Original Paper

Acta Neuropathologica

, Volume 124, Issue 5, pp 627-641

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

  • Fausto J. RodriguezAffiliated withDepartment of Pathology, Division of Neuropathology, Johns Hopkins Hospital, Johns Hopkins University Email author 
  • , Arie PerryAffiliated withDepartment of Pathology, University of California San Francisco
  • , Marc K. RosenblumAffiliated withDepartment of Pathology, Memorial Sloan-Kettering Cancer Center
  • , Sherry KrawitzAffiliated withHealth Sciences Centre
  • , Kenneth J. CohenAffiliated withThe Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Johns Hopkins University
  • , Doris LinAffiliated withDivision of Neuroradiology, Johns Hopkins University
  • , Stacy MosierAffiliated withDepartment of Pathology, Johns Hopkins Hospital, Johns Hopkins University
  • , Ming-Tseh LinAffiliated withDepartment of Pathology, Johns Hopkins Hospital, Johns Hopkins University
  • , Charles G. EberhartAffiliated withDepartment of Pathology, Division of Neuropathology, Johns Hopkins Hospital, Johns Hopkins University
    • , Peter C. BurgerAffiliated withDepartment of Pathology, Division of Neuropathology, Johns Hopkins Hospital, Johns Hopkins University

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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

Oligodendroglioma Glioneuronal 1p 1p19q Brain Spine Leptomeninges Pediatric glioma