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Multicenter Study
. 2017 Dec;120(6):782-792.
doi: 10.1111/bju.13705. Epub 2016 Dec 9.

Management and outcomes of patients with renal medullary carcinoma: a multicentre collaborative study

Amishi Y Shah  1 , Jose A Karam  1 , Gabriel G Malouf  2 , Priya Rao  1 , Zita D Lim  1 , Eric Jonasch  1 , Lianchun Xiao  1 , Jianjun Gao  1 , Ulka N Vaishampayan  3 , Daniel Y Heng  4 , Elizabeth R Plimack  5 , Elizabeth A Guancial  6 , Chunkit Fung  6 , Stefanie R Lowas  7 , Pheroze Tamboli  1 , Kanishka Sircar  1 , Surena F Matin  1 , W Kimryn Rathmell  8 , Christopher G Wood  1 , Nizar M Tannir  1
Affiliations
Multicenter Study

Management and outcomes of patients with renal medullary carcinoma: a multicentre collaborative study

Amishi Y Shah et al. BJU Int. 2017 Dec.

Abstract

Objective: To describe the management strategies and outcomes of patients with renal medullary carcinoma (RMC) and characterise predictors of overall survival (OS).

Patients and methods: RMC is a rare and aggressive malignancy that afflicts young patients with sickle cell trait; there are limited data on management to date. This is a study of patients with RMC who were treated in 2000-2015 at eight academic institutions in North America and France. The Kaplan-Meier method was used to estimate OS, measured from initial RMC diagnosis to date of death. Cox regression analysis was used to determine predictors of OS.

Results: In all, 52 patients (37 males) were identified. The median (range) age at diagnosis was 28 (9-48) years and 49 patients (94%) had stage III/IV. The median OS for all patients was 13.0 months and 38 patients (75%) had nephrectomy. Patients who underwent nephrectomy had superior OS compared to patients who were treated with systemic therapy only (median OS 16.4 vs 7.0 months, P < 0.001). In all, 45 patients received chemotherapy and 13 (29%) had an objective response; 28 patients received targeted therapies, with 8-week median therapy duration and no objective responses. Only seven patients (13%) survived for >24 months.

Conclusions: RMC carries a poor prognosis. Chemotherapy provides palliation and remains the mainstay of therapy, but <20% of patients survive for >24 months, underscoring the need to develop more effective therapy for this rare tumour. In this study, nephrectomy was associated with improved OS.

Keywords: #KCSM; #KidneyCancer; kidney cancer; nephrectomy; renal cell carcinoma; renal medullary carcinoma (RMC).

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Figures

Fig. 1
Fig. 1
OS: comparison of nephrectomy plus systemic therapy vs systemic therapy only (N = 52 patients).
Fig. 2
Fig. 2
OS of nephrectomy patients: comparison of preoperative systemic therapy vs upfront surgery followed by systemic therapy (N = 38).
Fig. 3
Fig. 3
OS by nephrectomy and performance status. (A) ECOG PS 0–1; (B) ECOG PS 2–3.
Fig. 4
Fig. 4
OS by best response to first-line therapy (CR/PR/SD vs PD).
Fig. 5
Fig. 5
Suggested treatment algorithm. RPLND. retroperitoneal lymph node dissection.
Fig. 6
Fig. 6
Suggested diagnostic algorithm. IHC, immunohistochemistry.
See this image and copyright information in PMC

Comment in

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