Mullerianosis: A Rare Tumor Like Lesion of the Urinary Bladder

Mullerianosis; bladder; surgical treatment DOI: 10.5336/caserep.2022-90551 CASE REPORT Correspondence: Fatih AKDEMİR Clinic of Urology, Terme State Hospital, Samsun, Türkiye E-mail: [email protected] Peer review under responsibility of Turkiye Klinikleri Journal of Case Reports. Re ce i ved: 16 Apr 2022 Ac cep ted: 02 Sep 2022 Available online: 08 Sep 2022 2147-9291 / Copyright © 2022 by Türkiye Klinikleri. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Turkiye Klinikleri Journal of Internal Medicine Türkiye Klinikleri Journal of Case Reports der and a cystic mass with a diameter of 3 cm on the anterolateral of the uterine corpus were observed ( Figure 1). In the cystoscopy, a cystic, polypoid mass of approximately 1.5 cm in size was observed on the posterior wall of the bladder. Therefore, transurethral resection was performed for the pathological diagno- sis. Resection was performed on the lesion in the en- dometrium by hysteroscopy, and endometrial curettage was performed. Pathological diagnosis was reported as mullerianosis and endometrial polyp for bladder and uterus, respectively. After excluding the possibility of malignancy, the patient underwent total hysterectomy and partial cystectomy. The patient whose pathological diagnosis was reported as en- dometrial/endocervical polyp and bladder mulleri- anosis was followed up ( Figure 2 , Figure 3 ). The patient allowed the use of photographs and all medi- cal documents related to her illness and signed an in- formed-consent agreement.

