{"paper_id":"caef752f-9954-4e0e-b71a-d5c823ea2855","body_text":"Turkiye Klinikleri J Case Rep. 2022;30(4):230-3\n230\nThe majority of bladder tumors are transitional \ncell cancers originating from the urothelium. How-\never, some variant tumors can be seen, which can \ncause difficulties in diagnosis and treatment due to \ntheir very rare nature. Sarcomas, malignant fibrous \nhistiocytoma, malignant peripheral nerve sheath tu-\nmors, small cell, micropapillary, nested variant, \nsarcomatoid carcinoma, hemangiopericytoma, para-\nganglioma are rare tumors.\n1,2 Mullerianosis was first \ndescribed by Young and Clement in 1996. 3 Bladder \nmullerianosis is a very rare condition and histologi-\ncally, the affected tissue has glands associated with \nendometrial, endocervical and tubal epithelium. Al-\nthough the lesions mimic malignancy, they are be-\nnign and involve the lamina propria and muscularis \npropria in the bladder. It can be seen radiologically \nand macroscopically as a polypoid mass in the blad-\nder dome or posterior wall. Clinically, there may be \nhematuria, dysuria, and pelvic pain related or unre-\nlated to menstruation. Although the pathogenesis is \nnot fully clear, implantation and metastatic origin are \nshown are causes.\n4  \n CASE REPORT \nA 41-year-old multiparous female patient was ad-\nmitted to our outpatient clinic 2 years ago with com-\nplaints of vaginal bleeding and gross hematuria. \nPrevious history was consistent with 3 caesarean and \ntubal ligation. Abdominal and speculum examination \nwere normal. In pelvic ultrasonography, nodular le-\nsion of approximately 3 cm in diameter was observed \nin the posterior wall of the bladder. Urine culture, \nhemogram and biochemical parameters were normal. \nTransurethral resection was performed on the lesion \nin the bladder. In addition, endometrial curettage was \nperformed  because it was in close relationship with \nthe anterior endometrial wall and there was vaginal \nbleeding. Histopathological diagnosis was negative \nfor malignancy and was reported as compatible with \nmullerianosis in the bladder. The patient, who had no \npostoperative complaints and was followed up, pre-\nsented again 2 years later with gross hematuria and \nvaginal bleeding. In the pelvic ultrasonography and \nmagnetic resonance imaging, a polypoid mass with a \ndiameter of 1.5 cm on the posterior wall of the blad-\nMullerianosis: A Rare Tumor Like Lesion of the  \nUrinary Bladder \n     Fatih AKDEMİRa,     Asuman ÇELİKb \naClinic of Urology, Terme State Hospital, Samsun, Türkiye \nbClinic of Pathology, Samsun Gazi State Hospital, Samsun, Türkiye \nABS TRACT Mullerianosis is a very rare condition and involves the coexistence of 2 or more different types of mullerian lesions such as e n-\ndometriosis, endocervicosis and endosalpingiosis in the extrauterine area. It involves the bladder more frequently in the urinary system and is more \ncommon in women of reproductive age. In urinary system involvements, it may present clinically with hematuria, dysuria and pelvic pain. It can \nmimic malignant lesions of the bladder histopathologically and clinically. Therefore, although they are usually benign lesions, malignancy should \nbe excluded. In this article, we presented a 41-year-old mullerianosis case who presented with gross hematuria and vaginal bleeding. \n \nKeywords: Mullerianosis; bladder; surgical treatment\nDOI: 10.5336/caserep.2022-90551\nCASE REPORT\nCorrespondence: Fatih AKDEMİR \nClinic of Urology, Terme State Hospital, Samsun, Türkiye \nE-mail: nfatihakdemir@hotmail.com  \nPeer review under responsibility of Turkiye Klinikleri Journal of Case Reports.  \nRe ce i ved: 16 Apr 2022          Ac cep ted: 02 Sep 2022          Available online: 08 Sep 2022  \n2147-9291 / Copyright © 2022 by Türkiye Klinikleri. This is an open \naccess article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).