Systematic review of squamous cell carcinoma of the gallbladder.

Background: Although gallbladder cancer is the most common biliary tract malignancy, squamous cell carcinoma of the gallbladder (GBSCC) is extremely uncommon, comprising approximately 1-4% of all malignant gallbladder tumors. Given its rare incidence, there are currently no established treatment guidelines for GBSCC.

Methods: We reviewed the current data available through a comprehensive search of PubMed/MEDLINE and Embase.

Results: Although the clinical presentations of GBSCC and gallbladder adenocarcinoma (GBAC) are similar, GBSCCs are oftentimes larger and present with a higher histologic grade and more advanced pathological stage. Due to these aggressive features, the overall prognosis of GBSCC is significantly worse than GBAC, even after R0 resection.

Conclusion: A combination of radical cholecystectomy with negative surgical margins along with systemic chemotherapy and/or radiotherapy appears to be the best treatment strategy based on the current limited literature. Mutational profiling using next-generation sequencing (NGS) can help clinicians identify and treat actionable mutations of this rare tumor.