal Gliomatosis in Children and Adults: A Morphological and Molecular Comparative Study With Literature Review.
Neurosurgery
; 78(3): 343-52, 2016 Mar.
Article
em En
| MEDLINE
| ID: mdl-26397750
ABSTRACT
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Neuroepiteliomatosas
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Carcinomatose Meníngea
Tipo de estudo:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adult
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Child
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Child, preschool
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Female
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Humans
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Male
Idioma:
En
Revista:
Neurosurgery
Ano de publicação:
2016
Tipo de documento:
Article
País de afiliação:
Panamá