\nTurkiye Klinikleri Journal of Internal Medicine \nTürkiye Klinikleri Journal of Case Reports\n\n\nder and a cystic mass with a diameter of 3 cm on the \nanterolateral of the uterine corpus were observed \n(\nFigure 1). In the cystoscopy, a cystic, polypoid mass \nof approximately 1.5 cm in size was observed on the \nposterior wall of the bladder. Therefore, transurethral \nresection was performed for the pathological diagno-\nsis. Resection was performed on the lesion in the en-\ndometrium by hysteroscopy, and endometrial \ncurettage was performed. Pathological diagnosis was \nreported as mullerianosis and endometrial polyp for \nbladder and uterus, respectively. After excluding the \npossibility of malignancy, the patient underwent total \nhysterectomy and partial cystectomy. The patient \nwhose pathological diagnosis was reported as en-\ndometrial/endocervical polyp and bladder mulleri-\nanosis was followed up (\nFigure 2 , Figure 3 ). The \npatient allowed the use of photographs and all medi-\ncal documents related to her illness and signed an in-\nformed-consent agreement. \n DISCUSSION \nBladder mullerianosis is defined as the involvement \nof at least 2 of the mullerian origin tissues such as en-\ndosalpinx, endometrium and endocervix in the lamina \npropria and muscularis propria of the bladder.\n3 The \nmost common of mullerian-derived tissues is en-\ndometriosis, while the rarest one alone is endocervi-\ncosis.5 It has been reported that it can also be seen in \nother areas such as the spinal cord, inguinal lymph \nnodes, ureter and mesosalpinx apart from the urinary \nsystem.\n6 The bladder is more affected in the urinary \nsystem, and half of the cases have undergone ce-\nsarean section or pelvic surgery. Often seen in \nwomen in the reproductive period.\n7 No cases of mul-\nlerianosis have been reported in men. However, it has \nbeen reported that endometriosis can be seen in men \nreceiving estrogen therapy for prostate cancer and \npostmenopausal women receiving exogenous estro-\ngen therapy.\n4 \nClinical symptoms such as hematuria, pelvic \npain, dysuria and renal colic may be seen in bladder \nmullerianosis. These symptoms are often concurrent \nwith the menstrual cycle. On cystoscopy, it can be \nFatih AKDEMİR et al. Turkiye Klinikleri J Case Rep. 2022;30(4):230-3\n231\nFIGURE 1: Magnetic resonance imaging. The lesion in the bladder (white \narrow) and the lesion originating from the endocervical canal and extending \ntowards the anterior wall of the uterus (yellow arrow) is observed. It is ob-\nserved that the integrity of the bladder wall is impaired.\nFIGURE 2: In the bladder transurethral resection material, polypoid tissue \nlined with urothelial epithelium (black arrow), single-layered and pseudostra-\ntified epithelium in the mucosa, locally cystic enlarged endometrial (blue \narrow), endocervical gland (red arrow) structures, and thick-walled vascular \nstructures with stroma were observed (H&E, 4x10).\nFIGURE 3: In immunohistochemical studies; positive (+) staining was ob-\nserved in the endometrial stroma (blue arrow) with CD10 (4x10).\n\nseen as a 1-4.5 cm polypoid mass or a cystic lesion, \nusually covered with hyperemic mucosa located on \nthe dome or posterior wall of the bladder.\n6,7 Benign \nand malignant conditions such as cystitis cystica and \nglandularis, urachal remnants, nephrogenic adenoma, \nbladder adenocarcinoma and cervical adenocarci-\nnoma metastasis should be kept in mind in the dif-\nferential diagnosis.\n6,8 Cystitis cystica, cystitis \nglandularis, and nephrogenic adenoma are superficial \nand do not involve the muscularis propria. Also, es-\ntrogen and progesterone receptors are not painted. \nUrachal remnants are usually observed in the bladder \ndome and appear as tubular structures covered with \nmucinous epithelium, surrounded by loose peritubu-\nlar fibromuscular tissue. It can be differentiated his-\ntologically by the presence of endometrial stroma \nthrough CD10 immunostaining. However, unlike \nmullerianosis, adenocarcinoma affects older individ-\nuals, and malignant transformation findings such as \nsignificant atypia, increased mitotic activity, and stro-\nmal reaction are observed in the glands in the urothe-\nlial epithelium.\n6,7,9 Malignant transformation of \nmullerianosis is a very rare condition, and Garavan F \net al. reported such a case.\n10 \nAlthough the pathogenesis of bladder mulleri-\nanosis has not been clearly explained, two main the-\nories have been proposed. According to the \nimplantation theory proposed by Young and \nClement, mullerian tissues are implanted in the blad-\nder during pelvic surgery or cesarean section.