Capillary hemangioblastomas are benign tumors, mostly observed in the central nervous system (intraneural hemangioblastoma). However, it can be observed elsewhere (extraneural hemangioblastoma).
It is a tumor of unknown histogenesis that arises primarily in the posterior cranial fossa, either as a sporadic event or in association with von Hippel-Lindau disease (VHL).
Capillary hemangioblastomas of the central nervous system are benign tumors and occur either sporadically or in 25% of cases as a manifestation of von Hippel-Lindau (VHL) disease. VHL alleles are reported to be inactivated in up to 50% of sporadic and 100% in VHL-associated hemangioblastomas.
A rarer cellular and a more common reticular variant can be distinguished on the basis of the abundance of the stromal cell component, with the cellular variant being significantly associated with a greater probability of recurrence.
They are characterized histologically by the 2 main components of large vacuolated stromal cells and a rich capillary network and can be subclassified into 2 variants on the basis of the abundance of the stromal cell component:
the rarer cellular hemangioblastomas defined by zellballen-like cellular clusters of uniform tumor cells (11.4%)
the more common reticular subtype showing abundant capillaries and stromal cells.
CGH (16949923)
Losses of
Gains
- Chr.4 gain
Cellular and reticular variants of hemangioblastoma differ in their cytogenetic profiles (16949923).
Molecular biology
3p25 LOH: VHL locus (15535134)
6q LOH (15535134, 11943349)
22q13 LOH (15343513)
References
Nonaka D, Rodriguez J, Rosai J. Extraneural Hemangioblastoma: A Report of 5 Cases. Am J Surg Pathol. 2007 Oct;31(10):1545-1551. PMID: 17895756
Rickert CH, Hasselblatt M, Jeibmann A, Paulus W. Cellular and reticular variants of hemangioblastoma differ in their cytogenetic profiles. Hum Pathol. 2006 Nov;37(11):1452-7. PMID: 16949923
Patton KT, Satcher RL Jr, Laskin WB. Capillary hemangioblastoma of soft tissue: report of a case and review of the literature. Hum Pathol. 2005 Oct;36(10):1135-9. PMID: 16226115
Roy S, Chu A, Trojanowski JQ, Zhang PJ. D2-40, a novel monoclonal antibody against the M2A antigen as a marker to distinguish hemangioblastomas from renal cell carcinomas. Acta Neuropathol (Berl). 2005 May;109(5):497-502. PMID: 15864611
Michal M, Vanecek T, Sima R, Mukensnabl P, Boudova L, Brouckova M, Koudepa K. Primary Capillary Hemangioblastoma of Peripheral Soft Tissues. Am J Surg Pathol. 2004 Jul;28(7):962-966. PMID: 15223969