SIU 2017: Bellini Duct Renal Cell Carcinoma – an Aggressive Tumor that Requires Thorough Monitoring

Lisbon, Portugal ( Bellini duct carcinoma occurs in less than 1% of all malignant kidney tumors and is thought to be very aggressive. The aim of our study was to identify clinical features and treatment options in patients with this rare aggressive tumor.
Between 2008 - 2016 , a total of 1,648 surgical interventions due to kidney tumors were performed in a single institution. Histologically, carcinoma of the renal tubules (Belini duct) was diagnosed in 27 (1.6%) patients. The age of patients ranged from 28 to 69 years (mean 54.3 + 11.8). Men to women ratio was 16 (59.3%):11 (40.7%). Tumor size ranged from 24 to 140 mm (mean 59.87 + 25.2 mm). The mean R.E.N.A.L. scores was (9.1 + 2.1). Metastatic lymph nodes were found in 16 (59.3%) patients, lung metastasis in 6 (22.2%) patients, and one (4.8%) patient had a metastasis in his ipsilateral adrenal gland. In 13 (48.1%) patients partial nephrectomy was performed, in 14 (51.9%) patients - radical nephrectomy was performed, supplemented by regional lymph node dissection.

Patients had initially manifested with pain in 55.5% of cases, with a paraneoplastic syndrome in 48.1%, and with hematuria in 29.6%. Surgery type did not affect cancer specific survival (p= 0.3). Death associated with progression occurred in 3 patients with metastatic cancer and 3 patients without metastasis during the first year of follow up. Another 2 (9,5%) M0 patients died 18 and 21 months after surgery, respectively. In 14 (51.9%) patients disease progressed to visceral organs. In one (3.7%) patient 20 months after partial nephrectomy a local recurrence was diagnosed, which led to radical nephrectomy. Average progression free survival was 21.7 months. Median overall survival has not yet been reached yet.

Belini duct carcinoma of the kidney manifests in more than half of the cases with pain. In 48.1% - the manifestation was paraneoplastic syndrome, and in 29.6% - the presenting symptom was hematuria. In 77.7% of patients the tumor was centrally located. Lymph node involvement was present in almost 60% of patients. No difference in survival was noticed whether partial or radical nephrectomy was performed. Vigilant monitoring is required to diagnose early progression.

Presented by: Iurii Vitruk
Affiliation:  Department of Plastic and Reconstructive Onco-Urology, National Cancer Institute, Kyiv, Ukraine

Written by: Hanan Goldberg, MD, Urologic Oncology Fellow (SUO), University of Toronto, Princess Margaret Cancer Centre.Twitter: @GoldbergHanan at the 37th Congress of Société Internationale d’Urologie - October 19-22, 2017- Lisbon, Portugal