\n3 The \nfact that most of the cases with mullerianosis have a \nhistory of cesarean section or pelvic surgery supports \nthis theory. However, this theory is insufficient to ex-\nplain its presence in people who have not undergone \npelvic surgery or in other parts of the body away from \nthe uterus.\n11,12 Donne et al. proposed the metaplastic \ntheory.13 Accordingly, endometrial, endocervical and \ntubal components of mullerian origin may prolifer-\nate in the bladder. The fact that mullerianosis occurs \nin the posterior wall, which is an area covered by the \nperitoneum and sensitive to female hormones, sup-\nports this theor.\n13 Branca and Barresi suggested that \nthe secondary mullerian system forming the peri-\ntoneal mesothelium may preserve its ability to dif-\nferentiate into endometrial, endocervical, and tubal \ntissues.\n6 On the other hand, Koren et al. described a \nsmall tubal-type metaplastic ciliary epithelial focus \nin cystitis glandularis that is continuous with the \nurothelium and stained immunohistochemically pos-\nitive for estrogen and progesterone receptors. There-\nfore, they stated that mullerianosis may occcur \nthrough urothelial metaplasia in the setting of chronic \ninflammation.\n14 In this case, there was a history of 3 \ncesarean section operations, supporting the implan-\ntation theory. \nInitial treatment for mullerianosis is transurethral \nresection. After the histopathological diagnosis is \nmade, medical and surgical treatments can be applied \naccording to the age of the patient, size, number,  \nlocation and depth of infiltration in the bladder.  \nMedical treatment options such as combined  \nestrogen-progesterone contraceptives, progestins, and \ngonadotrop releasing hormone agonists may provide \nregression of symptoms. Repeated transurethral re-\nsections can be performed in cases that do not re-\nspond to medical treatment or have recurrences. \nLesions on the serosal surface of the bladder can be \nremoved laparoscopically. If the lesion has involved \nall layers of the bladder with the uterus, partial cys-\ntectomy and hysterectomy should be performed to \nprevent recurrence.\n6,7,15 \nIn conclusion, bladder mullerianosis is a very \nrare benign lesion. However, it is important to define \nmullerianosis, detailed histopathological analysis, ap-\npropriate treatment, and careful differential diagno-\nsis with malignant conditions. \nSource of Finance \nDuring this study, no financial or spiritual support was received \nneither from any pharmaceutical company that has a direct con-\nnection with the research subject, nor from a company that pro-\nvides or produces medical instruments and materials which may \nnegatively affect the evaluation process of this study. \nConflict of Interest \nNo conflicts of interest between the authors and / or family mem-\nbers of the scientific and medical committee members or members \nof the potential conflicts of interest, counseling, expertise, working \nconditions, share holding and similar situations in any firm. \nAuthorship Contributions \nIdea/Concept: Fatih Akdemir, Asuman Çelik;  Design: Fatih \nAkdemir, Asuman Çelik; Control/Supervision: Fatih Akdemir; \nFatih AKDEMİR et al. Turkiye Klinikleri J Case Rep. 2022;30(4):230-3\n232\n\nFatih AKDEMİR et al. Turkiye Klinikleri J Case Rep. 2022;30(4):230-3\n233\nData Collection and/or Processing: Fatih Akdemir, Asuman \nÇelik; Analysis and/or Interpretation: Fatih Akdemir, Asuman \nÇelik; Literature Review: Fatih Akdemir; Writing the Article: \nFatih Akdemir, Asuman Çelik; Critical Review: Fatih Akdemir; \nReferences and Fundings: Fatih Akdemir; Materials: Fatih \nAkdemir; Other: Fatih Akdemir, Asuman Çelik.\n1. Yücel C, Keskin MZ. Rare malign tumors of bladder: Review of the liter-\nature. J Reconstr Urol. 2017;7(1):19-24. [Crossref]  \n2. Sonmez G, Tombul ST, Golbasi A, Demirtas T, Akgun H, Demirtas A. \nSymptomatic paraganglioma of the urinary bladder: a rare case treated \nwith a combined surgical approach. Urol Case Rep. 2020;33:101290. \n[Crossref]  [PubMed]  [PMC]  \n3. Young RH, Clement PB. Müllerianosis of the urinary bladder. Mod Pathol. \n1996;9(7):731-7. [PubMed]  \n4. Amir RAR, Taheini KM, Sheikh SS. 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