Disseminated oligodendroglial-like leptomeningeal tumor of childhood: A distinctive clinicopathologic entity Journal Article


Authors: Rodriguez, F. J.; Perry, A.; Rosenblum, M. K.; Krawitz, S.; Cohen, K. J.; Lin, D.; Mosier, S.; Lin, M. T.; Eberhart, C. G.; Burger, P. C.
Article Title: Disseminated oligodendroglial-like leptomeningeal tumor of childhood: A distinctive clinicopathologic entity
Abstract: Rare, generally pediatric oligodendrogliomalike neoplasms with extensive leptomeningeal dissemination have been interpreted variably as glial, oligodendroglial or glioneuronal. The clinicopathologic features have not been fully characterized. We studied 36 patients, 12 females and 24 males with a median age of 5 years (range 5 months- 46 years). MRI demonstrated leptomeningeal enhancement, frequently with cystic or nodular T2 hyperintense lesions within the spinal cord/brain along the subpial surface. A discrete intraparenchymal lesion, usually in the spinal cord, was found in 25 (of 31) (81 %). Tumors contained oligodendroglioma-like cells with low-mitotic activity (median 0 per 10 high power fields, range 0-4), and rare ganglion/ganglioid cells in 6 cases (17 %). Tumors were mostly low-grade, with anaplastic progression in 8 (22 %). Immunohistochemistry demonstrated strong reactivity for OLIG2 (7 of 9) (78 %), and moderate/strong S100 (11 of 12) (92 %), GFAP (12 of 31) (39 %) and synaptophysin (19 of 27) (70 %). NeuN, EMA, and mutant IDH1 (R132H) protein were negative. Median MIB1 labeling index was 1.5 % (range <1-30 %). FISH (n = 13) or SNP array (n = 2) demonstrated 1p loss/intact 19q in 8 (53 %), 1p19q co-deletion in 3 (20 %), and no 1p or 19q loss in 4 (27 %). Clinical follow-up (n = 24) generally showed periods of stability or slow progression, but a subset of tumors progressed to anaplasia and behaved more aggressively. Nine patients (38 %) died 3 months-21 years after diagnosis (median total follow-up 5 years). We report a series of a neoplasm with distinct clinicopathologic and molecular features. Although most progress slowly, a significant fraction develop aggressive features. © 2012 Springer-Verlag.
Keywords: immunohistochemistry; adult; child; clinical article; human tissue; preschool child; single nucleotide polymorphism; clinical feature; cancer growth; neuroimaging; nuclear magnetic resonance imaging; follow up; cancer grading; ki 67 antigen; immunoreactivity; cancer mortality; survival time; fluorescence in situ hybridization; brain; infant; contrast enhancement; spine; protein s 100; mitosis rate; oligodendroglioma; chromosome deletion; chromosome loss; meningeal metastasis; chromosome 1p; synaptophysin; 1p19q; chromosome 19q; 1p; leptomeninges; glioneuronal; pediatric glioma
Journal Title: Acta Neuropathologica
Volume: 124
Issue: 5
ISSN: 0001-6322
Publisher: Springer  
Date Published: 2012-11-01
Start Page: 627
End Page: 641
Language: English
DOI: 10.1007/s00401-012-1037-x
PROVIDER: scopus
PUBMED: 22941225
DOI/URL:
Notes: --- - "Export Date: 2 January 2013" - "CODEN: ANPTA" - "Source: Scopus"
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  1. Marc Rosenblum
    424 Rosenblum