VHLDVon Hippel-Lindau Disease
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Papillary cystadenoma bears a striking histologic resemblance to recently described clear cell papillary renal carcinoma and should not be mistaken for its malignant counterpart clear cell renal carcinoma especially in vHLD patients that may already carry a diagnosis of renal cell carcinoma like our patient.
In 1995, the cells of some sporadic PCEs were shown to harbor somatic VHLD gene mutations.
Our understanding of the pathogenesis of PCE has been greatly enhanced by 2 recent studies of epididymides from patients with VHLD. (22,23) These elegant studies have demonstrated the presence of precursor lesions confined to the efferent ductules of the head of the epididymis.
Both may occur in patients with VHLD. The mean age (37 years) of patients with renal cell carcinoma in VHLD is similar to the mean age of occurrence of PCE (35 years, our analysis).
The possibility of VHLD should be considered in all patients with PCE, since they may be at risk of developing other VHLD-associated tumors.
Papillary cystadenoma of the epididymis is a rare benign neoplasm of the epididymis, remarkable for its frequent association with VHLD and its morphologic similarity to clear cell RCC.