Multipathogenetic Origin of a Pelvic Mass

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1 european urology 55 (2009) available at journal homepage: Case Study of the Month Multipathogenetic Origin of a Pelvic Mass Marco Oderda 1, Paolo Mondino 1, Andrea Zitella, Dario Vigna, Chiara Fiorito, Donatella Pacchioni, Alessandro Tizzani, Paolo Gontero * University of Turin, Molinette Hospital, Turin, Italy Article info Article history: Accepted December 22, 2008 Published online ahead of print on December 31, 2008 Keywords: Abdominal mass Bladder neoplasm Bladder outlet obstruction Bladder stone Urachal cyst Abstract A 69-yr-old woman presented with a bulky hypogastric mass and abdominal pain. Computed tomography scan showed a mass anterosuperior and contiguous to the bladder wall, with a hypodense content, a voluminous bladder stone, and bilateral hydroureteronephrosis. Intraoperatively, the supravesical mass had the appearance of an infected urachal cyst. An unsuspected high-grade noninvasive papillary transitional cell carcinoma (TCC) of the bladder thoroughly surrounding the bladder stone became evident during the cystolithotomy. Postoperative videourodynamic study showed a normal voiding pattern with bilateral grade 4 vesicoureteral reflux. Early cystectomy was performed for uncontrolled recurrent bladder cancer, and the final pathology indicated pt1g3n0 TCC. # 2009 European Association of Urology. Published by Elsevier B.V. All rights reserved. 1 Marco Oderda and Paolo Mondino equally contributed to this manuscript. * Corresponding author. University of Turin, Urologia 1 Molinette Hospital, C.so Dogliotti 14, Torino, Italy. Tel ; Fax: address: paolo.gontero@unito.it (P. Gontero). 1. Case report 1.1. Surgery 1 A 69-yr-old woman came to the emergency unit with severe lower abdominal pain and fever. She reported a long history of urinary incontinence, the feeling of a bulge inside the abdomen during the previous 3 wk, and severe constipation for the last few days. The patient had undergone a total hysterectomy several years earlier because of a large fibroma of the womb. Clinical examination revealed a tender bulky hypogastric mass extending above the navel and a hard anterior vaginal wall not consistent with malignant disease. On catheterisation, a small amount of concentrated malodorous urine was drained. Serum creatinine level was elevated at 2.2 mg/dl. An abdominal computed tomography (CT) scan revealed bilateral hydroureteronephrosis, a thick and irregular bladder wall surrounding a 7-cm bladder stone, and a 10-cm extravesical mass located contiguous to the anterosuperior bladder (Fig. 1). The extravesical mass had a thin wall surrounded by a hypointense centre. No communication with the bladder was evident. The patient s high temperature was treated effectively with antibiotics, and the creatinine level dropped to /$ see back matter # 2009 European Association of Urology. Published by Elsevier B.V. All rights reserved. doi: /j.eururo

2 european urology 55 (2009) Fig. 1 Abdominal computed tomography picture showing a large bladder stone and a supravesical cystic mass. Fig. 2 Intraoperative image showing the voluminous bladder cancer after removal of the stone. The supravesical mass has been removed. The thick bladder wall shows a large laceration that occurred during the surgical manoeuvres mg/dl. Excision of the supravesical mass and cystolytolapaxy were planned via an extraperitoneal approach. Unexpected tenacious adhesions of small bowel loops to the mass caused inadvertent bowel perforation, requiring a shift to a transperitoneal approach to perform a safe adhesion lysis that required the resection of 50 cm of the bowel. The supravesical mass was progressively excised and revealed a pattern consistent with an abscess of a urachal cyst. The thick bladder wall was inadvertently lacerated while attempting to free the anterior wall from tenacious adhesions to the retropubic bone. Intraperitoneal contamination of diffuse papillary lesions surrounding the stone and thoroughly occupying the bladder mucosa occurred (Fig. 2). Intraoperative pathology revealed grade 2 non muscle-invasive transitional cell carcinoma (TCC), which was macroscopically removed together with the bladder stone Pathology The surgical specimen identified as a supravesical mass measured cm, with an irregular large surface and a smooth opposite one. Microscopic examination showed a wall of muscular fibres admixed with fibrotic stroma and adipous tissue with steatonecrosis. A rich lymphocyte infiltrate was present, and one surface showed superficial ulceration with granulation tissue. No epithelial cells were present in either surface or in any of the specimens and sections (Fig. 3). The pattern was consistent with a urachal cyst. The fragments referred to as a bladder dome showed areas of papillary high-grade TCC, focally invading the subepithelial connective tissue (Fig. 4) Follow-up and surgery 2 A videourodynamic study 1 mo after surgery showed bilateral grade 4 vesicoureteral reflux, a stable bladder with a slightly reduced compliance, and type 3 urinary stress incontinence (Valsalva leak point pressure: 50 cm H 2 O) with no signs of anatomic outlet obstruction or vesical-sphincter dyssynergia in the voiding phase (Figs. 5 and 6). At postoperative month 3, the finding of multiple and large recurrent pt1g3 TCCs invading up to the urethra prompted a cystourethrectomy and ileal conduit followed by uneventful recovery. The patient is disease free 1 yr later. 2. Discussion This case represents a unique mixture of rare conditions with apparently unrelated pathogenesis, which is a diagnostic challenge. The CT appearance and location of the anterosuperior extravesical mass was consistent with a urachal cyst, although the possibility of an underlying malignant disease could not be excluded. Urachal cyst is one of the four variants of urachal anomalies; these include, in order of frequency, urachal sinus, urachal cyst, patent urachus, and urachal diverticulum [1,2]. Urachal remnants are rarely observed, but, clini-

