Literature ReviewDisseminated Oligodendroglial-like Leptomeningeal Tumor in the Adult: Case Report and Review of the Literature
Introduction
Disseminated leptomeningeal glioneuronal tumor (DLGNT) is a new entity introduced in the 2016 World Health Organization updated classification under neuronal and mixed neuronal-glial tumors.1 It is a rare entity that occurs more commonly in pediatric patients and is known in the literature under a variety of similar terms, perhaps most notably as disseminated oligodendroglial-like leptomeningeal tumor of childhood.2, 3, 4, 5, 6, 7 These tumors manifest with diffuse leptomeningeal involvement and an inconsistent recognizable parenchymal component. DLGNT is considered an indolent neoplasm characterized by periods of stabilization and slow progression; however, considerable morbidity has been reported.1 Despite recent recognition as a distinct pathologic entity, DLGNT is far from being clearly understood, and further characterization and collection of evidence are crucial. Despite the prevalent presentation in pediatric patients, occasional cases have been reported in adults. The biologic behavior of these tumors is still unclear, specifically in rare adult sporadic manifestations. We report a case of DLGNT in a 36-year-old woman and review the literature to highlight this very uncommon entity with the aim to provide further epidemiologic and biologic details of this tumor.
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Case Report
A 36-year-old woman presented with an acute onset of neck pain with irradiation at the shoulders bilaterally. At admission, she was experiencing nausea, vomiting, and syncopal episodes. Magnetic resonance imaging (MRI) of the brain revealed a mass located in the pontine and peribulbar cisterns with presumably multiple cystic and nodular extra-axial origins (Figure 1). The tumor was hypointense on T1 and hyperintense on T2/fluid attenuated inversion recovery MRI sequences, with heterogeneous and
Discussion
Gardiman et al.3 first hypothesized DLGNT as a new pediatric entity characterized by 3 distinctive characteristics: 1) intense enhancement of subarachnoid space with cystic lesions, 2) diffuse leptomeningeal infiltration by glioneuronal cells without a primary mass, and 3) quite indolent course. Following further studies, DLGNT was introduced in the recent World Health Organization classification.1 It is defined as a tumor that manifests with diffuse leptomeningeal disease, with or without a
Conclusions
DLGNTs should no longer be considered only as low-grade tumors affecting pediatric patients. The spectrum of presentations also includes aggressive tumors affecting adults. Further clinical and pathologic data supported by cytogenetic and molecular investigations are mandatory to better characterize this entity.
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.