title: Coexpression of erythropoietin and erythropoietin receptor in von Hippel-Lindau disease-associated renal cysts and renal cell carcinoma.
abstract: Von Hippel-Lindau (VHL) disease is characterized by multiple tumors in specific target organs. The tumors at different sites share distinct morphologic and genetic characteristics but their cell of origin is unknown. We show that VHL disease-associated renal clear cell carcinomas (RCC) consistently coexpress erythropoietin (Epo) and Epo receptor (EpoR). In addition, coexpression of Epo and EpoR is detected in many renal cysts, providing further evidence that renal cysts are potential precursors for RCC. In conjunction with VHL gene deficiency, coexpression of Epo and EpoR in renal cysts and tumors may reflect a developmental arrest in immature mesenchymal cells. Such arrest may lead to autocrine stimulation, cell proliferation, and renal tumor development, similar to tumorigenesis of VHL disease-associated hemangioblastomas.
authors.affiliation: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Cancer Institute/NIH, 10 Center Drive, Bethesda, MD 20892, USA.
authors.forename: Youn-Soo
authors.initials: YS
authors.lastname: Lee
authors.forename: Alexander O
authors.initials: AO
authors.lastname: Vortmeyer
authors.forename: Irina A
authors.initials: IA
authors.lastname: Lubensky
authors.forename: Timothy W A
authors.initials: TW
authors.lastname: Vogel
authors.forename: Barbara
authors.initials: B
authors.lastname: Ikejiri
authors.forename: Sophie
authors.initials: S
authors.lastname: Ferlicot
authors.forename: Gérard
authors.initials: G
authors.lastname: Benoît
authors.forename: Sophie
authors.initials: S
authors.lastname: Giraud
authors.forename: Edward H
authors.initials: EH
authors.lastname: Oldfield
authors.forename: W Marston
authors.initials: WM
authors.lastname: Linehan
authors.forename: Bin T
authors.initials: BT
authors.lastname: Teh
authors.forename: Stéphane
authors.initials: S
authors.lastname: Richard
authors.forename: Zhengping
authors.initials: Z
authors.lastname: Zhuang
mesh_terms: D015153:Blotting, Western; D002292:Carcinoma, Renal Cell / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D004921:Erythropoietin / Q000235:genetics* / Q000378:metabolism; D005260:Female; D015870:Gene Expression; D006801:Humans; D007150:Immunohistochemistry; D052177:Kidney Diseases, Cystic / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D007680:Kidney Neoplasms / Q000235:genetics / Q000473:pathology*; D008297:Male; D012334:RNA, Neoplasm / Q000235:genetics / Q000378:metabolism; D017467:Receptors, Erythropoietin / Q000235:genetics* / Q000378:metabolism; D020133:Reverse Transcriptase Polymerase Chain Reaction; D006623:von Hippel-Lindau Disease / Q000150:complications*
chemical_list: D012334:RNA, Neoplasm; D017467:Receptors, Erythropoietin; D004921:Erythropoietin