SIU 2017: UP17 - Carcinoma of the Collecting Ducts of Bellini - Clinicopathological Features

Lisbon, Portugal ( Carcinoma of the collecting ducts of Bellini (CDC) is a rare disease, thought to be derived from the cells of the collecting duct of Bellini and accounts for <1% of renal malignancies. The rarity of these tumours and heterogeneity of histopathologic features, which occasionally overlap with papillary renal cell carcinoma, and urothelial carcinoma of the kidney, limit our understanding of it. Optimal management of CDC is lacking. The authors therefore aimed to investigate the clinicopathologic features of CDC.

All cases of CDC were analysed from January 2006 to December 2016. All pertinent clinical information was recorded.

A total of 4 patients were found, three men and one woman, with a mean age of 72.3 (+14,8) years. One of them (25%) presented with disseminated disease upon diagnosis. All 4 patients were treated with radical nephrectomy. Two patients (50%) developed disseminated disease after surgery. The mean time from surgery to the development of metastases was 5.5 months. Two patients received systemic adjuvant treatment with Sunitinib, without obvious response. One of these patients also received axinitinib, after 3 months of Sunitinib. Two patients had pulmonary metastases. The other patient with disseminated disease developed osseous metastases in the humerus, ribs and femurs. The latter patient was submitted to palliative radiotherapy. All tumours had a high Fuhrman nuclear grade. Unfortunately, the mean survival was only 11.0 months (9.25-13.75).

The authors concluded that due to the fact that CDC has a poor prognosis, applying the proper treatment represents a future challenge and targeted therapies may play a role in selected cases.

Presented by: Antunes H.