Hemangioblastoma
Overview
- Hemangioblastomas commonly present as an adult-onset, primary cerebellar neoplasm that is benign, G1, (thus, it's amenable to surgery), and has an important association with von Hippel-Lindau syndrome (VHL).
- In adults, hemangioblastoma is the most common primary cerebellar neoplasm.
- The cerebellar mass will often appear as a cyst with an enhancing mural nodule.
- Hemangioblastoma also commonly occurs in the brainstem or spinal cord (see: Spinal Cord Tumors).
- Retinal capillary hemangioblastomas (aka retinal angiomas) are a typical finding in VHL (less commonly sporadic).
Genetics
- Hemangioblastoma is associated with VHL in ~25% of cases (VHL gene, chromosome 3p25-26) but is most commonly sporadic.
EPO production
- Hemangioblastomas can produce erythropoietin, which leads to secondary polycythemia (we can predict this from its vascular histopathology).
Histopathology:
- Comprises both vascular channels and tumor nodule, thus appears as neoplastic cells in sheets interposed with closely-packed, thin-walled capillaries.
- Vascular channels comprise a dense network of closely-packed, innumerable thin-walled capillaries, hence "hemangio"-blastoma.
- Tumor nodule comprises foamy tumor cells, called stromal or interstitial cells.
- The tumor cells are large and filled with pinkish, foamy lipidized cytoplasm (contains PAS+ lipid-containing vacuoles).
- The mass is encapsulated and in addition to the neoplastic nodule (the tumor nodule) contains a cystic component, from cystic degeneration.
- Cerebellar cyst with an enhancing mural nodule in an adult, think: hemangioblastoma.
References
- Gray, Frangoise, Charles Duyckaerts, and Umberto De Girolami. Escourolle and Poirier’s Manual of Basic Neuropathology. OUP USA, 2013.
- Samuels, Martin A., Allan H. Ropper, and Joshua Klein. Adams and Victor’s Principles of Neurology 10th Edition. McGraw-Hill Education, 2014.
- Tonn, Jörg-Christian, Manfred Westphal, and J. T. Rutka. Oncology of CNS Tumors. Springer Science & Business Media, 2010.
- Yachnis, Anthony T., and Marie L. Rivera-Zengotita. Neuropathology E-Book: A Volume in the High Yield Pathology Series. Elsevier Health Sciences, 2012.
Image References
- MRI image of hemangioblastoma
- Image credited to Public Library of Science journal. (File:Hippel Lindau.gif) [CC-BY-SA-2.5 (http://creativecommons.org/licenses/by-sa/2.5) or CC-BY-2.5 (http://creativecommons.org/licenses/by/2.5)]
- Histopathologic slide of hemangioblastoma
- Nephron. English: High Magnification Micrograph of a Cerebellar Hemangioblastoma. HPS Stain. [object HTMLTableCellElement]. Own work. https://commons.wikimedia.org/wiki/File:Cerebellar_hemangioblastoma_high_mag.jpg.