Introduction: Gallbladder carcinosarcoma (GBCS) is a rare neoplasm, and previous studies regarding to GBCS were case reports/case series, absence of large retrospective analyses, or systemic review. This review summarizes the current literature on accurate information of GBCS to assist clinicians to accurately diagnose and treat this malignancy.
Areas covered: The authors retrieved relevant documents of GBCS from PubMed and Medline. This review elaborates on the knowledge of GBCS covering epidemiology, potential mechanism, clinical manifestation, diagnosis, treatment, and prognosis.
Expert opinion: The majority of GBCS patients are easily misdiagnosed as GBC and usually treated as GBC. However, the biological behavior and outcome of GBCS is different from that of GBC. GBCS should be considered as a separate disease.