Abstract
BACKGROUND: While papillary cystadenomas of the epididymis are relatively common, the occurrence of papillary cystadenomas in female individuals, particularly in the ovaries, remains exceedingly rare. CASE PRESENTATION: A 62-year-old white North African woman diagnosed with Von Hippel-Lindau disease in 2021 presented with multiple manifestations, including a left petrous bone tumor, left pheochromocytoma, left renal cell carcinoma, multi-cystic right kidney, and pancreatic masses. She underwent various treatments, including radiotherapy, adrenalectomy, nephrectomy, and cephalic duodenopancreatectomy. Ultrasonographic and magnetic resonance imaging examinations revealed a solid cystic mass in the left adnexal region. Laparoscopy identified cystic tumors in the right and left mesosalpinx. Following a hysterectomy with bilateral adnexectomy, histological examination revealed bilateral clear-cell papillary cystadenomas of the mesosalpinx and broad ligament, consistent with Von Hippel-Lindau disease. CONCLUSION: This case highlights the rare occurrence of bilateral clear-cell papillary cystadenomas of the mesosalpinx and broad ligament in a woman with Von Hippel-Lindau disease, a condition typically associated with men. The comprehensive diagnostic and therapeutic approach, including imaging studies, laparoscopy, and histological examination, underscores the importance of vigilant monitoring and tailored management in patients with this complex, multisystem disorder.