Coexpression of erythropoietin and its receptor in endolymphatic sac tumors

Timothy W.A. Vogel, Alexander O. Vortmeyer, Irina A. Lubensky, Youn Soo Lee, Makoto Furuta, Barbara Ikejiri, H. Jeffrey Kim, Russell R. Lonser, Edward H. Oldfield, Zhengping Zhuang

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10 Scopus citations


Object. Von Hippel-Lindau (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-associated hemangioblastomas (HBs) and renal cell carcinomas (RCCs) have indicated that VHL gene deficiency causes coexpression of erythropoietin (Epo) and its receptor (Epo-R), which facilitates tumor growth. Methods. 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-polymerase chain reaction and Western blot analysis. Conclusions. Expression of Epo appears to be a result of VHL 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-associated tumors indicates that VHL disease-associated tumors in different organs share common pathogenetic pathways.

Original languageEnglish (US)
Pages (from-to)284-288
Number of pages5
JournalJournal of neurosurgery
Issue number2
StatePublished - Aug 2005


  • Endolymphatic sac tumor
  • Erythropoietin
  • Erythropoietin receptor
  • Hemangioblastoma
  • Renal cell carcinoma
  • Tumorigenesis
  • Von Hippel-Lindau disease

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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    Vogel, T. W. A., Vortmeyer, A. O., Lubensky, I. A., Lee, Y. S., Furuta, M., Ikejiri, B., Kim, H. J., Lonser, R. R., Oldfield, E. H., & Zhuang, Z. (2005). Coexpression of erythropoietin and its receptor in endolymphatic sac tumors. Journal of neurosurgery, 103(2), 284-288.