Multinodular and vacuolating neuronal tumor associated with focal cortical dysplasia in a child with refractory epilepsy: a case report and brief review of literature
Multinodular and vacuolating neuronal tumor (MVNT) is a rare newly identified benign lesion, firstly included in the World Health Organization classification of tumors of the central nervous system in 2016, whose neoplastic or dysplastic nature remains unclear but with a distinctive cytoarchitectural pattern and radiological features. It is usually discovered as late-onset refractory epilepsy, headache related, or an incidental lesion of adulthood. As it is typically a stable disease, many opt for follow-up, as long as it keeps remaining asymptomatic, leaving surgery for refractory epilepsy, neurological deficits, or intracranial hypertension symptoms. A subtotal or complete resection seems to control seizures and neurological manifestations. We herein present the case of a child with refractory epilepsy related to MVNT and focal cortical dysplasia, a dual pathology case in a less frequent age group and without the typical radiological imaging. We report its radiologic features, histologic description, and management, and we present a brief literature review on MVNT focusing on the pediatric cases reported. MVNT should now be another probable low-grade epilepsy-associated lesion (LEAT) in patients of all ages, with a benign and stable course as it constitutes a curable cause of focal epilepsy. As all the refractory cases, surgery should be indicated after a comprehensive evaluation of a multidisciplinary epilepsy surgery team.
KeywordsMultinodular and vacuolation neuronal tumor Epilepsy MVNT CNS tumors Developmental tumor Epilepsy surgery
We would like to acknowledge and thank Andrés Morales La Madrid, from the Oncology Department of Sant Joan de Déu Barcelona Children’s Hospital; Teresa Ribalta, from the Pathology Department of Sant Joan de Déu Barcelona Children’s Hospital; and Alia Ramírez-Camacho, Carlos Valera, Cinzia Lavarino, Victoria San Antonio-Arce, and Jordi Rumiá, all from the Epilepsy Surgery Unit of Sant Joan de Déu Barcelona Children’s Hospital, for their contribution in the collection of clinical information, and in the clinical management of and editing of the text.
We are indebted to the “Biobanc de l’Hospital Infantil Sant Joan de Déu per a la Investigació” integrated in the Spanish Biobank Network of ISCIII for the sample and data procurement.
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
Informed consent was obtained from the individual participant included in the study.
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