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Review
Neuropathologic features of central nervous system hemangioblastoma
Rebecca A. Yoda, Patrick J. Cimino
J Pathol Transl Med. 2022;56(3):115-125.   Published online May 3, 2022
DOI: https://doi.org/10.4132/jptm.2022.04.13
  • 5,750 View
  • 252 Download
  • 4 Web of Science
  • 6 Crossref
AbstractAbstract PDF
Hemangioblastoma is a benign, highly vascularized neoplasm of the central nervous system (CNS). This tumor is associated with loss of function of the VHL gene and demonstrates frequent occurrence in von Hippel-Lindau (VHL) disease. While this entity is designated CNS World Health Organization grade 1, due to its predilection for the cerebellum, brainstem, and spinal cord, it is still an important cause of morbidity and mortality in affected patients. Recognition and accurate diagnosis of hemangioblastoma is essential for the practice of surgical neuropathology. Other CNS neoplasms, including several tumors associated with VHL disease, may present as histologic mimics, making diagnosis challenging. We outline key clinical and radiologic features, pathophysiology, treatment modalities, and prognostic information for hemangioblastoma, and provide a thorough review of the gross, microscopic, immunophenotypic, and molecular features used to guide diagnosis.

Citations

Citations to this article as recorded by  
  • Characterization of spinal hemangioblastomas in patients with and without von Hippel-Lindau, and YAP expression
    Ana-Laura Calderón-Garcidueñas, Steven-Andrés Piña-Ballantyne, Eunice-Jazmín Espinosa-Aguilar, Rebeca de Jesús Ramos-Sánchez
    Revista Española de Patología.2024;[Epub]     CrossRef
  • Case report: Hemangioblastoma in the brainstem of a dog
    Kirsten Landsgaard, Samantha St. Jean, Stephanie Lovell, Jonathan Levine, Christine Gremillion, Brian Summers, Raquel R. Rech
    Frontiers in Veterinary Science.2023;[Epub]     CrossRef
  • Intramedullary hemangioblastoma of the thoracic cord with a microsurgical approach: A case report and literature review
    Eduardo Cattapan Piovesan, Werner Petry Silva, Adroaldo Baseggio Mallmann, Felipe Severo Lanzini, Bruna Zanatta de Freitas, Francisco Costa Beber Lemanski, Charles André Carazzo
    Surgical Neurology International.2023; 14: 137.     CrossRef
  • Secondary Holocord Syringomyelia Associated With Spinal Hemangioblastoma in a 29-Year-Old Female
    Eric Chun-Pu Chu, Edouard Sabourdy, Benjamin Cheong
    Cureus.2023;[Epub]     CrossRef
  • Belzutifan in adults with VHL-associated central nervous system hemangioblastoma: a single-center experience
    Bryan J. Neth, Mason J. Webb, Jessica White, Joon H. Uhm, Pavel N. Pichurin, Ugur Sener
    Journal of Neuro-Oncology.2023; 164(1): 239.     CrossRef
  • Resection of Intramedullary Hemangioblastoma: Timing of Surgery and Its Impact on Neurological Outcome and Quality of Life
    Michael Schwake, Sarah Ricchizzi, Sophia Krahwinkel, Emanuele Maragno, Stephanie Schipmann, Walter Stummer, Marco Gallus, Markus Holling
    Medicina.2023; 59(9): 1611.     CrossRef
Original Article
Selective Neuronal Damage Produced by beta-fluoroethylacetate Intoxication in Rat Brain.
Ki Hyeong Lee, Beom Seok Jeon, Duk Lyul Na, Seong Ho Park, Je G Chi
Korean J Pathol. 1995;29(3):277-285.
  • 1,390 View
  • 12 Download
AbstractAbstract PDF
Beta-fluoroethylacetate has been extensively used as the rodenticide in Korea. In some patients with acute poisoning, beta-fluoroethylacetate caused cerebellar dysfunction as a single and persistent neurologic sequela after a period of an acute neurological disorder which is characterized by mental deterioration, seizures, and respiratory failure. But there has been no report of pathological findings to explain neurological deficit. We tried to verify the histologic changes of the central nervous systems in beta-fluoroethylacetate poisoned rats. Silver staining(Gallyas) was used to evaluate the histology. In acute intoxication experiment with LD50(7mg/Kg), beta-fluoroethylacetate elicited acute onset of consciousness deterioration, generalized tonic-clonic seizures and large amplitude tremulous activity involving whole body with full recovery after 24 hours. There was no discernible pathologic change in CNS in acutely poisoned rats. However, when poisoned with sublethal dose(5mg/Kg) daily for five days, a moderate degree of nerve cell degeneration was found selectively in dentate nucleus, Purkinie cell layer, vestibulo-cochlear nucleus and striatum. This change was not seen in hippocampus, cerebral cortex or cerebellar cortex. These findings were well correlated with the previous reports of selective pathology in human 5-FU intoxication cases. Our preliminary results suggest that beta-fluoroethylacetate, a kind of cellular metabolism inhibitor may induce selective neuropathology mainly involving cerebellar output pathway in rats.

J Pathol Transl Med : Journal of Pathology and Translational Medicine