Urachal adenocarcinoma in adults
Keywords:
adenocarcinoma, urachus, hematuria, umbilical ligament.Abstract
Introduction: Urachal adenocarcinoma is a rare, aggressive malignant tumor first described in 1893 by Hue and Jaequin. It mainly affects male patients in their fifth and sixth decades of life. It represents 0.7% of all bladder cancers and 35% of all bladder adenocarcinomas. It is more frequent in men than in women, with a ratio of 5:1.
Objective: Determine the diagnosis and updated management of urachal adenocarcinoma in adults.
Methods: Initial long-standing studies published in the period 2010-2020 were consulted in the databases Medline, PubMed and Google Scholar. The full-text versions were obtained via open access to PubMed, HINARI and other sources.
Discussion: Urachal carcinomas emerge from the medial umbilical ligament stemming from the allantois, which extends from the dome of the bladder to the umbilicus. Obliteration of this extension before birth and its persistence as a fibrous cord constitute the urachus. Urachal cysts are formed when the urachus obliterates at the vesical and umbilical ends until a permeable portion remains between these ends which accumulates secretions acquiring a cystic appearance.
Conclusions: Malignant transformation of urachal cysts is rare, and no prophylactic treatment is recommended. Urachal carcinomas emerge from embryonic remains. The histology of the carcinoma should be similar to that of the enteric type, and other primary types should also be excluded before diagnosing a primary urachal carcinoma.
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References
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33. Dong-Dong Y, Hui D, Zhi-Gang W, Yun-Bei X, Chao-Feng Z, Qin Q, et al. U-shaped relationship of age at diagnosis and cancer-specific mortality in primary urachal adenocarcinoma: a cohort study. Transl Androl Urol. 2020;9(3):1073-81. DOI: http://dx.doi.org/10.21037/tau-19-863
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35. Davaro F, Schaefer J, May A, Raza J, Siddiqui S, Hamilton Z. Invasive non-urachal adenocarcinoma of the bladder: analysis of the National Cancer Database. World J Urol. 2019;37(3):497-505. DOI: http://dx.doi.org/10.1007/s00345-018-2411-7
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7. Szarvas T, Modos O, Niedworok C, Reis H, Szendroi A, Szász MA, et al. Clinical, prognostic, and therapeutic aspects of urachal carcinoma-A comprehensive review with meta-analysis of 1,010 cases. Urol Oncol. 2016;34(9):388–98. DOI: http://dx.doi.org/10.1016/j.urolonc.2016.04.012
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9. Natale C, Leinwand GZ, Chiang J, Silbersteing JL, Krane LS. Reviewing the Demographic, Prognostic, and Treatment Factors of Primary Adenocarcinoma of the Bladder: A SEER Population-based Study. Clin Genitourin Cancer. 2019;17(5):380-8. DOI: http://dx.doi.org/10.1016/j.clgc.2019.06.010
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12. Alfred Witjes J, Lebret T, Compérat EM, Cowan NC, De Santis M, Bruins HM, et al. Updated 2016 EAU guidelines on muscle-invasive and metastatic bladder cancer. Eur Urol. 2017;71(3):462-75. DOI: http://dx.doi.org/10.1016/j.eururo.2016.06.020
13. Claps M, Stellato M, Zattarin E, Mennitto A, Sepe P, Guadalupi V, et al. Current Understanding of Urachal Adenocarcinoma and Management Strategy. Current Oncology Reports. 2020;22:9. DOI: http://dx.doi.org/10.1007/s11912-020-0878-z
14. Hamilou Z, North S, Canil C, Wood L, Hotte S, Sridhar SS, et al. Management of urachal cancer: A consensus statement by the Canadian Urological Association and Genitourinary Medical Oncologists of Canada. Can Urol Assoc J. 2020 [acceso 24/01/2021];14(3):E57-64. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7053367/
15. Chen X, Kang C, Zhang M. Imaging Features of Urachal Cancer: A Case Report Front Oncol. 2019 [acceso 24/01/2021];9:1274. Disponible en: https://www.frontiersin.org/articles/10.3389/fonc.2019.01274/full
16. Noel Palacio A, Pozuelo Segura O, Pozuelo Segura C, Roca Vanaclocha Y, Mones Jiménez L, Torramilans Lluis A. Patología de la pared abdominal y TC. Sociedad Española de Radiología Médica. 2008. Disponible en: http://seram2008.seram.es/modules.php?name=posters&file=viewpaper&idpaper=1044&idsection=3&in_window=&forpubli=
17. Parada Villavicencio C, Adam SZ, Nikolaidis P, Yaghmai V, Miller FH. Imaging of the urachus: Anomalies, complications, and mimics. Radiographics. 2016;36(7):2049-63. DOI: http://dx.doi.org/10.1148/rg.2016160062
18. Tubulin M, Borhani, Furlan A, Heller M. Diagnostic Imaging Genitourinary. 3rd ed. Elsevier; 2016. Capítulo 109, Urinary Bladder Carcinom [acceso 24/01/2021];418-9. Disponible en: https://www.rad.pitt.edu/node/143
