REVIEW   Free access

Minerva Urologica e Nefrologica 2019 April;71(2):121-6

DOI: 10.23736/S0393-2249.18.03192-2

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: English

Clinical and histopathological features of carcinosarcoma of the renal pelvis: a systematic review of a rare tumor

Guglielmo MANTICA ^{1, 2} ✉, Andrea BENELLI ³, Hilgard ACKERMANN ², Zoliswa NXUMALO ², Abdullwahed SOLAIMAN ², Federico DOTTA ¹, André van der MERWE ², Carlo TERRONE ¹

¹ Department of Urology, San Martino Polyclinic Hospital, University of Genoa, Genoa, Italy; ² Department of Urology, Tygerberg Hospital, Stellenbosch University, Cape Town, South Africa; ³ Department of Urology, ASST Rhodense, Garbagnate Milanese, Milan, Italy

INTRODUCTION: Carcinosarcoma of the kidney pelvis (CRP) is a rare but not exceptionally unique tumor in which in literature are reported almost only case studies and small series. We aim to provide a comprehensive overview of the disease, examining epidemiology, symptoms, pathological features, treatment and outcomes which are still missing, in order to offer a landmark paper to urologists which have to manage patients with this type of disease.
EVIDENCE ACQUISITION: A review of the current literature was conducted through the NCBI PubMed database in June 2017 following the updated PRISMA guidelines. The filters used for the research were: “carcinosarcoma renal pelvis,” “carcinosarcoma kidney pelvis,” and “urinary tract carcinosarcoma.” Only studies with confirmed histological diagnosis of CRP and a description of patient characteristics (demographics and/or pathological) were included in the review.
EVIDENCE SYNTHESIS: Only 15 studies fulfilled the inclusion criteria. The main symptom at presentation was macroscopic hematuria, followed by abdominal pain. The carcinomatous pattern made of transitional cell carcinoma in 73.3% of patients and squamous carcinoma in 33%, while the sarcomatoid was spindle cells in 40% of patients, chondrosarcoma and undifferentiated in 33.3%. Despite radical surgical treatment was performed in all patients, there are no articles reporting a survival rate of more than two years.
CONCLUSIONS: Carcinosarcoma of the kidney is a rare tumor of which little is known, especially about pathogenesis and oncological outcomes in the different therapeutic approaches described. Current literature is almost exclusively a case report that does not provide adequate information especially about survival and progression free survival. The creation of a shared database could in a matter of years lead us to increase the information about the oncological outcomes of the various possible treatments.

KEY WORDS: Carcinosarcoma; Kidney pelvis; Pathology; Systematic review