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Keywords:

  • bladder tumor;
  • partial cystectomy;
  • transitional cell carcinoma;
  • urachal cyst

Abstract

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. References

Abstract:  Incomplete involution of the allantoic duct can result in different pathological forms of urachus which can give rise to inflammation or late malignant changes. Among urachal tumors, adenocarcinoma is most frequent, although other histological types can also be found. The synchronous presentation of a urachal transitional cell tumor, along with recurrent superficial bladder tumors has not been reported previously. We are reporting a 49-year-old male patient in whom transitional cell carcinoma of a urachal cyst was found with recurrent, multiple bladder tumors. The diagnosis of urachal cyst tumor was established according to ultrasonography and computed tomography. Most of the bladder tumors were resected transurethrally while open surgical excision of the urachal cyst with en bloc resection of the bladder dome was performed. Recurrent bladder tumors were afterwards treated with Bacillus Calmette Guerin (BCG) instillations. A year after surgery the patient has no signs of local recurrence or distant metastases of transitional cell carcinoma.


Introduction

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. References

The urachus is an embryonic remnant resulting from involution of the allantoic duct and the ventral cloaca. It tethers the apex of the bladder to the umbilical ring. This vestigial structure, though constant in man, is formed by a whole series of embryological mechanisms which are still controversial.1 This duct becomes progressively obliterated during fetal life. It may subsequently persist as different variants after birth, some regarded as normal, such as the median umbilical ligament, others as pathological, due to incomplete closure. The four variants of urachal anomalies in order of frequency are urachal sinus, urachal cyst, patent urachus and urachal diverticulum.2 Although urachal remnants are rarely observed clinically, 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. Among them, transitional cell carcinoma is extremely rare. We are presenting a patient in whom transitional cell carcinoma in urachal cyst was synchronous with multiple recurrent transitional cell carcinoma of the bladder.

Case report

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. References

A 49-year-old male patient was admitted for recurrent transitional cell carcinoma (TCC) of the bladder with the perivesical mass suspected to be a diverticulum. A thorough diagnostic work up was performed. A palpable non-dolorous suprapubic mass was revealed on ultrasound (US) to be a hypoechogenic, limited, 7 cm large zone located antero-superiorly to the bladder dome. Intravenous pyelography (IVP) did not show any abnormality of the upper urinary tract. The wall of the vesical dome was irregular with the filling defect at the base due to the bladder tumor. Cystography failed to fill the suspected diverticulum. Computed tomography (CT) scan demonstrated multiple bladder tumors and an ovoid cystic mass located anterocranialy to the bladder. A cystic mass filled with dense liquid. At the junction with the bladder a polypoid proliferation, 3.7 × 2.6 cm in diameter, was seen (Fig. 1).

image

Figure 1. Computed tomography scan with tumor at the vesical dome and the urachal cyst.

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Cystoscopy revealed multiple papillary tumors on the right vesical wall, bladder neck and at the dome of the bladder which were resected, but no diverticula was found. On bimanual palpation a resistance, 7 cm in diameter was palpated anteriorly to the bladder. The palpable mass with possible tumor initiated an open surgical approach with diagnostic and therapeutic aim. The access was suprapubic. After the division of the rectus muscle a large midline supravesical cystic mass in continuity with the bladder dome was identified. The bladder was sagittally opened revealing a residual tumor at the dome which was adjacent to the cystic mass. The mass extended cranially toward the umbilicus. It was completely and easily mobilized and removed together with the bladder dome which was excised with a 1 cm wide safety margin around the tumor (Fig. 2).

image

Figure 2. Intraoperative aspect of urachal cyst in continuity with vesical dome.

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The enucleated cystic mass was opened and found to be filled with 200 mL of brownish liquid. A tumor, 2 cm in diameter was adjacent to the bladder tumor but without any communication with the excised bladder dome. The immediate postoperative course was uneventful (Fig. 3).

image

Figure 3. Opened bladder with the tumor at the dome.

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The histology analysis declared all the tumors to be TCC gr II (Fig. 4).

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Figure 4. Pathohistological aspect of the urachal tumor: transitional cell carcinoma grade II.

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Regular cystoscopies are performed every three months. Twice the patient had superficial Ta TCC, for which he was scheduled for BCG treatment. A control CT scan performed a year after surgery did not show a recurrence of the urachal tumor or any metastatic spread.

Discussion

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. References

Urachal cancers have variable clinical presentation, so uniform guidelines for evaluation and treatment are lacking. The most common histological type is adenocarcinoma, which may produce mucus that is a valuable aid in diagnosis.3 The incidence of the disease ranges from 0.55 to 1.2% of bladder tumors in Japan in contrast with 0.07–0.70% in Western countries. Males patients predominate in most series, the highest age incidence occurring between the fifth and sixth decades. The most common presenting symptom is hematuria (71%). Adenocarcinoma accounted for 88% of the tumors, most being mucous producing.4 The explanation for the predominance of adenocarcinoma, even though the urachus is normally comprised of transitional epithelium, is in the inherent potentiality of those epithelial cells to change into glandular epithelium of the mucinous variety, a recapitulation of the embryonic epithelium of the hindgut from which the urachus and urinary bladder are derived.5 Exceptionally, urachal carcinoma can be associated with transitional cell carcinoma of the urinary bladder6 or the ureter.7

Classical urological work up such as cystoscopy might not be conclusive. In our patient, cystoscopy did not reveal the diagnosis except the bimanual palpation which was performed after TUR of bladder tumors. US and CT examinations were crucial for the diagnosis.

The current performance of ultrasonography allows a very precise analysis of the antero-superior surface of the bladder, which may reveal the diagnosis, but CT scan or NMR are even more specific: a midline, calcified, supravesicular mass is highly suspicious, if not pathognomonic, for urachal carcinoma.8

The stages of the urachal tumors have recently been assigned according to the new Mayo 4-category staging system,9 which has proved to be less complicated than the previously used Sheldon system. The stages are I: tumors confined to the urachus and/or bladder; II: tumors extending beyond the urachus and/or bladder; III: tumors infiltrating the regional lymph nodes; IV: tumors infiltrating non-regional lymph nodes or other distant sites. According to this classification our patient had a Stage I, which explains the good result achieved by the treatment. Besides the stage, the most important predictors of prognosis are tumor grade and surgical margin status.

Surgical treatment is the treatment of choice. Although many authors advocated radical cystectomy it appears that long-term disease specific survival can be achieved with partial cystectomy with en bloc resection of the bladder dome; removal of urachal ligament and umbilicus10 is advisable.

CONCLUSION: An antero-superior extravesical mass should arouse suspicion of urachal pathology. US and CT scans are crucial for diagnosis. Long-term survival can be achieved with surgical treatment comprising en bloc resection of the urachus and the bladder dome.

References

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. References