Diffuse leptomeningeal glioneuronal tumour: where to biopsy? Case report and literature review
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CASE REPORT
Diffuse leptomeningeal glioneuronal tumour: where to biopsy? Case report and literature review Miguel Sáez-Alegre 1 & Javier Manuel Saceda Gutiérrez 1 & Cristrina Utrilla Contreras 2 & Francisco Javier Aracil Santos 3 & Pablo García-Feijoo 1 & Fernando Carceller Benito 1 Received: 1 October 2020 / Accepted: 28 October 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Purpose Diffuse leptomeningeal glioneuronal tumour (DLGT) is an infrequent entity. Diagnosis is made with biopsy but with so few cases described management, prognosis remains undefined. There are not currently any articles regarding most effective place to biopsy. Methods Current literature review and introduction of the case of a 3-year-old male presenting at the emergency room with irritability, vomiting and nuchal rigidity. A head CT was made showing tetraventricular enlargement and a posterior fossa cyst. Results Patient underwent urgent ventriculoperitoneal shunting surgery with complete symptomp resolution. Brain MRI showed diffuse leptomeningeal enhancing, predominantly in basal cisterns, and multiple cystic-solid lesions along the neural axis. After ruling other conditions, a biopsy among intraoperative samples, was obtained of a PET positive gadolinium spinal enhancing lesion at D8 level, with the final diagnosis of DLGT. Conclusion DLGT can present as acute hydrocephalus. Biopsy stablishes the diagnosis but the place to take the sample can be difficult to select. Our experience suggests that PET-CT and intraoperative biopsy analysis can improve the effectivity of a representative sample. Keywords Diffuse leptomeningeal glioneuronal tumor . Paediatrics . Neurosurgery
Introduction Diffuse leptomeningeal glioneuronal tumour (DLGT), also previously known as disseminated oligodendroglial-like leptomeningeal tumour of the childhood, is a rare and newly listed entity in the WHO 2016 tumour classification [1, 2]. More common in the paediatric group, it can also occur in adult patients, predominantly in the young, with a light predilection towards males.
* Miguel Sáez-Alegre [email protected] 1
Department of Paediatric Neurosurgery, Hospital La Paz, Paseo de la Castellana, 261, 28046 Madrid, Spain
2
Department of Radiology, Hospital La Paz, Paseo de la Castellana, 261, 28046 Madrid, Spain
3
Department of Paediatrics, Hospital La Paz, Paseo de la Castellana, 261, 28046 Madrid, Spain
Radiologically it presents as a nodular leptomeningeal enhancing thickening, predominantly around the basal cisterns, extending all over the CNS [3]. Some specific findings have been related to this condition, such as the presence of small and multiple subpial cyst located in the inferior part of the cerebral hemispheres, posterior fossa and spinal cord. Although having this characteristic radiology findings, its rarity and the variable clinical presentation can lead to misdiagnosis. Cases have been reported mimicking tuberculosis [4], with basal cisterns enhancement and acute hydrocephalus. With headache, back and articu
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