title: Clear cell renal carcinoma presenting as a bleeding cyst in pregnancy: inaugural manifestation of a von Hippel-Lindau disease.
abstract: Von Hippel-Lindau (VHL) disease is a dominant autosomal disorder inducing the development of many tumors, such as hemangioblastomas in the central nervous system and retina, cysts or tumors (benign or malignant) in the kidneys and/or the pancreas. We report the case of a pregnant woman who presented with a voluminous hemorrhagic cyst of the right kidney with an exophytic lesion detected in the lower median part of the cyst wall. As an anamnestic inquiry resulted in a familial history of VHL disease, a screening imaging was performed and detected three medullary hemangioblastomas. Considering the active bleeding of the renal cyst and its potential malignancy, a unilateral nephrectomy was carried out after pregnancy interruption. Histological analysis confirmed a multilocular clear cell renal carcinoma. This case underlines the importance of screening procedures such as abdominal ultrasonography and medullary magnetic resonance imaging in all pregnant women with a familial history of VHL disease.
authors.affiliation: Department of Nephrology, Erasme Hospital, Universite Libre de Bruxelles, Brussels, Belgium.
authors.forename: I
authors.initials: I
authors.lastname: Simon
authors.forename: S
authors.initials: S
authors.lastname: Rorive
authors.forename: C
authors.initials: C
authors.lastname: Kirkpatrick
authors.forename: T
authors.initials: T
authors.lastname: Roumeguere
authors.forename: J L
authors.initials: JL
authors.lastname: Nortier
mesh_terms: D000328:Adult; D002292:Carcinoma, Renal Cell / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006417:Hematuria / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D006801:Humans; D052177:Kidney Diseases, Cystic / Q000175:diagnosis*; D007680:Kidney Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D008279:Magnetic Resonance Imaging; D009392:Nephrectomy / Q000379:methods; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000175:diagnosis