Background: Bellini carcinomas, rare tumors of kidney, are aggressive and have a poor prognosis. For these cancers, there is no standard treatment regimen and chemotherapy for urothelial cancer is usually used.
Case Report: In a 44-year-old man with hematuria, a tumor was diagnosed in the right kidney. After radical nephrectomy, pathologic analysis revealed Bellini carcinoma, staged pT3apN0, Fuhrman grade 3. Secondary pulmonary lesions occur one year later. Chemotherapy (gemcitabine, cisplatin and bevacizumab) was started and after 2 cycles of chemotherapy, Thoracic CT scans showed good response to treatment, with almost complete regression of the pulmonary lesions. After the third cycle of chemotherapy, maintenance treatment with bevacizumab continued. Fifteen months after diagnosing pulmonary metastases, hilar adenopathies progressed slightly and cisplatin-gemcitabine was started again leading to a partial response after five courses. Approximately 2 years after the diagnosis of lung metastases, the patient presented a second relapse, so carboplatin-gemcitabine was started, while bevacizumab was continued. 24 months after the diagnosis of lung metastases, the patient was still alive with controlled disease.
Conclusions: In view of our findings, a prospective multicenter trial with cisplatin, gemcitabine and bevacizumab in patients with metastatic collecting duct carcinoma is planned.

Keywords: Bellini carcinoma, complete response, bevacizumab