Kidney cancer. Principles and practice. Second edition. Primo N. Lara, Jr. Eric Jonasch (Editors). Springer International Publishing (2015)

General considerations

The management of hereditary renal carcinomas, though far from perfect, is not a terribly complex algorithm. Traditionally, bilateral nephrectomy was considered for patients with hereditary RCC. Such an approach predestined all of these patients to a lifelong dependence on dialysis. Some of the more fortunate individuals, who are free of recurrence, became candidates for renal transplantation [74, 75]. Current practice has departed from the paradigm of removing all renal tissue; instead, preservation of nephrons has become a principal objective. For VHL, BHD, HPRC, and familial renal cancer of unknown cancer (FRC), the 3 cm threshold for surgical intervention has been implemented with success. Utilizing systematic resection of tumors in a stepwise fashion, from most accessible to the most challenging, the surgeon and anesthesiologist maximize hemodynamic stability for the longest duration possible. Enucleation, taking no additional margin of benign tissue, has also become the preferred technique in several forms of hereditary RCC. It has been utilized successfully with good outcome in VHL, BHD, and even HPRC [76, 77]. On the other hand, patients with the more aggressive subtypes of hereditary RCC (HLRCC and SDH) demonstrate greater propensity locoregional dissemination and thereby would benefit from a more aggressive surgical approach.

Complementary to surgical intervention, there are systemic therapies currently undergoing clinical testing. These drugs are derived from the signaling pathways unique to the hereditary RCCs, but their application may someday encompass the sporadic forms of kidney cancer.


Classically, a significant majority of patients with VHL would expire from metastatic kidney cancer. Traditional management to avoid dissemination of RCC was bilateral radical nephrectomies followed by renal replacement therapy [78]. Current opinion has shifted away from such a practice. Management of these complex patients now consists of early detection, meticulous surveillance (beginning in childhood), and a balanced consideration of surgical intervention and preservation of renal function. Nephron-sparing interventions such as partial nephrectomies are now first line, with the goal of limiting procedures, maximizing kidney function, and preventing risk of metastasis. Ablative techniques have also been utilized for patients who may be too ill for open or laparoscopic surgeries. BHD and HPRC can be managed in a similar fashion, as shown by a 10-year operative experience on hereditary kidney cancer published by Herring et al. [79].


The management of HLRCC differs from the other forms of inherited RCC. Unlike VHL or BHD, the 3 cm criterion for invoking surgical intervention does not apply—early resection with a wide margin is vital due to the infiltrative nature of these tumors (Fig. 8.2). HLRCC exhibits a very aggressive course, with early series reporting over one half of the patient population demonstrating regional or metastatic disease despite small primary tumors [34]. Although there is no consensus on a surveillance protocol, experts have proposed early genetic testing, screening, and abdominal imaging, followed by annual MRI due to the high lethality of the kidney cancer [35]. Akin to HLRCC, management of kidney tumors in SDH individuals involves early detection and wide surgical excision [80]. There has also been a recent report of VEGF-targeted therapy for a female patient with metastatic disease, where treatment with sunitinib was met with a near complete response [81].

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