1/3 associated with von Hippel-Lindau disease, in which cases tumors are often bilateral.
~1/5 of patients with von Hippel-Lindau disease have PCE.
Usually occurs in younger adult males.
Originates from the efferent ductules of the head of the epididymis.
Mainly present with a slowly growing painless mass.
Gross: Well circumscribed masses measuring 1-5 cm; may appear solid or cystic.
Intracystic papillary infoldings lined by columnar cells with abundant clear cytoplasm (image A) & (image B).
May resemble clear cell RCC because of the clear cytoplasm.