Acta Neuropathologica

, Volume 124, Issue 5, pp 627–641

Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive clinicopathologic entity

Authors

    • Department of Pathology, Division of Neuropathology, Johns Hopkins HospitalJohns Hopkins University
  • Arie Perry
    • Department of PathologyUniversity of California San Francisco
  • Marc K. Rosenblum
    • Department of PathologyMemorial Sloan-Kettering Cancer Center
  • Sherry Krawitz
    • Health Sciences Centre
  • Kenneth J. Cohen
    • The Sidney Kimmel Comprehensive Cancer Center at Johns HopkinsJohns Hopkins University
  • Doris Lin
    • Division of NeuroradiologyJohns Hopkins University
  • Stacy Mosier
    • Department of Pathology, Johns Hopkins HospitalJohns Hopkins University
  • Ming-Tseh Lin
    • Department of Pathology, Johns Hopkins HospitalJohns Hopkins University
  • Charles G. Eberhart
    • Department of Pathology, Division of Neuropathology, Johns Hopkins HospitalJohns Hopkins University
  • Peter C. Burger
    • Department of Pathology, Division of Neuropathology, Johns Hopkins HospitalJohns Hopkins University
Original Paper

DOI: 10.1007/s00401-012-1037-x

Cite this article as:
Rodriguez, F.J., Perry, A., Rosenblum, M.K. et al. Acta Neuropathol (2012) 124: 627. doi:10.1007/s00401-012-1037-x
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Abstract

Rare, generally pediatric oligodendroglioma-like 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.

Keywords

OligodendrogliomaGlioneuronal1p1p19qBrainSpineLeptomeningesPediatric glioma

Supplementary material

401_2012_1037_MOESM1_ESM.doc (88 kb)
Supplementary material 1 (DOC 88 kb)

Copyright information

© Springer-Verlag 2012