{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T16:24:50Z","timestamp":1787761490247,"version":"build-2784847793"},"reference-count":27,"publisher":"SAGE Publications","issue":"4","license":[{"start":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T00:00:00Z","timestamp":1654041600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Int J Surg Pathol"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:p>Hemangioblastoma, one of the characteristic tumors associated with Von Hippel-Lindau (VHL) disease, most often presents in the central nervous system (CNS) but can uncommonly arise in extraneuraxial, or previously referred to as peripheral, locations. Without the clinical context of known VHL disease, hemangioblastoma may not enter the differential for a soft tissue mass outside the CNS. Here, we present two patients with diagnostically challenging extraneuraxial hemangioblastoma to highlight the importance of considering this entity within the differential diagnosis of soft tissue neoplasms containing clear cells and delicate vasculature. We review the relevant diagnostic features, including a suggested immunohistochemical panel, along with the potential associated clinical implications of making this diagnosis. It is recommended that affected patients be offered genetic counseling to assess for underlying VHL disease.<\/jats:p>","DOI":"10.1177\/10668969221102560","type":"journal-article","created":{"date-parts":[[2022,6,2]],"date-time":"2022-06-02T01:15:16Z","timestamp":1654132516000},"page":"419-426","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":2,"title":["Extraneuraxial Hemangioblastoma: An Unusual Soft Tissue Neoplasm that Mimics More Common Entities but Carries Distinct Clinical Implications"],"prefix":"10.1177","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5180-1527","authenticated-orcid":false,"given":"Robert T.","family":"Chung","sequence":"first","affiliation":[{"name":"Geisel School of Medicine at Dartmouth"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yvonne Y.","family":"Cheung","sequence":"additional","affiliation":[{"name":"Geisel School of Medicine at Dartmouth"},{"name":"Dartmouth-Hitchcock Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric R.","family":"Henderson","sequence":"additional","affiliation":[{"name":"Geisel School of Medicine at Dartmouth"},{"name":"Dartmouth-Hitchcock Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konstantinos","family":"Linos","sequence":"additional","affiliation":[{"name":"Geisel School of Medicine at Dartmouth"},{"name":"Dartmouth-Hitchcock Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3228-2044","authenticated-orcid":false,"given":"Darcy A.","family":"Kerr","sequence":"additional","affiliation":[{"name":"Geisel School of Medicine at Dartmouth"},{"name":"Dartmouth-Hitchcock Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2022,6,1]]},"reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10689-004-5347-6"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/s101430050024"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.15586\/jkcvhl.2017.88"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1097\/pap.0000000000000176"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2021.07.123"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.prp.2018.05.007"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182a266c1"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.300.11.1334"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.3171\/2014.1.jns131431"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182064d11"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.3800351"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1177\/1066896915579198"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2613.2007.00535.x"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1007\/bf00688119"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000170343.09562.39"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41379-021-00782-1"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2014.109"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1097\/pas.0000000000000398"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1097\/PAI.0b013e318244ff4b"},{"issue":"8","key":"e_1_3_2_21_2","first-page":"4498","article-title":"Hemangioblastoma: clinicopathologic study of 42 cases with emphasis on TFE3 expression","volume":"12","author":"Yang Y","year":"2020","unstructured":"Yang Y, Gao H, Zhen T, et al. Hemangioblastoma: clinicopathologic study of 42 cases with emphasis on TFE3 expression. Am J Transl Res. 2020;12(8):4498\u20104510.","journal-title":"Am J Transl Res"},{"issue":"12","key":"e_1_3_2_22_2","first-page":"4380","article-title":"Histopathologic characteristics and immunotypes of perivascular epithelioid cell tumors (PEComa)","volume":"12","author":"Bao L","year":"2019","unstructured":"Bao L, Shi Y, Zhong J, et al. Histopathologic characteristics and immunotypes of perivascular epithelioid cell tumors (PEComa). Int J Clin Exp Pathol. 2019;12(12):4380\u20104389.","journal-title":"Int J Clin Exp Pathol"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1582-4934.2001.tb00165.x"},{"issue":"11","key":"e_1_3_2_24_2","first-page":"1209","article-title":"A review of the histology, ultrastructure, immunohistology, and molecular biology of extra-adrenal paragangliomas","volume":"113","author":"Kliewer KE","unstructured":"Kliewer KE, Cochran AJ. 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