{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T23:04:14Z","timestamp":1790291054896,"version":"4.1.0"},"reference-count":27,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2005,8]]},"abstract":"<jats:p content-type=\"fine-print\"><jats:italic>Object.<\/jats:italic>Von Hippel\u2014Lindau (VHL) disease is characterized by multiple tumors in specific organs. The cell of origin and the reason for the particular organ distribution of the tumors remains unknown. Endolymphatic sac tumor (ELST) is one of the lesions associated with VHL disease. Data from previous studies of VHL disease\u2014associated hemangioblastomas (HBs) and renal cell carcinomas (RCCs) have indicated that<jats:italic>VHL<\/jats:italic>gene deficiency causes coexpression of erythropoietin (Epo) and its receptor (Epo-R), which facilitates tumor growth.<\/jats:p><jats:p content-type=\"fine-print\"><jats:italic>Methods.<\/jats:italic>The authors studied ELSTs from five patients with VHL germline mutations. Analysis of the five ELST samples revealed loss of the wild-type allele, consistent with Knudson's two-hit hypothesis for tumorigenesis. All five ELST specimens were characterized microscopically and by immunohistochemical analysis. Coexpression of Epo and Epo-R was found in all five tumors on immunohistochemical studies and confirmed through reverse transcription\u2014polymerase chain reaction and Western blot analysis.<\/jats:p><jats:p content-type=\"fine-print\"><jats:italic>Conclusions.<\/jats:italic>Expression of Epo appears to be a result of<jats:italic>VHL<\/jats:italic>gene deficiency, whereas the simultaneous coexpression of Epo-R may reflect a developmental mechanism of tumorigenesis. Coexpression of Epo and Epo-R in ELSTs together with the morphological and genetic similarities of these lesions with other VHL disease\u2014associated tumors indicates that VHL disease\u2014associated tumors in different organs share common pathogenetic pathways.<\/jats:p>","DOI":"10.3171\/jns.2005.103.2.0284","type":"journal-article","created":{"date-parts":[[2009,5,13]],"date-time":"2009-05-13T18:03:41Z","timestamp":1242237821000},"page":"284-288","source":"Crossref","is-referenced-by-count":7,"title":["Coexpression of erythropoietin and its receptor in endolymphatic sac tumors"],"prefix":"10.3171","volume":"103","author":[{"given":"Timothy W. 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