Gallbladder carcinosarcoma (GBCS) is a rare and aggressive malignancy with extremely poor prognosis. Although surgery is regarded as the primary therapy for GBCS, the effective therapeutic strategies for unresected lesions have been poorly defined.

We presented a case of a 74-year-old male who underwent radical resection of gallbladder carcinoma at a local hospital. Seven months later, he was admitted to our hospital due to right upper abdominal discomfort. Postoperative radiological examinations showed multiple hepatic lesions, hilar lymph node metastasis, and main portal vein tumor thrombus. The pathological consultation results confirmed GBCS and immunohistochemical examinations revealed PD-L1 expression in 20% of tumor cells. Then, the patient received chemotherapy (Gemcitabine plus Oxaliplatin, GEMOX) in combination with anti-PD-1 therapy. After nine courses of the combination therapy, complete regression of the tumors was achieved with no evidence of relapse till now.

We, for the first time, reported a patient with recurrent GBCS who benefited from the combined chemotherapy and immunotherapy, providing a potential effective management strategy for the refractory malignant tumor.

• Carcinosarcoma
• Gallbladder
• Multidisciplinary therapy
• Surgical resection

• Carcinoma
• Carcinosarcoma
• Gallbladder
• Gallstone
• Malignancy
• Sarcoma

• Carcinosarcoma
• epithelial
• gallbladder
• mesenchymal
• treatment

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Gallbladder carcinosarcoma is a rare cancer with a worldwide prevalence of less than 1%.

Carcinosarcoma is an extremely rare subtype of gallbladder cancer with a worldwide prevalence of less than 1%. Carcinosarcomas are composed of a mixture of epithelial and mesenchymal components. Preoperative diagnosis of carcinosarcoma is challenging considering the non-specific clinical presentation and radiological findings.

A 63 years old female presented with right hypochondrium abdominal pain for 6 months. CA 19.9 level was high. Computed tomography of abdomen showed gallstones with enhancing wall thickening, along with enlarged portacaval lymph node. Endoscopic ultrasound-guided biopsy of porta hepatis lymph node was done which showed moderately differentiated adenocarcinoma. Hence, she underwent extended cholecystectomy with extended porta hepatis lymph node dissection. Final histopathological result showed carcinosarcoma (pT2a, pN1 M0). Unfortunately, she passed away 7 months post-operatively.

The majority of patients with carcinosarcomas are asymptomatic but can develop unspecific symptoms in advanced stages. The diagnosis is usually made incidentally on histopathological examination. Surgical resection is the mainstay treatment and longer survival can be achieved if combined with chemotherapy and radiation therapy. BILCAP trial suggested that capecitabine can improve survival in patients with gallbladder cancer. Unfortunately, even with these treatment modalities prognosis remains poor with a survival rate ranging from 2.9 to 6 months.

Gallbladder carcinosarcoma is a rare tumor with a poor prognosis. However, some cases reported a good survival rate after surgery. Further research is needed to understand the behavior of these tumors well and identify the role of chemotherapy and radiotherapy and which patients would benefit the most from surgery.

• Adenocarcinoma
• BILCAP
• Cancer
• Carcinosarcoma
• Case report
• GBCS
• Gallbladder
• Sarcomatoid

• Cholangiocarcinoma
• Neuroendocrine tumor
• Primary hepatic MiNEN