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.

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.

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.

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.

A granuloma is a discrete collection of activated macrophages and other inflammatory cells. Hepatic granulomas can be a manifestation of localized liver disease or be a part of a systemic process, usually infectious or autoimmune. A liver biopsy is required for the detection and evaluation of granulomatous liver diseases. The prevalence of granulomas on liver biopsy varies from 1% to 15%. They may be an incidental finding in an asymptomatic individual, or they may represent granulomatous hepatitis with potential to progress to liver failure, or in chronic disease, to cirrhosis. This review focuses on pathogenesis, histological features of granulomatous liver diseases, and most common etiologies, knowledge that is essential for timely diagnosis and intervention.

Hepatobiliary tuberculosis is a challenging disease that poses diagnostic difficulties due to its resemblance to other etiologies. Delayed diagnosis may lead to inadequate treatment, thus necessitating an urgent need for accurate diagnosis and appropriate management.

To systematically review case reports on hepatobiliary tuberculosis, focusing on symptomatology, diagnostic procedures, management, and outcomes to provide patient safety and ensure an uneventful recovery.

A systematic search was conducted on PubMed from 1992 to 2022, using keywords such as hepatobiliary, liver, tuberculosis cholangitis, cholangiopathy, and mycobacterium. Only case reports or case series in English were included in the study, and research papers published as abstracts were excluded. The search yielded a total of 132 cases, which were further narrowed down to 17 case studies, consisting of 24 cases of hepatobiliary tuberculosis.

The 10 most common symptoms observed in these cases were fever, abdominal pain, weight loss, jaundice, anorexia, generalized weakness, pruritus, chills, fatigue, and chest pains. Objective findings in these cases included hepatomegaly, hepatic nodules, elevated liver enzymes, and elevated bilirubin. Computed tomography scan and ultrasound of the abdomen were the most useful diagnostic tools reported. Histologic demonstration of Mycobacterium tuberculosis confirmed the cases of hepatobiliary tuberculosis. Treatment regimens commonly used included Rifampicin, Isoniazid, Pyrazinamide, and Ethambutol. Out of the 24 cases, 18 presented improvements while 4 had completely recovered.

Hepatobiliary tuberculosis is a disease that requires accurate diagnosis and appropriate management to avoid complications.

Hepatic granulomas can have various causes and their detection requires a systematic diagnostic evaluation. First, identification of risk factors for granulomatous diseases and the exclusion of extrahepatic organ manifestation are necessary. Laboratory investigations and serological screening for the most common underlying diseases of liver granulomas in Germany, such as primary biliary cholangitis (PBC), sarcoidosis and infectious causes (primarily tuberculosis and hepatitis C infections), are recommended. A liver biopsy is essential for confirming the diagnosis, whereby a minilaparoscopically guided tissue sampling offers many advantages, such as the macroscopic detection of granulomas on the liver surface, on the peritoneum or on the spleen. Whether the detection of hepatic granulomas results in a therapeutic consequence, depends decisively on the underlying primary disease. If hepatic granulomas are present without concomitant liver parenchymal damage or other manifestations that would make treatment necessary, a watch and wait approach under close clinical and laboratory monitoring is sufficient. If liver values increase or in cases of hepatic parenchymal damage, urgent treatment of the underlying disease is indicated.