The Surgical Approach to Multifocal Renal Cancers: Hereditary Syndromes, Ipsilateral Multifocality, and Bilateral Tumors

doi:10.1016/j.ucl.2012.01.006

Urologic Clinics of North America

Volume 39, Issue 2, May 2012, Pages 133-148

https://doi.org/10.1016/j.ucl.2012.01.006 Get rights and content

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Definitions of multifocality and bilaterality

Sporadic, unilateral renal tumors account for most patients with kidney cancer; however, patients with multiple renal masses are not uncommon, and clinicians who specialize in RCC will eventually be faced with the management of these patients.³ Multifocality in kidney cancer refers to having more than one renal tumor, which can further be divided into whether the additional tumors are ipsilateral or bilateral. Bilateral tumors refer to lesions on both kidneys and they can present in a

Description of hereditary renal syndrome phenotypes

Hereditary kidney cancer is believed to represent 1% to 4% of RCC cases. Of these patients, some may fit into well-characterized hereditary kidney cancer syndromes, whereas many more patients with RCC may have a genetic component that is not fully recognized or understood. Because of our current poor understanding of genetics and cancer susceptibility genes, the 1% to 4% estimate may be a gross underestimation. A generational study from Iceland (dating back 11 centuries) has aggregated nearly

Principles and goals of surgical management

For patients with sporadic, solitary renal tumors, the goal of therapy is cancer cure while maximizing renal function. With sporadic patients, a single surgery is likely to be sufficient, with surveillance most commonly aimed at identification of cancer dissemination. In the sporadic population, RCC usually presents in patients in their 60s, and although not ideal, a radiographically normal-appearing contralateral kidney gives the clinician the option of sacrificing an affected kidney in the

Workup and management

Patients presenting with bilateral, multifocal, and possibly hereditary renal tumors present management dilemmas. Decisions on timing of genetic testing, role of biopsy, likelihood of pathologic concordance, order of surgery, sequence of tumor removal, optimal methods of nephron preservation, and surveillance for de novo lesions are unique to this patient population. Management of these patients is largely based on more than 20 years of NCI experience with excellent oncologic outcomes. We

Technical considerations

An operation on patients with bilateral, multifocal, and hereditary kidney cancer has unique technical aspects that differ from that of sporadic renal masses. The likelihood of requiring repeat renal surgeries is high and therefore the surgeon must be cognizant of maximizing renal function while minimizing unnecessary steps that could hinder future surgical approaches. Such steps may limit unnecessary morbidity of future operations that are often fraught with complications. Although none of

Special follow-up concerns: recurrence versus de novo tumor development

In patients with sporadic renal tumors, a major goal of follow-up is aimed at early identification of distant disease. Although this is also true with those with bilateral, multifocal, and hereditary syndromes, the likelihood of new tumor development is much higher. Understanding the definition of recurrence and how to manage tumors when they occur is critical for any clinician taking care of this patient population.

Summary

Although the management of sporadic renal tumors is challenging enough, dealing with those with bilateral, multifocal, and hereditary kidney cancer adds an additional level of complexity. A clinician managing this patient population must understand the hereditary syndromes and the genetic testing available. If affected with a germline mutation, other family members should be offered screening and surveillance if they are found to be carriers of a germline mutation. In patients without an