Surgical treatment of locally advanced urachal cancer: A single case with a long-term follow up and literature review

CASE REPORT

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.

SUMMARY 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.

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 dis-

Table 1. Sheldon and Mayo staging system for urachal cancer.

Sheldon staging

Stage I Urachal cancer confined to urachal mucosa (no invasion beyond urachal mucosa) Stage II Urachal cancer with invasion confined to urachus itself Stage IIIA Local urachal cancer extension to bladder Stage IIIB Local urachal cancer extension to abdominal wall Stage IIIC Local urachal cancer extension to peritoneum Stage IIID Local urachal cancer extension to viscera other than bladder Stage IVA Metastatic to regional lumph node Stage IVB Metastatic urachal cancer to distant sites

Mayo staging

Stage I Tumor confined to urachus and/or bladder Stage II Tumor extending beyond the muscular layer of urachus and/or the bladder Stage III Tumor infiltrating the regional lymph node Stage IV Tumor infiltrating non-regional lymph nodes or other distant sites eases to reduce the adverse features of a surgical treatment, but its adoption in clinical practice is limited by the high risk of seeding and of a non-complete tumor resection (2). In this study we report the management, surgical treatment and subsequent longterm follow up of a patient affected by advanced stage UrC.

CASE REPORT

In February 2010, a 57-year-old patient presenting with hematuria was submitted to abdominal ultrasonography (US), which revealed the presence of a 5 cm expansive esophytic neoformation of the bladder, in contact with direct contiguity to the overlying ileal loop. A computerized tomography with urographic phase (Uro-CT) was then performed and it reported the presence of a voluminous expansive process >8cm, extensively infiltrating the posterior bladder wall and expanding cranially, with the presence of a gaseous component, suggesting

Figure 1. Pre-operative CT scan showing a voluminous solid tumor infiltrating the bladder bottom, and expanding upwards into the abdominal cavity, with gaseous component in the cranial portion.

ileal deep involvement (Figure 1). The patient was then submitted to cystoscopy and resection of the tumor (TURB-T). Pathological examination described an adenocarcinoma with intestinal differentiation, morphologically consistent with UrC. In order to complete the clinical staging, a bone scan was performed and resulted negative for bone metastasis. In March 2010, a radical cystectomy with nerve-sparing technique, resection of the involved ileo segment and bilateral ureterocutaneostomy with single outlet was performed. The hospital stay was 9 days. At discharge, the patient was in good clinical status, Hb was 9.5 mg/dl, and serum creatinine 1.25 mg/dL. Pathological examination revealed moderately differentiated adenocarcinoma, G2, intestinal type, compatible with UrC, infiltrating the bladder wall, the fat and the ileal loop. 6 months after surgery the patient was submitted to a chestabdomen CT scan, with no signs of recurrence. From 2011 to 2015 US and Uro-CT were alternatively performed every 6 months and resulted negative for relapse or metastasis. From 2016 to 2019 Uro-CT was annually performed and reported negative for recurrences and metastasis. In 2017, 7 years after the UrC removal, the patient underwent surgery to convert the ureterocutaneostomy into an ileal conduit (uretero-ileo-cutaneostomy), which led to an improvement in quality of life and reduced infection risks related to ureteral stenting. Nowadays the patient is disease-free, and the ileal conduit allows him to lead an active and sporting life.

CONCLUSIONS

UrC is an aggressive neoplasm with surgery still representing the mainstay of therapy. Radical cystectomy proved to be a safe alternative to partial cystectomy in advancedstage UrC, reducing recurrence risk factors and providing an acceptable long-term quality of life.

REFERENCES

1. Szarvas T, Modos O, Niedworok C, 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):388-398. 2. Tomita K, Tobisu KI, Kume H, et al. Long survival with extended surgery for urachal carcinoma involving adjacent organs. J Urol. 1998; 159(4):1298. 3. Herr HW, Bochner BH, Sharp D, et al. Urachal carcinoma: contemporary surgical outcomes. J Urol. 2007; 178(1):74-78; discussion 78.

CORRESPONDENCE

Umberto Barbaresi, MD Department of Urology, S.Orsola Malpighi University Hospital Via Palagi, 9 – 40138 Bologna Phone: 0512142942 e-mail: ubarbaresi@libero.it

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