Bladder mullerianosis is defined as the involvement of at least 2 of the mullerian origin tissues such as en- dosalpinx, endometrium and endocervix in the lamina propria and muscularis propria of the bladder. 3 The most common of mullerian-derived tissues is en- dometriosis, while the rarest one alone is endocervi- cosis.5 It has been reported that it can also be seen in other areas such as the spinal cord, inguinal lymph nodes, ureter and mesosalpinx apart from the urinary system. 6 The bladder is more affected in the urinary system, and half of the cases have undergone ce- sarean section or pelvic surgery. Often seen in women in the reproductive period. 7 No cases of mul- lerianosis have been reported in men. However, it has been reported that endometriosis can be seen in men receiving estrogen therapy for prostate cancer and postmenopausal women receiving exogenous estro- gen therapy. 4 Clinical symptoms such as hematuria, pelvic pain, dysuria and renal colic may be seen in bladder mullerianosis. These symptoms are often concurrent with the menstrual cycle. On cystoscopy, it can be Fatih AKDEMİR et al. Turkiye Klinikleri J Case Rep. 2022;30(4):230-3 231 FIGURE 1: Magnetic resonance imaging. The lesion in the bladder (white arrow) and the lesion originating from the endocervical canal and extending towards the anterior wall of the uterus (yellow arrow) is observed. It is ob- served that the integrity of the bladder wall is impaired. FIGURE 2: In the bladder transurethral resection material, polypoid tissue lined with urothelial epithelium (black arrow), single-layered and pseudostra- tified epithelium in the mucosa, locally cystic enlarged endometrial (blue arrow), endocervical gland (red arrow) structures, and thick-walled vascular structures with stroma were observed (H&E, 4x10). FIGURE 3: In immunohistochemical studies; positive (+) staining was ob- served in the endometrial stroma (blue arrow) with CD10 (4x10). seen as a 1-4.5 cm polypoid mass or a cystic lesion, usually covered with hyperemic mucosa located on the dome or posterior wall of the bladder. 6,7 Benign and malignant conditions such as cystitis cystica and glandularis, urachal remnants, nephrogenic adenoma, bladder adenocarcinoma and cervical adenocarci- noma metastasis should be kept in mind in the dif- ferential diagnosis. 6,8 Cystitis cystica, cystitis glandularis, and nephrogenic adenoma are superficial and do not involve the muscularis propria. Also, es- trogen and progesterone receptors are not painted. Urachal remnants are usually observed in the bladder dome and appear as tubular structures covered with mucinous epithelium, surrounded by loose peritubu- lar fibromuscular tissue. It can be differentiated his- tologically by the presence of endometrial stroma through CD10 immunostaining. However, unlike mullerianosis, adenocarcinoma affects older individ- uals, and malignant transformation findings such as significant atypia, increased mitotic activity, and stro- mal reaction are observed in the glands in the urothe- lial epithelium. 6,7,9 Malignant transformation of mullerianosis is a very rare condition, and Garavan F et al. reported such a case. 10 Although the pathogenesis of bladder mulleri- anosis has not been clearly explained, two main the- ories have been proposed. According to the implantation theory proposed by Young and Clement, mullerian tissues are implanted in the blad- der during pelvic surgery or cesarean section. 3 The fact that most of the cases with mullerianosis have a history of cesarean section or pelvic surgery supports this theory. However, this theory is insufficient to ex- plain its presence in people who have not undergone pelvic surgery or in other parts of the body away from the uterus. 11,12 Donne et al. proposed the metaplastic theory.13 Accordingly, endometrial, endocervical and tubal components of mullerian origin may prolifer- ate in the bladder. The fact that mullerianosis occurs in the posterior wall, which is an area covered by the peritoneum and sensitive to female hormones, sup- ports this theor. 13 Branca and Barresi suggested that the secondary mullerian system forming the peri- toneal mesothelium may preserve its ability to dif- ferentiate into endometrial, endocervical, and tubal tissues. 6 On the other hand, Koren et al. described a small tubal-type metaplastic ciliary epithelial focus in cystitis glandularis that is continuous with the urothelium and stained immunohistochemically pos- itive for estrogen and progesterone receptors. There- fore, they stated that mullerianosis may occcur through urothelial metaplasia in the setting of chronic inflammation. 14 In this case, there was a history of 3 cesarean section operations, supporting the implan- tation theory. Initial treatment for mullerianosis is transurethral resection. After the histopathological diagnosis is made, medical and surgical treatments can be applied according to the age of the patient, size, number, location and depth of infiltration in the bladder. Medical treatment options such as combined estrogen-progesterone contraceptives, progestins, and gonadotrop releasing hormone agonists may provide regression of symptoms. Repeated transurethral re- sections can be performed in cases that do not re- spond to medical treatment or have recurrences. Lesions on the serosal surface of the bladder can be removed laparoscopically. If the lesion has involved all layers of the bladder with the uterus, partial cys- tectomy and hysterectomy should be performed to prevent recurrence. 6,7,15 In conclusion, bladder mullerianosis is a very rare benign lesion. However, it is important to define mullerianosis, detailed histopathological analysis, ap- propriate treatment, and careful differential diagno- sis with malignant conditions. Source of Finance During this study, no financial or spiritual support was received neither from any pharmaceutical company that has a direct con- nection with the research subject, nor from a company that pro- vides or produces medical instruments and materials which may negatively affect the evaluation process of this study. Conflict of Interest No conflicts of interest between the authors and / or family mem- bers of the scientific and medical committee members or members of the potential conflicts of interest, counseling, expertise, working conditions, share holding and similar situations in any firm. Authorship Contributions Idea/Concept: Fatih Akdemir, Asuman Çelik; Design: Fatih Akdemir, Asuman Çelik; Control/Supervision: Fatih Akdemir; Fatih AKDEMİR et al. Turkiye Klinikleri J Case Rep. 2022;30(4):230-3 232 Fatih AKDEMİR et al. Turkiye Klinikleri J Case Rep. 2022;30(4):230-3 233 Data Collection and/or Processing: Fatih Akdemir, Asuman Çelik; Analysis and/or Interpretation: Fatih Akdemir, Asuman Çelik; Literature Review: Fatih Akdemir; Writing the Article: Fatih Akdemir, Asuman Çelik; Critical Review: Fatih Akdemir;

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