19. Koster IM, Cleyndert P, Giard RWM. Carcinoma de uraco. Radiographics. 2009; 29:939-42. DOI: http://dx.doi.org/10.1148/rg.293085152
20. Royce TJ, Lin CC, Gray PJ, Shipley WU, Jemal A, Efstathiou JA. Clinical characteristics and outcomes of non urothelial cell carcinoma of the bladder: Results from the National Cancer Data Base. Urol Oncol. 2018;36(2):78.e1-78.e12. DOI: http://dx.doi.org/10.1016/j.urolonc.2017.10.013
21. Krasnow RE, Drumm M, Roberts HJ, Niemierko A, Wu CL, Wu S, et al. Clinical Outcomes of Patients with Histologic Variants of Urothelial Cancer Treated with Trimodality Bladder-sparing Therapy. Eur Urol. 2017;72(1):54-60. DOI: http://dx.doi.org/10.1016/j.eururo.2016.12.002
22. Hong SH, Kim JC, Hwang TK. Laparoscopic partial cystectomy with en bloc resection of the urachus for urachal adenocarcinoma. Int J Urol. 2007 [acceso 24/01/2021];14(10):963-5. DOI: http://dx.doi.org/10.1111/j.1442.2042.2007.01855.x
23. Bao B, Hatem M, Wong JK, Urachal adenocarcinoma: a rare case report. Radiol Case Rep. 2017 [acceso 24/01/2021];12(1):65-9. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5310256
24. Tsironis G, Bamias A. Treating bladder adenocarcinoma. Transl Androl Urol. 2018 [acceso 24/01/2021];7(Suppl6):S699-701. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323267
25. Vetterlein MW, Seisen T, Leow JJ, Preston MA, Sun M, Friedlander DF, et al. Effect of nonurothelial histologic variants on the outcomes of radical cystectomy for nonmetastatic muscle-invasive urinary bladder cancer. Clin Genitourin Cancer. 2017;S1558-7673(17)30248-3. DOI: http://dx.doi.org/10.1016/j.clgc.2017.08.007
26. Yaegashi H, Kadomoto S, Naito R, Makino T, Iwamoto H, Nohara T, et al. Metastatic urachal cancer treated effectively with gemcitabine/cisplatin combination chemotherapy and radiotherapy: A case report. Mol Clin Oncol. 2019 [acceso 24/01/2021];11(2):139-42. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6587011/
27. García Álvarez C, González Álvarez RJ, Plata Bello AC, Álvarez-Arguelles Cabrera H, Castro Díaz D, Concepción Masip T. Multimodal therapy in urachal carcinoma with oligometastatic bone disease. Urol Case Rep. 2019 [acceso 24/01/2021];26:100936. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6597527/
28. Paner G, López A, Sirohi D, Amin M. Updates in the pathologic diagnosis and classification of epithelial neoplasms of urachal origin, Advances in Anatomic Pathology.2016;23(2):71-83. DOI: http://dx.doi.org/10.1097/PAP.0000000000000110
29. Christopher R, Williams M, Keyur Ch. En Bloc Robot-assisted Laparoscopic Partial Cystectomy, Urachal Resection, and Pelvic Lymphadenectomy for Urachal Adenocarcinoma. Rev Urol. 2015 [acceso 24/01/2021];17(1):46-9. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4444777/
30. Voutsadakis IA. Successful treatment of locally advanced urachal adenocarcinoma with peri-operative gemcitabine–cisplatin combination therapy: a case report and perspective on targeted therapies. Cent European J Urol. 2020 [acceso 24/01/2021];73(4):476-81. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7848837/
31. National Cancer Database. NCDB Public Benchmark Reports. Cases Diagnosed 2009-2018 [acceso 24/01/2021]. Disponible en: https://www.facs.org/qualityprograms/cancer/ncdb
32. Mylonas KS, O’Malley P, Ziogas IA, El-Kabab L, Nasioudis D. Malignant urachal neoplasms: A population-based study and systematic review of literature. Urol Oncol 2017;35(1):33.e11-33.e19. DOI: http://dx.doi.org/10.1016/j.urolonc.2016.07.021
33. Dong-Dong Y, Hui D, Zhi-Gang W, Yun-Bei X, Chao-Feng Z, Qin Q, et al. U-shaped relationship of age at diagnosis and cancer-specific mortality in primary urachal adenocarcinoma: a cohort study. Transl Androl Urol. 2020;9(3):1073-81. DOI: http://dx.doi.org/10.21037/tau-19-863
34. Alanee S, Alvarado-Cabrero I, Murugan P, Kumar R, Nepple KG, Paner GP, et al. Update of the International Consultation on Urological Diseases on bladder cancer 2018: non-urothelial cancers of the urinary bladder. World J Urol. 2019;37(1):107-14. DOI: http://dx.doi.org/10.1007/s00345-018-2421-5
35. Davaro F, Schaefer J, May A, Raza J, Siddiqui S, Hamilton Z. Invasive non-urachal adenocarcinoma of the bladder: analysis of the National Cancer Database. World J Urol. 2019;37(3):497-505. DOI: http://dx.doi.org/10.1007/s00345-018-2411-7
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2021-09-06
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Suárez Salgado SM, Rosero Morillo MF, Álvarez Rivera CG. Urachal adenocarcinoma in adults. Rev. Cubana Urol. [Internet]. 2021 Sep. 6 [cited 2025 Jun. 4];10(3). Available from: https://revurologia.sld.cu/index.php/rcu/article/view/752
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