Association between papillary cystadenoma of the epididymis (PCE) and Von Hippel-Lindau Disease (VHLD) is well known and stronger for bilateral tumours. 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 young.

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

Miscia, Maria Enrica
Primo
;
Di Renzo, Dacia
Secondo
;
Chiesa, Pierluigi Lelli
Ultimo
2018-01-01

Abstract

Association between papillary cystadenoma of the epididymis (PCE) and Von Hippel-Lindau Disease (VHLD) is well known and stronger for bilateral tumours. 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 young.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11564/692968
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