V07-12: Minimally Invasive Nephron-Sparing Surgical Management of Multifocal Renal Masses in the Hereditary Kidney Cancer Syndrome Patient
Patients with hereditary kidney cancer syndromes such as von Hippel-Lindau (VHL) syndrome are at risk for multifocal, recurrent, bilateral renal masses, often presenting at a young age. This presents a challenge for surgical management, with minimally-invasive nephron-sparing procedures of utmost importance. Additionally, the associated syndromic co-morbidities can present unique challenges with surgery in previously-operated fields.
A 29 year old male with VHL was referred for management of multiple bilateral renal masses. His history included a Whipple procedure performed for a benign pancreatic neuroendocrine tumor as well as several other stigmata of VHL elsewhere in the body. MRI showed 10 masses in the left kidney that ranged between 0.3 to 1.7 cm, and multiple masses in the right renal hilum/sinus measuring up to 3.5 cm. He was offered a radical right nephrectomy at an outside institution. Nuclear medicine kidney scan showed normal split function. Preoperative creatinine was 0.9 mg/dL. In November 2016 he underwent a right robot-assisted partial nephrectomy on four tumors. Three separate solid tumors in the renal sinus and hilum were removed on-clamp, with total clamp time of 25 minutes and 26 seconds. A fourth tumor was removed in the medial upper aspect of the kidney off clamp. Estimated blood loss was 1L. Total operative time was 3 hours, with 2.5 hours of console time, including 30 minutes of adhesiolysis.
The patient was discharged home on postoperative day 4 after an uncomplicated postoperative course. His hemoglobin nadir was 10.8 g/dL. Creatinine at discharge was 1.0 mg/dL and at two week follow-up was 0.9 mg/dL, unchanged from prior to surgery. Final pathology determined all four masses to be clear cell RCC, Furman grade 3, with negative margins.
Complex partial nephrectomies of centrally-located renal masses are technically feasible and safe and can provide excellent oncologic and nephron-sparing outcomes in patients with hereditary kidney cancer syndromes