Primary Leptomeningeal Medulloblastoma in Adults: A Diagnostic Challenge—Case Report and Systematic Review

Abstract: Primary leptomeningeal medulloblastoma (PL-MB) in adults is a rare disease with a severe prognosis. A 35-year-old woman presented with headaches, diplopia, and gait ataxia, with triventricular hydrocephalus and descent of the cerebellar tonsils beyond the foramen magnum. Endoscopic third ventriculostomy was performed. Six months later, headaches recurred. Dilatation of the supratentorial ventricular system and massive cerebellar swelling without contrast-enhancing nodularities were reported. Occipitocervical decompression with duraplasty was performed. A bioptic diagnosis of PL-MB was made. Craniospinal irradiation and chemotherapy were administered. After 18 months, no recurrence was observed. Few cases of PL-MB have been reported: patients die before treatment or within a few days after surgery. Our long-term survival could be ascribable to a slow clinical presentation and an early diagnosis that allowed surgical treatment and the administration of a combined chemoradiotherapy protocol. Cerebellar swelling, even without associated enhancing lesions, with or without hydrocephalus, should be a neuroradiological alarm sign, and PL-MB should be considered.

Location
Deutsche Nationalbibliothek Frankfurt am Main
Extent
Online-Ressource
Language
Englisch

Bibliographic citation
Primary Leptomeningeal Medulloblastoma in Adults: A Diagnostic Challenge—Case Report and Systematic Review ; volume:17 ; number:04 ; year:2022 ; pages:651-655
Asian journal of neurosurgery ; 17, Heft 04 (2022), 651-655

Contributor
Fabbro, Sara
Pegolo, Enrico
Piccolo, Daniele
Cramaro, Antonio
Mascarin, Maurizio
Coassin, Elisa
Skrap, Miran
Tuniz, Francesco

DOI
10.1055/s-0042-1757726
URN
urn:nbn:de:101:1-2023020910034530841529
Rights
Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
Last update
14.08.2025, 10:49 AM CEST

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Associated

  • Fabbro, Sara
  • Pegolo, Enrico
  • Piccolo, Daniele
  • Cramaro, Antonio
  • Mascarin, Maurizio
  • Coassin, Elisa
  • Skrap, Miran
  • Tuniz, Francesco

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