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ORLANDO, FL USA (UroToday.com) - Bilateral multifocal (BMF) papillary type I renal cell carcinoma (RCC) has traditionally been associated with hereditary papillary renal cell carcinoma (HPRC) with mutations of the met proto-oncogene. However, non-familial forms of this cancer also exist, yet are poorly characterized. Okoro et al. from the National Cancer Institute (NCI) describe their institutional experience with this rare clinical entity.

auaThe authors retrospectively evaluated 61 patients who had been treated at the NCI for BMF papillary type I RCC, which was negative for the met mutation, between 1996 and 2013. Over this time period, patients were managed with percutaneous tumor biopsy and partial nephrectomy when the tumor reached 3 cm in size. The NCI group found that patients with this type of RCC were disproportionately men (93.4%) and African American (34.4%). At presentation, this patient cohort presented with an average serum creatinine of 1.48 mg/dl and eGFR of 62.5 mL/min, findings of stage II CKD. At average follow-up of 32.9 months, no patients presented with metastatic disease. However, African American patients experienced an especially marked decrease in renal function.

The authors concluded that management with active surveillance and nephron sparing surgery when tumors are ≥ 3 cm is both reno-protective and oncologically sound in this patient population. Further research is necessary to determine the metabolic and genetic basis for this form of BMF papillary type I RCC, as well as to determine which specific populations are at the greatest risk.

Presented by Chionyerem Okoro, MD at the American Urological Association (AUA) Annual Meeting - May 16 - 21, 2014 - Orlando, Florida USA

National Cancer Institute (NCI), Bethesda, MD USA

Written by Dima Raskolnikov, National Cancer Institute (NCI)

 

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