Unusual coexistence of low-grade mucinous cystic neoplasm and idiopathic granulomatous hepatitis.

Introduction And Importance: Mucinous cystic neoplasms (MCNs) of the liver are rare precancerous lesions, accounting for less than 5 % of all hepatic cysts. The coexistence of MCNs with idiopathic granulomatous hepatitis is exceedingly uncommon and lacks documentation in the existing literature.

Case Presentation: A 43-year-old Tunisian woman with no significant medical history presented with persistent right hypochondrium pain. Clinical examination revealed a palpable mass in the right hypochondrium. Laboratory tests indicated elevated liver enzymes and cholestasis. Imaging studies identified an 18 × 11 cm cystic formation in the right liver lobe, initially suspected to be a type I hydatid cyst. The patient underwent a right hepatectomy, and histological examination confirmed the presence of a low-grade MCN of the liver coexisting with idiopathic granulomatous hepatitis. The patient experienced an uncomplicated postoperative recovery.

Clinical Discussion: In our case, the concomitant presence of MCN and idiopathic granulomatous hepatitis was incidental, without any causal link. The definitive diagnosis of these two conditions relies on histopathological examination. It is essential to surgically remove the MCN and identify the cause of granulomatous hepatitis to effectively manage the patient.

Conclusion: This case underscores the uncommon occurrence of both MCN and idiopathic granulomatous hepatitis in the liver, highlighting the diagnostic challenges associated with the latter. Accurate diagnosis through thorough evaluation is essential for effective management.