Extraneuraxial Hemangioblastoma: Clinicopathologic Features and Review of the Literature

摘要: Extraneuraxial hemangioblastoma occurs in nervous paraneuraxial structures, somatic tissues, and visceral organs, as part of von Hippel-Lindau disease (VHLD) or sporadic cases. The VHL gene plausibly plays a key role the initiation tumorigenesis both central system extraneuraxial hemangioblastoma, therefore, underlying molecular genetic mechanisms tumor growth are initially reviewed. clinical criteria for diagnosis VHLD summarized, with emphasis on distinction from form fruste (eg, hemangioblastoma-only VHLD). world literature topic hemangioblastomas has been comprehensively reviewed ∼200 cases reported to date: up 140 paraneuraxial, mostly proximal spinal nerve roots, 65 peripheral, 15 soft tissue, 6 peripheral nerve, 5 bone, 39 internal viscera, including 26 renal 13 nonrenal. A handful possible yet uncertain older not included this review. clinicopathologic features selectively presented by anatomic site origin, differential is emphasized these subsets. Reference made also 10 authors' personal hemangioblastomas, which include 4 (2 tissue renal).