Clinical, prognostic, and therapeutic aspects of urachal carcinoma—A comprehensive review with meta-analysis of 1,010 cases

• Published in: Urologic oncology Vol. 34; no. 9; pp. 388 - 398
• Main Authors: Szarvas, Tibor, Ph.D, Módos, Orsolya, M.D, Niedworok, Christian, M.D, Reis, Henning, M.D, Szendröi, Attila, M.D, Szász, Marcell A., M.D, Nyirády, Péter, M.D
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-09-2016
• Subjects: Chemotherapy; Humans; Prognosis; Prospective Studies; Therapy; Urachal cancer; Urachal carcinoma; Urachus; Urachus - pathology; Urinary Bladder Neoplasms - diagnosis; Urinary Bladder Neoplasms - therapy; Urology; Urachus; Urachal carcinoma; Therapy; Chemotherapy; Prognosis; Urachal cancer
• ISSN: 1078-1439; 1873-2496
• DOI: 10.1016/j.urolonc.2016.04.012

Abstract Background and objectives Urachal carcinoma (UrC) is a rare and poorly investigated disease. Our current knowledge is mainly based on single-institutional studies. Despite growing interest in UrC, the included case numbers in recently published studies are still low. Therefore, we aimed to provide a comprehensive meta-analysis on the clinical, prognostic, and therapeutic aspects of UrC. Methods A systematic Medline/PubMed search was performed on UrC using the terms “urachal carcinoma,” “urachal cancer,” and “urachus.” Original articles and reviews in English language with case numbers>10 were selected. Results The vast majority (91%, 489/532) of UrCs are diagnosed at later stages (Sheldon≥III) when the tumor invades the urinary bladder. About 21% (136/646) of UrC patients have distant metastasis at first presentation. Although for patients with non–metastatic UrC surgical treatment provides an acceptable disease control, the systemic treatment of patients with progressed/metastatic UrC—in lack of prospective clinical trials—are less well established. Comparing cisplatin-based and 5-FU-based therapies in 74 published UrC cases, we found the latter to be superior in terms of radiographic response rates (9% vs. 44%, P = 0.043), but the combination of these 2 therapies provided the lowest progression rate (14%) with a similarly high response rate (43%). Conclusions Owing to the lack of evidence-based guidelines, the therapy of UrC remains challenging. Given the infrequency of UrC, large prospective studies comparing different systemic therapies can hardly be conducted. Our metadata indicates that 5-FU-containing chemotherapy regimens are more effective than cisplatin-based treatment modalities, whereas their combination seems to provide the strongest antitumor effect. Nevertheless, in the lack of evidences from prospective clinical trials, therapeutic decision-making necessarily remains on an individual basis. In this situation, targeted therapies may provide a reasonable alternative. Therefore, better understanding of the molecular background of UrC is needed to rationalize treatment decisions in UrC.