A ganglioneuroma with areas of melanosis was resected from the cerebellum of a 6.5-year-old girl. At 2.5 years of age, she was diagnosed to have cerebellar neuroblastoma, which was incompletely resected and then radiated. Histologic, ultrastructural, and immunocytochemical studies undertaken on tissue from both stages of the tumor demonstrated a neuroblastic origin and differentiation into a predominantly neuronal tumor with limited astroglial participation. In addition, widespread deposition of basal lamina material, perineuronal distribution of S-100 protein-bearing cells and melanosis were found. The various features and unusual biology of the tumor are discussed in the light of a review of the literature.
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http://dx.doi.org/10.1002/1097-0142(19870101)59:1<69::aid-cncr2820590117>3.0.co;2-8 | DOI Listing |
Zh Nevrol Psikhiatr Im S S Korsakova
December 2024
Kemerovo State Medical University, Kemerovo, Russia.
Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder characterized by a combination of main symptoms: opsoclonus, myoclonus, ataxia, psychoemotional and behavioral disturbances. OMS can develop in children as a result of immunopathological processes against the background of infectious or oncological pathology and lead to persistent neurological deficit. A case of ten-year observation of paraneoplastic OMS associated with neuroblastoma in a child is presented.
View Article and Find Full Text PDFNeurobiol Dis
December 2024
Goethe University Frankfurt, University Hospital, Clinic of Neurology, Exp. Neurology, Heinrich Hoffmann Str. 7, 60590 Frankfurt am Main, Germany. Electronic address:
The autosomal recessive disease ataxia-telangiectasia (A-T) presents with cerebellar degeneration, immunodeficiency, radiosensitivity, capillary dilatations, and pulmonary infections. Most symptoms outside the nervous system can be explained by failures of the disease protein ATM as a Ser/Thr-kinase to coordinate DNA damage repair. However, ATM in adult neurons has cytoplasmic localization and vesicle association, where its roles remain unclear.
View Article and Find Full Text PDFbioRxiv
November 2024
Department of Cell Biology, Emory University, Atlanta, Georgia, USA, 30322.
Pediatr Neurol
November 2024
Department of Neurology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China. Electronic address:
Background: Anti-Purkinje cell cytoplasmic antibody type 2 (PCA-2) is associated with various neurological conditions in adults. However, related studies have not been conducted in children. The present study aimed to characterize the clinical features and outcomes of PCA-2-related autoimmune cerebellar degeneration in pediatric patients.
View Article and Find Full Text PDFBr J Cancer
September 2024
Hopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany.
Background: Certain paediatric nervous system malignancies have dismal prognoses. Retinoic acid (RA) is used in neuroblastoma treatment, and preclinical data indicate potential benefit in selected paediatric brain tumour entities. However, limited single-agent efficacy necessitates combination treatment approaches.
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