Disseminated oligodendroglial cell-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity
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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.
KeywordsDiffuse leptomeningeal neuroepithelial tumor Leptomeningeal gliomatosis Disseminated oligodendroglial-like leptomeningeal tumor Diffuse leptomeningeal glioneuronal tumor Hydrocephalus
The study group thanks the neuropathologic colleagues who provided the first biopsy results and granted reviewing of the specimens: Prof. Dr. med. Till Acker, Department of Neuropathology, University Gießen und Prof. Dr. med. Markus Bergmann, Department of Clinical Neuropathology, Klinikum Bremen-Mitte as well as Dr. Talal Aklan and Prof. Dr. Irina Berger, Department of Pathology, Klinikum Nordhessen GmbH, Kassel. Furthermore we want to thank Prof. Dr. Veit Braun, Department of Neurosurgery Siegen, for providing data and support of the study. The present study has not been funded or sponsored by third parties.
Conflict of interest
All authors have no conflict of interest in publication of this manuscript.
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