A Long Survival Woman with Primary Small-Cell Carcinoma of the Gallbladder: Role of Chemotherapy Maintenance

Gallbladder small-cell carcinoma (SCC) is an extremely rare cancer characterized by early metastases and associated with poor survival outcomes. The therapeutic options are limited in this indication and dedicated prospective trials are difficult to achieve. Maintenance chemotherapy is an evolving concept in medical oncology whose goal is to prolong chemotherapy-induced response. The role of maintenance therapy has been demonstrated especially in many cancers but the results remain controversial in small cell cancer. We report a case of a 49-year-old woman admitted in our institution with biliary colic, postprandial bilious vomiting, right hypochondrial mass, and deteriorated general condition. Abdominal computed tomography (CT) revealed a mass of the gallbladder with lymphadenopathy in the hepatic hilum and multiple liver metastases. CT-guided biopsy was performed that showed small, round cells with high nuclear-to-cytoplasmic ratio, and frequent atypical mitosis, which is consistent with high-grade small cell neuroendocrine carcinoma. Tumor cells were positive for chromogranin A, synaptophysin, and CD56. Ki-67 shows a high proliferation rate with 90% tumor cell staining and the diagnosis of gallbladder SCC was confirmed. The treatment used a combination of carboplatin and etoposide, interrupted by the generalized discomfort and shortness of breath during the second course of the etoposide. Repeated CT scan showed a partial radiological response in the order of 35% and carboplatin monotherapy was maintained with good tolerance and stability of the disease until the 11th cure. Thrombocytopenia at 70,000 per mm³ appeared, and its persistence forced the cessation of this treatment. Five months later, the disease progressed and second-line chemotherapy by irinotecan was given weekly. The death occurred 18 months after initiation of medical treatment due to hepatocellular insufficiency. Maintenance therapy in SCC should be considered as a promising therapeutic option when it is well tolerated.