Collecting Duct (Bellini Duct) Renal Cell Carcinoma: Oncologic Outcome and Therapeutic Management

摘要

Objectives: To evaluate treatment and prognosis of collecting duct renal cell carcinoma (CDRCC) in three institutions. Methods: The data of CDRCC patients were collected retrospectively from 3 participating institutions. Results: A total of 24 patients were identified in 3 institutions with an incidence of 0.5% - 0.6%. Among them, the median age was 63.0 years and male gender was predominant (66.7%). At least 45.7% (11/24) of the patients were symptomatic at presentation. Moreover, distant metastasis at initial diagnosis was present in 13 patients (54.2%) and 6 patients (25.0%) developed distant metastasis during the course of disease. Almost all these patients were at high stage (87.5%) and poorly differentiated (79.2%). Besides, nodal involvement and major vein extension were observed in 14 (58.3%) and 10 (41.7%) patients, respectively. All the patients in this cohort underwent surgery with a median cancer specific survival of 11.3 months. Of the 14 patients with chemotherapy, gemcitabine/cisplatin was dominantly given in 6 patients (42.9%). Conclusions: CDRCC is rarely seen. Most of CDRCC patients had advanced stage, high nuclear grade, regional nodal involvement, distant metastasis at presentation and consequent poor prognosis. To date, no standard protocol for the treatment of CDRCC exists. Current standard in systemic therapy of CDRCC is chemotherapy with gemcitabine and cisplatin.