Management and outcomes of patients with renal medullary carcinoma (RMC): A multicenter retrospective study of 39 patients.

Abstract

438 Background: RMC is a rare and aggressive neoplasm that afflicts primarily young black patients with sickle cell trait. Our primary objective is to report the outcomes of RMC patients through a collaborative initiative. Methods: After IRB approval, we retrospectively reviewed records of RMC patients treated from 2000-2013 at 7 academic institutions. Overall survival (OS) was calculated from RMC diagnosis to date of death. Descriptive statistics were used. Results: A total of 39 patients were included. Median age was 27.8 years and 29 (74%) were males. 38 (97%) had sickle cell trait. Median renal tumor size at presentation was 5.8 cm (range 3.4-11.4 cm). 3 patients presented with stage I disease, 13 with stage III disease, and 23 with stage IV disease. 22 patients had ECOG PS 0-1 and 17 had ECOG PS 2-3. The most common metastatic sites at diagnosis were lymph nodes in 27 and lung in 13. 26 patients (66.7%) had a nephrectomy. Pathologic stage was pT1-2 in 6 and pT3-4 in 20. 23 of these patients (88.5%) had positive lymph nodes at RPLND. 7 patients received preoperative systemic therapy. Median duration of pre-op therapy was 16 weeks (range 12-27 weeks). 1 patient had CR, 3 had PR and 3 had SD as best response to pre-op therapy. All patients received at least 1 line of systemic therapy. Median OS was 12.2 months. One patient had clinical CR with preoperative chemotherapy, near path CR on nephrectomy, and is currently NED at 27.4 mos. 5 patients are alive to date, though no patients have survived past 37 months. Conclusions: Patients withRMC have a grave prognosis. Nephrectomy (upfront or delayed) was associated with improved OS, even in patients with poor PS, though poor PS patients should be considered for upfront chemo. More effective therapies are desperately needed to improve patient outcomes. [Table: see text]