Primary disseminated leptomeningeal oligodendroglioma with 1p deletion

Abstract

The authors report the case of a 2-year-old boy with a primary, diffuse leptomeningeal oligodendroglioma in which the deletion of chromosome arm lp was identified by performing a fluorescence in situ hybridization (FISH) analysis. This previously healthy child initially presented with malaise, anorexia, nausea, vomiting, and macrocephaly. Imaging studies confirmed the presence of hydrocephalus, and a ventriculoperitoneal shunt was placed. The postoperative course was complicated by emesis, continued weight loss, and numerous seizurelike episodes. A contrast-enhanced magnetic resonance imaging study performed approximately 10 weeks postoperatively showed diffuse leptomeningeal thickening and enhancement without evidence of an intraparenchymal mass lesion. A right frontal lobe brain biopsy revealed a hypercellular proliferation of small oligodendroglioma-like cells, which occupied the leptomeninges diffusely and spared the underlying cortical gray matter. The tumor cells displayed prominent perinuclear clearing and had evenly spaced, uniformly round nuclei. Occasional mitotic figures were observed. Background vessels were thin and delicate, and there was no evidence of necrosis. The tumor cells showed strong immunoreactivity for S100 protein; the results of immunohistochemical staining were negative for glial fibrillary acidic protein, vimentin, epithelial membrane antigen, NeuN, and synaptophysin. The deletion of lp was demonstrated by FISH analysis; lq, 19p, and 19q were intact. This appears to be the first reported case of a primary diffuse leptomeningeal oligodendroglioma in which a lp deletion was identified.

Disseminated oligodendroglial-like leptomeningeal tumor with anaplastic progression and presumed extraneural disease: case report

“…Low-grade neoplasms diffusely and primarily involving the leptomeninges have been reported sporadically throughout the literature [3][4][5][6][7][8][9][10], with recent case series examining genetic, histopathologic, radiographic, and clinical characteristics [1,2]. Although they occur predominately in pediatric populations, similar neoplasms have been reported in adults [11,12].…”

Primary Leptomeningeal Oligodendroglioma, IDH-Mutant, 1p/19q-Codeleted

“…The intraoperative observations of the neurosurgeon (solid tumor without an identifiable connection to brain parenchyma) and the results of the assessment of the resected tissue (as detailed above) indicate that this is an example of primary leptomeningeal oligodendroglioma (2–4). The other entity in the differential diagnosis is diffuse leptomeningeal glioneuronal tumor (DLGT), which very rarely can show combined 1p/19q codeletion (isolated 1p deletion is more common).…”

Primary leptomeningeal anaplastic oligodendroglioma with a 1p36–19q13 deletion: Report of a unique case successfully treated with Temozolomide

“…Autopsy revealed a leptomeningeal oligodendroglioma. Bourne et al [10] reported the first patient with a primary leptomeningeal oligodendroglioma harbouring a 1p deletion. The patient remained clinically stable after 6 cycles of chemotherapy with Cisplatin, Vincristine, Cyclophosphamide and Etoposide.…”

“…Less than 50 cases have been reported [2], mostly astrocytomas and glioblastomas [3]. A literature review revealed only 9 cases of a primary leptomeningeal oligodendroglioma [3][4][5][6][7][8][9][10][11] ( Table 1). Diagnosis was based on the biopsy of contrast-enhancing areas, but sometimes definite diagnosis was established only at autopsy [9].…”