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REPORT
Surgical treatment of locally advanced urachal cancer: A single case with a long-term follow up and literature review Alessandro Bertaccini 1, Umberto Barbaresi 2, Alessandro Colella 2, Fabio Manferrari 1, Riccardo Schiavina 1. 1
Department of Experimental, Diagnostic and Specialty Medicine (DIMES), Cardio-Nephro-Thoracic Sciences Doctorate, University of Bologna, Bologna, Italy; 2 Department of Urology, University of Bologna, Bologna, Italy.
Urachal carcinoma (UrC) is rare and it is related to a poor prognosis. We report the surgical treatment and the subsequent long-term follow up of a patient affected by advanced stage UrC. A 57-year-old patient presenting a voluminous expansive process >8cm, extensively infiltrating the posterior bladder wall was submitted to endoscopic resection with a diagnosis of UrC. Radical cystectomy with resection of the involved ileo segment and bilateral ureterocutaneostomy was then performed. 7 years after, ureterocutaneostomy was converted into an ileal conduit, with subsequent improvement in quality of life. Nowadays the patient is living and disease-free. Surgery still represents the mainstay of therapy for UrC. Radical cystectomy proved to be a safe and effective alternative to partial cystectomy in advanced-stage UrC.
SUMMARY
KEY WORDS: Urachus, urachal carcinoma, radical cystectomy, partial cystectomy, cancer diagnosis, cancer treatment.
INTRODUCTION The urachus is a tubular structure that connects the fetal bladder to the allantois. During the fourth and fifth month in embryonic life, the urachus gradually degenerates into a rudimentary fibromuscular closed canal, which is known as the median umbilical ligament (1). The urachus comprises 3 distinct tissue layers: an epithelial canal lined by urothelium, submucosal connective tissues, and an outer layer of smooth muscle. Urachal neoplasms can arise in any of these layers and can be epithelial or mesenchymal. Like urothelium at other sites, the epithelium often demonstrates focal glandular metaplasia, and this provides a morphologic basis for the development of intestinal-type tumors. Urachal carcinoma (UrC) is rare and comprises 0.35% to 0.7% of all bladder cancers and 22% to 35% of vesical adenocarcinomas (1). The median age at diagnosis of UrC is 51 years, and the prognosis is relatively poor with a 5-year survival of 37 % and a 10-year survival of 17 %. Although hematuria is the most common symptom, the disease is usually advanced when this symptom appears. The common metastatic sites include lymph nodes, peritoneum and lung (1). Diagnosis is based on CT imaging with evidence of a cystic or solid structure in the bladder dome or in the bladder midline. Several criteria have been proposed to help in UrC diagnosis: location in the dome of the bladder, absence of cystitis cystica or cystitis glandularis, predominant involvement of the muscularis rather
than the submucosa, demonstration of an urachal remnant connected to the neoplasm and the presence of a suprapubic mass (2). Several staging classifications have been developed through years, but the most commonly adopted is the Sheldon-Mayo staging system (Supplementary Table 1) (1). Its nature of a well-differentiated tumour with a theorical good prognosis is contrasted by the rarity of symptoms in the earlier stages. This often led to a late presentation of the UrC at advanced stage and frequently because of adjacent organs invasion and/or distant metastasis. The mainstay of treatment for these tumors is partial cystectomy with en bloc resection of the median umbilical ligament up to the umbilicus (3). Leaving the umbilicus in place provides inadequate control and has been associated with a higher risk of relapse. In our case the disease involved an ileal tract and the bladder wall at the time of diagnosis, so we decided to perform radical cystectomy with ileal segment resection. Despite the high recurrence rate of UrC, the radical treatment we adopted in this context led to a long-term disease-free survival. According to literature, tumor stage and surgical margin status are the strongest predictors of survival (3). Radical treatment reduces the positive surgical margins rate, and help avoiding recurrences, associated to a poor prognosis, as adjuvant or salvage chemotherapies benefits are still a matter of debate. This is probably related to the very advanced stage at presentation of the disease (3). Partial cystectomy has been proposed in a limited amount of low stage disAdvances in Urological Diagnosis and Imaging - 2021; 4,1
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