Clinico-Radiological Presentation and Management of Gallbladder Paraganglioma: A Systematic Review

Gallbladder paraganglioma (GPG) is a rare neuroendocrine tumour discovered incidentally on final histopathological examination. The objective was to evaluate the clinical findings and management strategies using relevant literature for reported cases of gallbladder paraganglioma. A Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA)-guided literature search was conducted using data from PubMed, Scopus, and Google Scholar to identify the studies with histopathologically confirmed cases of GPG published between January 2000 and December 2024. Study quality was assessed using the Joanna Briggs Institute checklist, and data analysis was done using descriptive statistics and tabulations. A total of 13 case reports were selected, satisfying the inclusion and exclusion criteria. Among 13 reported cases, 10 (76.3%) were females. The mean age of the patients was 53.8 ± 12.59 years. Right hypochondrial pain was the most common presentation in seven (53.9%) cases, while three (23.1%) cases were asymptomatic. Ultrasound was the initial imaging modality in seven (53.9%) patients, followed by computed tomography scans in six (46.2%). Histopathology showed a nested pattern of chief and sustentacular cells in five (38.5%) cases. On immunohistochemistry, 11 (84.6%) cases were positive for synaptophysin A and nine (69.2%) were positive for chromogranin A. Laparoscopic cholecystectomy was the most common procedure performed in 10 (76.3%) cases. The average duration of follow-up was 66.5 ± 82.10 weeks, which was mentioned for only five patients. GPG should be considered in the differential diagnosis of gallbladder and biliary disorders presenting with features of acute cholecystitis and non-functional tumours. Patients often present with non-specific symptoms aligning with mass effects and cholecystitis. When a growth is suspected, it should be thoroughly evaluated before surgery to rule out other types of invasive gallbladder tumours. If investigations confirm a non-metastatic lesion, proceeding with a simple cholecystectomy is appropriate.