Natural history and growth kinetics of clear cell renal cell carcinoma in sporadic and von Hippel-Lindau disease

Abstract

Background: To evaluate and compare the natural history and growth kinetics of sporadic clear cell renal cell carcinoma (ccRCC) with those of ccRCC in von Hippel-Lindau disease (VHL).Methods: Sixty patients in the sporadic group with 61 tumors and 15 patients in the VHL group with 30 tumors whom all underwent delayed surgery after at least 12 months of active surveillance (AS) were enrolled to conduct a retrospective cohort study. The growth rate was calculated, and the growth kinetics between the sporadic and VHL groups were compared. The patient and tumor characteristics were reviewed, and their correlation with growth rate was analyzed. Results:The mean growth rate of sporadic ccRCC was 0.91 cm/year (ranging from 0-4.74 cm/year) and that of VHL ccRCC was 0.47 cm/year (ranging from 0.04-1.89 cm/year). The growth rate of sporadic ccRCC showed a tendency of being faster than that of VHL ccRCC but did not reach statistical significance (P=0.07). The factors affecting the growth rate were different between the two groups. For VHL ccRCC, the only factor that correlated with growth rate was initial tumor diameter (P<0.001), but for sporadic ccRCC, the only factor was pathological nuclear grade (P<0.001). Conclusions:The growth rate of VHL-associated ccRCC might be slower than that of sporadic ccRCC. Furthermore, we identified a disparity in growth kinetics between sporadic and VHL-associated ccRCC.

The Clinical and Molecular Features in the VHL Renal Cancers; Close or Distant Relatives with Sporadic Clear Cell Renal Cell Carcinoma?

“…Poston et al also documented that ccRCC in VHL disease tends to have a lower grade, with low local invasiveness [ 84 ]. Other authors, on the other hand, have reported a similar growth rate between sporadic and VHL disease ccRCCs [ 82 , 89 , 90 ].…”

A 23-year bibliometric analysis of the development of global research on hereditary renal carcinoma

“…Bilateral and multifocal renal carcinomas occur in almost half of VHL patients. 25% to 60% of patients with VHL syndrome develop RCC, with an average age of onset between 40 and 45 years; this is approximately 20 years earlier than the average age of onset for sporadic renal cancer (8). With advancing age, the patient will experience a constant occurrence of new RCC in both kidneys, resulting in the presence of multiple tumors.…”