3 1226 european urology 55 (2009) Fig. 3 Pathologic sample of the supravesical cystic mass: fibromuscular wall with sclerosis (deeper part in upper inset) and superficial ulceration (lower inset) at low power magnification. This pattern is consistent with a urachal cyst. cally, they often give rise to a number of problems such as infection and late malignant changes. Urachal cancers are uncommon malignancies with a location that often permits considerable local extension before they are discovered [1]. The possibility of a diverticulum, secondary to the bladder outlet obstruction that had favoured the large bladder stone, was considered unlikely due to the absence of a collar and the unusual location on the anterior bladder wall [3,4]. Fig. 5 Voiding phase of the videourodynamic investigation: bilateral grade 4 vesicoureteral reflux. The bladder neck is widely opened. Fig. 4 Pathologic sample of a large papillary lesion surrounding the bladder stone and occupying most of the bladder surface: high-grade transitional cell carcinoma. Bladder stone formation usually implies incomplete urine voiding favoured either by a detrusor hypocontractility or by a bladder outlet obstruction [5,6]. In females, a urethral stricture or a vesicalsphincter dyssynergia may account for the latter. Notably, the postoperative videourodynamic study failed to demonstrate any of these conditions. The hypertropic detrusor muscle may be a pattern secondary to a severe bladder outlet obstruction

4 european urology 55 (2009) Fig. 6 Videourodynamic results show no signs of detrusor overactivity, a slight reduction of the vesical compliance, and a normal voiding phase (maximal flow rate: 43 ml/s). The vesical catheter is ejected immediately after the onset of micturition. The morphology of the abdominal pressure curve shows a slight increase consistent with abdominal strain. During the voiding phase, the bladder neck relaxes normally without evidence of urethral stricture. Overall, urodynamic parameters rule out a bladder outlet obstruction. sustained by the stone. Bilateral hydroureteronephrosis due to grade 4 vesicoureteral reflux, as shown in the voiding phase of the videourodynamic study, would further suggest an underlying lower urinary tract obstruction. Alternatively, the aetiological mechanism of stone formation could have been recurrent urinary tract infections in the field of a primary vesicoureteral reflux. The correlation between the large bladder stone and the widespread transitional non muscle-invasive bladder cancer is even more intriguing. Inflammation of the bladder in the presence of stones may play some role in human bladder cancer development. In humans, the association between inflammation and tumours applies almost exclusively to squamous cell cancer, whereas in rodents, most tumours are TCCs [7,8]. In conclusion, some questions remain unanswered in the complex pathogenetic origin of this case. One possible explanation is that a number of unrelated diseases may have independently contributed to the development of this unique pelvic mass. Conflicts of interest: The authors have nothing to disclose. EU-ACME question Please visit to answer the below EU-ACME question on-line (the EU-ACME credits will be attributed automatically). Question: Which of the following are potential risk factors for bladder transitional cell carcinoma? A. Bladder stones B. Cystitis cystica C. Chronic bacterial and viral urinary tract infections D. None of the above References [1] Maletic V, Cerovic S, Lazic M, Stojanovic M, Stevanovic P. Synchronous and multiple transitional cell carcinoma of the bladder and urachal cyst. Int J Urol 2008;15:554 6.

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