Disseminated oligodendroglial cell-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity

Matthias Preuss, Holger Christiansen, Andreas Merkenschlager, Franz Wolfgang Hirsch, Wieland Kiess, Wolf Müller, Stefanie Kästner, Andreas Henssler, Arnulf Pekrun, Holger Hauch, Michaela Nathrath, Jürgen Meixensberger, Torsten Pietsch, Klaus Kuchelmeister

Research output: Contribution to journalArticlepeer-review

39 Scopus citations

Abstract

Pediatric tumors of the central nervous system composed of oligoid tumor cells showing diffuse leptomeningeal spread without a primary mass lesion seem to represent a novel tumor entity. The terms “diffuse leptomeningeal glioneural tumor” or—preferably—“disseminated oligodendroglial-like leptomeningeal tumor of childhood” (DOGLT) were proposed. Four patients were identified with clinico-neuropathologic findings compatible with DOGLT and a mean follow-up time of 54 months was determined. Seven different biopsies obtained from the four patients were histologically evaluated. Clinical course, diagnostic measures, histopathologic and radiologic features and treatment suggestions were recorded, on the basis of which diagnostic and therapeutic algorithm was proposed. Patients with DOGLT presented with hydrocephalus as first symptom, requiring neurosurgical therapy. Open arachnoid biopsy was necessary to confirm diagnosis. The oligoid cells in a desmoplastic or focally myxoid matrix showed OLIG2-, MAP2-, S-100 and rare HuC/HuD protein-immunopositivity. IDH1 (R132H)- and CD99-immunohistochemistry was negative in all patients. None of the evaluable biopsies of three patients showed chromosome 1p/19q deletion, neither as isolated nor combined allelic loss. Chemotherapy according to the SIOP-LGG 2004 standard induction and consolidation protocol resulted in complete response and partial response, respectively, in 50 % of the patients. However, after discontinuation of chemotherapy, two patients experienced tumor progression and one of them succumbed to the disease after 19 months. Radiological criteria as well as preliminary treatment results are presented after observation of four clinical cases. Prognosis and long-term clinical courses remain to be observed.

Original languageEnglish
Pages (from-to)65-74
Number of pages10
JournalJournal of Neuro-Oncology
Volume124
Issue number1
DOIs
StatePublished - 23 Aug 2015
Externally publishedYes

Keywords

  • Diffuse leptomeningeal glioneuronal tumor
  • Diffuse leptomeningeal neuroepithelial tumor
  • Disseminated oligodendroglial-like leptomeningeal tumor
  • Hydrocephalus
  • Leptomeningeal gliomatosis

Fingerprint

Dive into the research topics of 'Disseminated oligodendroglial cell-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity'. Together they form a unique fingerprint.

Cite this