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- 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.
- Histology:
- May resemble clear cell RCC because of the clear cytoplasm.
- Excellent prognosis.