Unilateral Papillary Cystadenoma of the Epididymis as a First Presentation of Von Hippel-Lindau Disease

Association between papillary cystadenoma of the epididymis (PCE) and Von Hippel-Lindau Disease (VHLD) is well known and stronger for bilateral tumors. Unilateral PCE occurs either as a sporadic tumor without evidence of VHLD or in the context of a known diagnosis of VHLD, indeed it has never been r...

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Bibliographic Details
Published in:Urology (Ridgewood, N.J.) N.J.), 2018-08, Vol.118, p.189-191
Main Authors: Miscia, Maria Enrica, Di Renzo, Dacia, Persico, Antonello, Tarallo, Luciana, Chiesa, Pierluigi Lelli
Format: Article
Language:English
Subjects:
Adolescent
Cystadenoma, Papillary - etiology
Epididymis
Genital Neoplasms, Male - etiology
Humans
Male
von Hippel-Lindau Disease - complications
von Hippel-Lindau Disease - diagnosis
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Summary:Association between papillary cystadenoma of the epididymis (PCE) and Von Hippel-Lindau Disease (VHLD) is well known and stronger for bilateral tumors. Unilateral PCE occurs either as a sporadic tumor without evidence of VHLD or in the context of a known diagnosis of VHLD, indeed it has never been reported as the first manifestation of VHLD. In contrast, we report the case of a boy with an apparently isolated, unilateral PCE that resulted to be the first manifestation of an unknown VHLD. Thus, we recommend screening for VHLD in patients with a new diagnosis of unilateral PCE, especially if the patients are young.
ISSN:0090-4295
1527-9995
DOI:10.1016/j.urology.2018.04.025