Disseminated oligodendroglial cell-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity
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- 作者：Matthias Preuss ; Holger Christiansen ; Andreas Merkenschlager…
- 关键词：Diffuse leptomeningeal neuroepithelial tumor ; Leptomeningeal gliomatosis ; Disseminated oligodendroglial ; like leptomeningeal tumor ; Diffuse leptomeningeal glioneuronal tumor ; Hydrocephalus
- 刊名：Journal of Neuro-Oncology
- 出版年：2015
- 出版时间：August 2015
- 年：2015
- 卷：124
- 期：1
- 页码：65-74
- 全文大小：2,569 KB
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Holger Christiansen (1)
Andreas Merkenschlager (2)
Franz Wolfgang Hirsch (3)
Wieland Kiess (4)
Wolf Müller (5)
Stefanie K?stner (6)
Andreas Henssler (8)
Arnulf Pekrun (7)
Holger Hauch (9)
Michaela Nathrath (10)
Jürgen Meixensberger (12)
Torsten Pietsch (11)
Klaus Kuchelmeister (11)
12. Department of Neurosurgery, University Leipzig, Liebigstrasse 20, 04103, Leipzig, Germany
1. Division of Pediatric Oncology and Haematology, University Leipzig, Leipzig, Germany
2. Division of Neuropediatrics, University Leipzig, Leipzig, Germany
3. Department Pediatric Radiology, University Leipzig, Leipzig, Germany
4. Department of Children’s and Adolescence Health, University Leipzig, Leipzig, Germany
5. Insitute of Neuropathology, University Leipzig, Leipzig, Germany
6. Department of Neurosurgery, Klinikum Nordhessen GmbH, Kassel, Germany
8. Department of Neurosurgery, Klinikum Bremen-Mitte, Bremen, Germany
7. Department of Pediatric Hematology and Oncology, Klinikum Bremen-Mitte, Bremen, Germany
9. Department of Pediatric Hematology and Oncology, University Gie?en, Giessen, Germany
10. Department of Pediatric Hematology and Oncology, Klinikum Nordhessen GmbH, Kassel, Germany
11. Institute of Neuropathology, University Bonn, Bonn, Germany - 刊物类别：Medicine
- 刊物主题：Medicine & Public Health
Oncology - 出版者：Springer Netherlands
- ISSN：1573-7373
文摘
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.