extraneural hemangioblastoma
Hemangioblastoma is a morphologically distinctive tumor that can occur sporadically or in association with von Hippel-Lindau disease, and which involves the central nervous system in the majority of the cases (intraneural hemangioblastomas.
Rare occurrences of hemangioblastoma in peripheral nerves and extraneural tissues have been reported.
The histogenesis of this tumor remains uncertain. Various cell lineages such as vascular, glial, neural, fibrohistiocytic, and smooth muscle/myofibroblastic have been proposed for the so-called stromal cells, which are thought to represent the neoplastic component of these lesions.
The "stromal" cells of hemangioblastoma can demonstrate a variety of mature specific lineages, such as smooth muscle/myofibroblastic, or neuroendocrine, depending on the location and possibly the microenvironment.
Localization
presacral region
maxilla
kidney
adrenal glands
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#