Delaying surgery safe in von Hippel-Lindau patients

San Francisco-Even though patients with von Hippel-Lindau disease are at risk for renal tumors and eventual metastatic RCC, parenchymal-sparing surgery can be delayed until the largest tumor reaches 3 cm with minimal concern that the patient will develop metastatic renal cell carcinoma, according to updated data from an ongoing National Institutes of Health study.

None of the 108 patients with von Hippel-Lindau disease and solid renal tumors <3 cm in size on computed tomography followed for a median of 41 months developed metastatic disease, said Branden Duffey, DO, during a presentation at the AUA annual meeting. Renal tumors >3 cm were found in 73 other patients with von Hippel-Lindau disease, and 20 of these patients (27.4%) developed metastases at a median follow-up of 59 months (range, 0 to 321 months).

Mets increase with tumor size According to Dr. Duffey, the frequency of renal tumor metastases increased with increasing tumor size.

Members of the <3-cm group underwent nephron-sparing procedures more often than patients with tumors >3 cm. Coincidentally, both patient groups had a total of 124 surgeries among their patients. However, in the <3-cm group, 120 of 124 procedures (97%) were parenchymal-sparing surgeries, while only 85 of 124 (69%) were so among the >3-cm patients. This difference was statistically significant, said Dr. Duffey, now a urology resident at the University of Minnesota, Minneapolis.

"Patients [with tumors >3 cm] had nephrectomy more often than those [with tumors <3 cm] due to the severity of their disease," Dr. Duffey explained.

"Patients with von Hippel-Lindau disease are at risk for multiple, bilateral, recurrent renal tumors and metastases," he said, adding that, as a consequence, these patients are at risk for losing renal function, needing renal replacement therapy, and needing multiple surgeries.

In the study, the 181 patients with von Hippel-Lindau disease and renal cell carcinoma were identified by screening or retrospective review of medical records. Patients with tumors <3 cm were followed with serial imaging. Surgery was recommended when the largest tumor reached 3 cm.

Surgery recommended at 3 cm Surgical resection without delay was recommended to patients who initially presented with tumors >3 cm. Patients not undergoing screening often had large renal tumors at first diagnosis, Dr. Duffey said.

As von Hippel-Lindau disease frequently involves multiple organ systems, the following criteria can be used for diagnosis: more than one tumor in the brain or eye; a single tumor in the brain or eye plus one elsewhere in the body, such as in the pancreas, kidney, liver, or adrenal gland; or definite family history of von Hippel-Lindau disease plus any one of the above tumors.

Up to 45% of patients with von Hippel-Lindau disease will develop renal cancer, which is the leading cause of death in these patients.

"In von Hippel-Lindau patients, the 3-cm threshold for performing renal parenchymal-sparing surgery appears to be safe with regard to the development of metastatic renal cell carcinoma," Dr. Duffey said. "We advocate a 3-cm threshold for parenchymal-sparing surgery in patients with von Hippel-Lindau disease as a means to reduce the risk of metastatic disease while preserving renal function, avoiding or delaying the need for renal replacement therapy, and reducing the number of operations which a patient may undergo."