Young and Clement [3] reported the first case of mllerianosis in the urinary bladder in 1996 and defined this disease as a lesion seen at any site containing admixtures of endosalpingiosis, endometriosis, and endocervicosis. Twenty-one cases occurring in the urinary bladder have been documented in 19 articles (Table1) in the English literature, accounting for the majority of cases of mllerianosis [18]. Some sporadic cases have been reported in the ureter, spinal cord, inguinal lymph nodes and mesosalpinx [2]. To the best of our knowledge, this is the first report to describe mllerianosis of the umbilicus. Due to its rarity, the prevalence of mllerianosis is unknown.

Several hypotheses of the pathogenesis of mllerianosis of the urinary bladder have been proposed; however, the mechanism is not clearly understood. Implantation, one of the most discussed theories, suggests that mllerian tissue implants into the urinary bladder wall during pelvic surgery [3]. However, this cannot explain why mllerianosis is found in surgery-nave patients or in organs other than the urinary bladder. Ten of the 19 cases (52.6%) with detailed surgical records, the present case included, had not experienced any pelvic surgery such as hysterectomy or cesarean delivery. Another theory, metaplasia, advanced by Donne et al., [4] is based on the presence of two or more mllerian-derived tissues, which implies the potential of mllerian epithelium to differentiate rather than the implantation of a single type of the tissue. Moreover, of the 20 cases with marked tumor locations, a total of 16 tumors (80%) were situated on the posterior wall or the dome of the urinary bladder, a place adjacent to the peritoneum and more sensitive to female hormones. Koren et al. [9] in 2006 reported a case supporting the metaplasia theory with the metaplastic epithelium in continuity with the urothelium. The present case is also supportive of metaplasia because of concurrent occurrence of the bladder and the umbilical masses.

Mllerianosis of the urinary bladder mainly affects women of the reproductive age, with a mean age at diagnosis of 44.6years (range 27 to 70years). Of the total 22 patients with mllerianosis of the urinary bladder, five were postmenopausal females. The most common symptoms have been reported to be irritative voiding symptoms (54.5%) including frequency, urgency, urge incontinence and dysuria, abdominal/pelvic pain (45.5%) and gross hematuria (36.4%). These symptoms occurred cyclically in six cases (27.3%), mostly during the menstrual period. One patient with a tumor on the ureterovesical junction presented with renal colic [13]. Our case had none of the symptoms associated with bladder lesions, and only had umbilical bleeding.

Grossly, mllerianosis of the urinary bladder has been described as sessile polypoid, [17] submucosal, [6] or smooth with normal appearing overlying mucosa [14] on cystoscopic examinations. The gross appearance of the bladder tumor in our case revealed the similar findings as a mass with hemorrhage underneath the intact epithelium. Histologically, it revealed multiple foci of glands with variable size lined by tubal, endocervical, or endometrial epithelium situated in the lamina propria and muscularis propria [2, 15]. These glandular cells were immunohistochemically positive for ER and progesterone receptor (PR) [9]. In addition, the stroma surrounding the endometrial glands diffusely expressed CD10, and the epithelia showed positive staining for Ca-125 [15].

Both medical and surgical treatments can be used for mllerianosis of the urinary bladder. Regardless of the lack of consensus on the choice of therapy, most physicians and patients chose the surgical intervention in the reported cases, and most of the patients had a favorable prognosis. Sixteen patients underwent TURBT, only five of whom required subsequent medical treatment, [12, 13] or partial cystectomy [16, 18, 19] due to persistent symptoms or tumor recurrence. None of the four patients who underwent partial cystectomy had recurrence; by contrast, in two cases using gonadotropin-releasing hormone (GnRH) analogues to inhibit pituitary and gonadal function to reduce the tumor volume as medical treatment, cystoscopy showed smaller but persistent tumors at 3months and 2years of follow up. There was no documented distant metastasis or mortality in the cases with short-term follow-up (124months).

Mllerianosis of the urinary bladder mimics several bladder neoplasms such as cystitis glandularis and nephrogenic adenoma [9]. It is most important to differentiate between mllerianosis and invasive adenocarcinoma, both of which exist in the lamina propria and muscularis propria. A case of bladder endometrioid adenocarcinoma complicating mllerianosis has been reported [8].

In conclusion, mllerianosis is a rare neoplasm which mainly affects women of reproductive age. The common presentations of mllerianosis of the urinary bladder include irritative voiding symptoms, abdominal/pelvic pain and gross hematuria. In our case, multifocal mllerianosis were separately located on the urinary bladder and the umbilicus, which implies that the possibility of concurrent bladder mllerianosis should be considered when mllerianosis is found at other location. Despite a favorable prognosis in the majority of patients, in view of the importance of establishing the correct pathological diagnosis, we suggest treatment with a surgical intervention consisting of TURBT and partial nephrectomy if recurrence occurs with persistent symptoms.

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Concurrent mllerianosis of the urinary bladder and the umbilicus presenting with umbilical bleeding: a rare case report and review of the literature -...

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