Abstract
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.
Highlights
Renal cell carcinoma (RCC),which is the 7th most common cancer in men and the 9th most common in women, accounts for 2% - 3% of all malignant tumors in adults [1]
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
The aim of this study is to describe and evaluate the patient and tumor characteristics, treatment modalities and oncological outcome of collecting duct renal cell carcinoma (CDRCC) in three institutions
Summary
Renal cell carcinoma (RCC),which is the 7th most common cancer in men and the 9th most common in women, accounts for 2% - 3% of all malignant tumors in adults [1]. Among them, collecting duct (Bellini duct) renal cell carcinoma (CDRCC) is an extremely rare RCC variant with a very low frequency ranging from 0.4% to 1.8% of all RCCs [4]. Most of reported patients with CDRCC have under gonesurgery, radical nephrectomy alone seems not to favor the prognosis of the metastatic cases except for cytoreductive or palliative reasons. Efficacy of immune therapy has been reported in a few small series, it is considered to be not effective in treatment of metastatic CDRCC. No evidence except a few small series or case reports supports the efficacy of targeted therapy for CDRCC so far. Several trials are under way to evaluate the role of targeted therapies in non-clear cell RCC [6] [12] [13] [14]. The aim of this study is to describe and evaluate the patient and tumor characteristics, treatment modalities and oncological outcome of CDRCC in three institutions
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