The ‘Bladder Penis’: Longitudinal Polypoid Tumor of the Distal Ureter, a Rare Case of Urinary Tract Endometriosis

Ureteral endometriosis is difficult to diagnose and rarely presents with symptoms of acute pyelonephritis. Here we present this unique case of ureteral obstruction with consecutive pyelonephritis due to an intravesical long polypoid tumor, which originates from the right ureter. This entity of strong pleomorphism was managed by a combined approach of medical therapy and interventional procedures. Case Report A 50-year-old woman was admitted with complaints of acute colic-like flank pain and a fever of up to 39°C. No history of recurrent colic, hematuria, urinary tract infections or irregular dysmenorrhea for the last 3 months was reported. With physical examinations being unremarkable beside a left flank pain, laboratory results showed a leucocytosis (WBC 19,800/μl) with normal blood urea nitrogen and serum creatinine values. Urine microscopy and culture revealed Escherichia coli. On ultrasound a unilateral right-sided hydronephrosis was diagnosed. Consecutive intravenous urography verified the right-sided hydronephrosis with dilation of the right ureter down to the pelvic brim. The distal part of right ureter was not visible. Intravenous antibiotic therapy with ceftriaxon 2 g iv was started. The following cystoscopy demonstrated an intravesical longitudinal polypoid tumor with a penis-like outer shape with its origin from the right ureter (fig. 1). Retrograde ureteropyelography showed a suspect distal ureteral narrowing up to the linea terminalis of the pelvis. A ureteral stent was placed and intravenous antibiotic therapy continued. CT studies of the abdomen and pelvis demonstrated partial ureteral compression by surrounding tissue of unknown origin. Nuclear imaging showed an uncompromised excretory renal function. Ureteroscopic resection of the tumor was done after successful treatment of acute pyelonephritis. The histological analysis revealed endometriotic tissue. The patient was then started on gonadotropin-releasing hormone (GnRH)-analoga therapy consisting of goserelin for 6 months and the ureteral stent was left in place. However, this therapy regimen failed as demonstrated by the results of a retrograde pyelography and CT scans. Given these drawbacks, it was decided to proceed with an open surgical intervention. Through a right ilioinguinal mini-laparotomy intense ureterolysis, resection of affected ureteral segment, and ureterocystoneostomy with a psoas hitch were performed. The intra- and post-operative courses were uneventful with discharge of the patient from the hospital at postoperative day 10. After 2 weeks the ureteral stent was removed and an intravenous urography revealed a patent anastomosis as well as normal renal function. The patient reported complete relief of symptoms. The post-operative hormone therapy with GnRH-analoga regimen will be continued until menses cease. At follow-up 4 months after intervention no recurrence of urogenital infection or compromised renal function was observed.

Endometriosis, defined as the histological presence of ectopic endometrial glands outside the uterine cavity, is the second most common pathological condition of the female pelvis, affecting up to 20% of premenopausal women []. In contrast, endometriosis of the urinary tract is reported with an estimated prevalence of only 0.01 to 1%, affecting the ureter in about 15% of all those cases []. As observed in the presented case, the endometriotic lesions of the ureter are usually found below the pelvic brim, and more frequently on the left side []. Ureteral endometriosis is thought to originate from pre-existing foci of pelvic endometriosis and can involve the ureter extrinsically (80%) or intrinsically (20%) with ectopic tissue infiltrating the ureter from inside the lumen as observed in this patient []. Pleomorphism in the clinical presentation of ureteral endometriosis is strong, and it is assumed that due to a potential high percentage of undiagnosed cases as many as 25 to 50% of renal units are lost in ureteral endometriosis []. Signs and symptoms are mostly non-specific, presenting as gross or cyclical hematuria, flank or abdominal pain, menstrual dysfunction, pelvic mass, or nodularity of the uterosacral ligaments []. So far, there is no report of such a long and large polypoid tumor as described in this case. The finding of acute flank pain and fever was predominantly leading to immediate diagnostic work-up as described. Ureteroscopy with resection of the polypoid lesion with histological examination finally established the diagnosis and remains in cases of ureteral endometriosis the gold standard for a definitive diagnosis []. Management of ureteral endometriosis is controversial []. Treatment depends on location of ureteral involvement, degree of obstruction and kidney function, patient age, menopausal status, and fertility desires []. Regarding kidney function, nephrectomy is recommended if kidney scintigraphy reveals less than 15% of functional renal parenchyma []. For ureteral obstruction, resection of the affected segment is the mainstay of therapy. In the present case it was decided to perform endoluminal resection of the polypoid lesion with stenting of the ureter and adjuvant hormonal ablation. Likewise, a conservative approach for ureteral endometriosis has been recommended by several authors. Magrina et al. (1996) [] found ureterolysis to be as effective as segmental resection for ureteral obstruction. In contrast, the retrospective review by Nezhat et al. (1996) [] and Antonelli et al. (2006) [] reported the excision of the complete affected segment to present a superior approach based on the concept of necessary resection of the dense fibrosis, which almost invariably accompanies ureteral endometriosis. Until now, only a single report has been published on a successful medical management and the high risk of recurrent ureteral obstruction is well described []. In the present case the failure of a combined management with endoluminal resection of the lesion, hormone ablation, and ureteral stent placement was also observed, and following this, it was decided to proceed with surgical intervention. After excision of the narrowed prevesical ureteral segment ureterocystoneostomy with a psoas hitch was performed. The patient received an adjuvant hormone therapy as recommended for cases with moderate to extensive stages of endometriosis. Possible regimens include danazol, progestins, or GnRH agonists []. In conclusion, it appears that ureteral endometriosis is a highly pleomorphic disease capable of forming tumor like polypoid lesions. Surgical therapy in combination with hormone ablation remains the current therapy of choice for the treatment of ureteral endometriosis.

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