Pure large cell neuroendocrine carcinoma of the gallbladder is a rare disease. However, the prognosis of this aggressive tumor is poor with short survival after diagnosis. We are describing in this manuscript a case of pure large cell neuroendocrine carcinoma in which survival exceeded 26 months, after performing two curative surgeries. We are reporting the case of a 68-year-old woman with a history of recovered right breast carcinoma and operated 6 years later for a completely asymptomatic gallbladder tumor of 31 mm. In this case, curative surgery was performed allowing monobloc resection of the gallbladder and the hepatic segments IVb and V, a lymph node dissection was performed to. The histological examination of the specimen and immunohistochemistry confirms that the tumor was a grade 3 pure large cell neuroendocrine carcinoma of gallbladder with lymph node invasion, the hepatic and biliary surgical margins were free. Postoperative adjuvant chemotherapy was administered and the evolution was eventless until the discovery at 20 months of a lymph node considered being metastatic recurrence. A second surgery was performed allowing removal of three lymph nodes. This time, a different protocol of chemotherapy was administered to our patient who remains alive and without recurrence at 26 months from her first surgery. Surgical relentlessness with free margins resections associated with appropriate chemotherapy probably improves the survival of patients suffering from this rare and aggressive tumor.

Surgical management of unresectable perihilar cholangiocarcinoma is no longer relevant. Often these patients benefit from less invasive endoscopic or radiological management, but in the event of failure of these techniques, and in selected patients, cholangiojejunostomy in segment 3 can be an effective option. We describe the case of a 48‐year‐old patient presenting with an unresectable perihilar cholangiocarcinoma, in whom palliative endoscopic management failed two times and an open segment 3 intrahepatic cholangiojejunostomy was performed. The patient was discharged on the 7th day, has a complete resolution of jaundice at 2 months and is alive at 6 months without any readmission.

The patients in the combined-therapy group were associated with older age (p ¼ 0.033) and larger tumor size (p¼0.042) compared with patients in the sorafenib-only group. After 1:2 PSM, 25 patients in the combined-therapy group were well matched with 50 patients in the sorafenib-only group. The combined-therapy group showed significantly improved overall response rate (ORR) and disease control rate (DCR) compared with the sorafenib-only group (24.0% vs 4.0%, p¼0.025; 48.0% vs 24.0%, p¼0.036). The median PFS was significantly longer in the combined-therapy group than the sorafenib-only group (7.1 months vs 6.0 months, p¼0.040), while the median OS was similar in the two groups (7.4 vs 7.0 months, p¼0.513). No difference was noted in the rate of adverse reactions between the combined-therapy group and the sorafenib-only group (32.0% vs 22.0%, p¼0.348), and the symptoms were relieved after treatment. Conclusion:The combination treatment of camrelizumab with sorafenib showed promising efficacy with acceptable safety for the management of advanced HCC, but further studies with larger sample sizes and longer follow-up duration or randomized trials are needed to identify the potential therapeutic value.Legal entity responsible for the study: The authors.