A Rare Case of Multiple Papillary Cystadenomas Found in a Female Patient with Von Hippel-Lindau Syndrome

Papillary cystadenoma (PC) is a benign epithelial tumor believed to be of mesonephric origin and is recognized as one of von Hippel-Lindau syndrome (VHL)-related neoplasms. PC tends to develop frequently in male VHL patients but rarely in females and arises from the mesosalpinx or broad ligament in females. Having experienced a case of multiple PCs in a female patient, we here report it with literature review. The patient was a 34-year-old woman. In her past medical history, VHL including multiple hemangioblastomas of the central nervous system, pancreatic neuroendocrine tumor, and multiple clear cell renal cell carcinoma (CCRCC) of the bilateral kidneys was diagnosed. In addition, a PC of the left ovary was resected seven years earlier. In the present report, she was admitted to our hospital because of lower abdominal pain, and then computed tomography revealed swelling of the right ovary and ascites. She was diagnosed with right ovarian torsion and thus salpingo-oophorectomy was performed. Histopathologically, while the tumor cells with pale or eosinophilic cytoplasm showed a papillary growth with partial cystic changes, none of apparent nuclear atypia, mitotic figure, and necrosis were observed. We finally diagnosed it as PC of the right ovary. Its pathological findings were similar to those of the previously resected left ovarian lesion. Although PC is rare in females, the presence of VHL should be kept in mind when PC is encountered. Moreover, metastatic CCRCC should also be considered to be one of differential diagnoses in VHL patients.