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

Fausto J. Rodriguez, Arie Perry, Marc K. Rosenblum, Sherry Krawitz, Kenneth J. Cohen, Doris Lin, Stacy Mosier, Ming Tseh Lin, Charles G. Eberhart, Peter C. Burger

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)627-641
Number of pages15
JournalActa neuropathologica
Volume124
Issue number5
DOIs
StatePublished - Nov 1 2012

Keywords

  • 1p
  • 1p19q
  • Brain
  • Glioneuronal
  • Leptomeninges
  • Oligodendroglioma
  • Pediatric glioma
  • Spine

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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