Thirteen patients with medulloblastoma were admitted to the Clinica Puerta de Hierro (Department of Radiotherapy) between 1964 and 1977. Two subtypes of medulloblastoma (classical and desmoplastic) with different histologic pattern, age of onset, clinical manifestations and clinical course have been identified. Radical surgery does not increase overal survival. X-ray treatment should be applied according to the following principles: a) the central nervous system should be irradiated; b) dosage on the posterior cranial fossa over 5,000 rad (50 Gy); c) dosage on the neuroaxis under 2,000-3,000 rad (25-30 Gy). Tumor recurrences should be treated in order to obtain a more comfortable life and a prolonged survival. Association of chemotherapy to surgery and radiotherapy have yielded promising results.
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Children (Basel)
November 2024
Department of Interdisciplinary Medicine, University of Bari and Aldo Moro, 70124 Bari, Italy.
Background: Posterior fossa syndrome (PFS), also known as cerebellar mutism syndrome, occurs in about 25% of pediatric patients undergoing resection of a posterior cranial fossa medulloblastoma. It is characterized primarily by mutism or reduced/impaired speech and may include variable symptoms such as motor dysfunction (apraxia, ataxia, hypotonia), supranuclear cranial nerve palsies, neurocognitive changes, and emotional lability. Long-term multidisciplinary rehabilitation is typically required, with recovery taking approximately six months, though many children experience long-term residual deficits.
View Article and Find Full Text PDFJ Neurosurg Case Lessons
January 2025
Department of Neurosurgery, Children's Hospital of Nanjing Medical University, Nanjing, Jiangsu, China.
Background: The diagnosis of intracranial extraosseous Ewing's sarcoma (EES) poses challenges due to the absence of specific clinical and imaging features prior to surgery. It is crucial to differentiate the tumor from other small round cell malignancies postoperatively.
Observations: A 7-year-old patient was admitted to the authors' hospital due to the in situ recurrence of a posterior fossa tumor more than 1 month after the initial surgery for headache.
J Pers Med
November 2024
Department of Radiation Oncology, Miulli General Regional Hospital, Acquaviva delle Fonti, 70021 Bari, Italy.
. Adult medulloblastoma (AMB) patients should receive postoperative craniospinal irradiation (CSI) as a standard treatment. Volumetric intensity-modulated arc therapy (VMAT) is a promising method for CSI.
View Article and Find Full Text PDFJ Med Case Rep
December 2024
UZ Leuven, Plastic, Reconstructive and Aesthetic Surgery, Herestraat 49, 3000, Louvain, Belgium.
Background: NeoDura (Medprin Biotech Gmbh) is an absorbable dural repair patch consisting of degradable poly-L-lactic acid and porcine gelatin that provides a hermetic closure of the dura mater (Medprin Biotech. Neodura. Dural Repair Patch [Brochure].
View Article and Find Full Text PDFActa Neuropathol Commun
December 2024
Michigan Center for Translational Pathology, University of Michigan, Ann Arbor, MI, USA.
We identified a rare heterozygous germline loss-of-function variant in the tumor necrosis factor receptor-associated factor 2 (TRAF2) in a young adult patient diagnosed with medulloblastoma. This variant is located within the TRAF-C domain of the E3 ubiquitin ligase protein and is predicted to diminish the binding affinity of TRAF2 to upstream receptors and associated adaptor proteins. Integrative genomics revealed a biallelic loss of TRAF2 via partial copy-neutral loss-of-heterozygosity of 9q in the medulloblastoma genome.
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