Patients at risk with localized disease should be tested before surgery if they are suspected to harbor a hereditary syndrome. Testing should be done if it is safe to delay surgery in the meantime. However, if the patient has metastatic disease, with a high suspicion for a genetic syndrome, it is advisable to test him only if testing might change the proposed treatment.
Surgical intervention should be timed based on disease biology, with a balance needed between risk of dissemination and morbidity of frequent intervention. Hereditary leiomyomatosis and renal cell cancer (HLRCC) / Succinate dehydrogenase (SDH) deficient renal cell carcinoma (SDH-RCC) are very aggressive and need early intervention at any lesion size.
There is a “3” cm solid lesion rule for Birt-Hogg-Dubé Syndrome (BHD) , Von Hippel-Lindau (VHL) syndrome, Hereditary Papillary Renal Carcinoma (HPRC), and chromosome 3 translocation RCC. The solid component is the one that counts and represents the actual tumor, while the cystic component is not significant. It must be emphasized that in the same renal unit, all lesions should be removed.
The principles of surgery in hereditary renal surgery (whether open or robotic), is to prioritize partial nephrectomies as long as it is not compromising oncological control. All syndromes predispose patients to future tumors. The standard partial nephrectomy with a large negative margin is not feasible for most hereditary cancer patients. Therefore, enucleation of the tumors should be the standard of care. This technique enables removal of a large number of tumors and still enables the patient to retain good renal function. Additionally, important principles include drafting a pre-planned map with the goal to leave nothing big behind. Also, it is important to start with the removal of the easier tumors first, which are the ones with the lowest likelihood to require clamping and ischemia time. Later, the more difficult tumors should be removed, those with the highest chance of requiring clamping. This strategy minimizes ischemic time for the entire case. Additionally, bolsters should not be placed until the end of the procedure. Lastly, coordination with anesthesia is required, to allow them, to “catch up”.
It is important for the surgeons to understand what they are embarking on. Before performing the first surgery, it is important to make sure that the surgeon is willing to pursue surgery number 2,3, and so forth. Therefore, the “surgical footprint” should be minimized, as the surgeon will most probably need to be back in that place again in the future for a removal of yet another tumor. Minimizing the surgical footprint can be done by closing the peritoneum and placing a bowel pack in anatomic locations to minimize adhesions to the body wall. There is no need to dissect each hilar vessel out as it could increase the risk of vascular injury. Gerota’s fascia and the perinephric fat should be preserved, and it should be closed to prevent bowel adhesions. There are several products that are used to prevent adhesion formation. Metal clips should be avoided when possible as they could cause CT scatter and prevent the interpretation of future CTs. These complex cases can start robotically and can always be converted to open cases if required.
The “hostile abdomen” with a history of several surgeries can make the operation quite difficult. In these cases, it is important to not leave skin/muscle on its own (islands). Also, when redoing a flank incision, it is important to go through the same site but “cheat a little” to one direction in order to have at least some “virgin” territory. In any case, despite a higher rate of complications in these patients, the functional outcomes and percentage of complications in these patients are acceptable.
In summary, there are limited guidelines for these recurrent surgeries in these specific patients. If genetic testing could influence management, it should be performed, and surgery should be delayed. The “3” cm solid rule should be used, and tumors should be enucleated when possible. In the actual surgery, the easier tumors should be removed first, and the “surgical footprint” should be minimized.
Presented By: Brian M. Shuch, Yale School of Medicine
Written by: Hanan Goldberg, MD, Urologic Oncology Fellow, SUO, University of Toronto, Princess Margaret Cancer Centre, @GoldbergHanan, at the 19th Annual Meeting of the Society of Urologic Oncology (SUO), November 28-30, 2018 – Phoenix, Arizona