{"paper_id":"cef730ca-b499-4560-8ade-6b541b01f7a5","body_text":"Urinary Bladder Mullerianosis Causing\nRecurrent Abdominal Pain: A Rare Case Report\nand Review of the Literature\nManjeet Kumar 1 Kailash Chander Barwal 1 Girish Kumar Sharma 1 Kavita Mardi 2 Pamposh Raina 1\nSanjeev Sharma 3\n1 Department of Urology, Indira Gandhi Medical College & Hospital,\nShimla, Himachal Pradesh, India\n2 Department of Pathology, Indira Gandhi Medical College & Hospital,\nShimla, Himachal Pradesh, India\n3 Department of Surgery, Rajiv Gandhi Government Post Graduate\nAyurvedic College, Paprola, Kangra, Himachal Pradesh, India\nJ Health Allied Sci\nNU 2024;14:133 –136.\nAddress for correspondence Manjeet Kumar, MBBS, MS, FMAS, MCH,\nDepartment of Urology, Indira Gandhi Medical College & Hospital,\nShimla, Himachal Pradesh 171001, India\n(e-mail: manjeetkumar.1014@gmail.com).\nIntroduction\nMullerianosis in the urinary bladder is a very rare and\ncomplex tumor-like lesion. It is diagnosed when at least\ntwo types of Mullerian tissues, that is, endometriosis, endo-\ncervicosis, and endosalpingiosis, are seen in the urinary\nbladder tumor specimen.\nClement and Young first described Mullerianosis in\n1996.\n1,2 This is seen in young females, especially after pelvic\nsurgeries, typically hysterectomy, caesarean surgery, etc.\nThese lesions present with dysuria, hematuria, and pain\nlower abdomen. Radiologically, it presents as a mass in the\nposterior wall or dome of urinary bladder. Mullerianosis of a\nurinary bladder resembles bladder tumors, so transurethral\nresection is vital for diagnosis and treatment. We present a\ncase report of Mullerianosis in a female patient presenting\nwith recurrent lower abdominal pain.\nCase Summary\nA 31-year-old female presented with recurrent pain lower\nabdomen and dysuria. She did not have a history of hematu-\nria, pyuria, or previous pelvic surgery. She had consulted\nmultiple gynecologists with no relief. On evaluation for lower\nabdominal pain, detailed history and clinical examination\nwere done. Abdominal examination and per speculum\nKeywords\n► Mullerianosis\n► urinary bladder\n► transurethral\nresection of bladder\nAbstract Mullerianosis of the urinary bladder is a rare bladder lesion with fewer than 30 cases\nreported in the literature. It describes the ectopic presence of endocervical, endome-\ntrium, and endosalpingial tissues inside th e urinary bladder. It is diagnosed when at\nleast two of three Mullerian tissues, endome triosis, endocervicos is, and endosalpin-\ngiosis, are identi fied. Mullerianosis presents in females of the reproductive age group,\nespecially after pelvic surgery. Treatment involves resection of the bladder mass and\nruling out malignant pathology. Follow-up of Mullerianosis is vital, as recurrence is\ncommon. Medical therapy is also indicated w hen a diagnosis is established after the\nhistopathological examination of the tumor.\nWe report a 31-year-old female presenting with recurrent lower abdominal pain.\nCystoscopy showed a mass of 3 /C23 cm in the urinary bladder. Transurethral resection of\nbladder tumor was resected, and histopathology suggested a combination of endo-\nmetriosis, endocervicosis, and endosalpin giosis. Pain was resolved after surgery, but\nthe lesion recurred at 3 months, which was subsequently resected.\narticle published online\nApril 18, 2023\nDOI https://doi.org/\n10.1055/s-0043-1764360.\nISSN 2582-4287.\n© 2023. The Author(s).\nThis is an open access article published by Thieme under the terms of the\nCreative Commons Attribution License, permitting unrestricted use,\ndistribution, and reproduction so long as the original work is properly cited.\n(https://creativecommons.org/licenses/by/4.0/)\nThieme Medical and Scienti fic Publishers Pvt. Ltd., A-12, 2nd Floor,\nSector 2, Noida-201301 UP , India\nTHIEME\nCase Report 133\nArticle published online: 2023-04-18\n\nexamination were normal. Ultrasound abdomen and pelvis\nsuggested a urinary bladder mass for which cystoscopy\nwas planned. On cystoscopy, a mass of approximately\n3 /C23 cm at the posterior wall of urinary bladder was\nseen. Computed tomography scan pelvis showed a solid\nmass of 3 /C23 cm in the posterior wall of the urinary wall.\nTransurethral resection and biopsy of the mass were done\n(\n►Fig. 1A –D).\nBiopsy revealed an admixture of endometrial glands and\nstroma along with the endocervical type of glands lined by\nciliated cells in the lamina propria and muscularis propria,\nwithout cytological atypia or evidence of malignancy in the\nglands. No urothelial differentiation was noted. The presence\nof the deeply seated benign glandular structures lined by\ndifferent types of Mullerian epithelium was consistent with\nMullerianosis. All three types of Mullerian tissues —endome-\ntriosis, endocervicosis, and endosalpingiosis —were found\n(\n►Fig. 2).\nOn follow-up cystoscopy, a residual lesion was seen,\nwhich was resected and fulgurated. She was started on a\ncombination of estrogens and progestins. At the 6 months\nfollow-up, no intravesical lesion was seen on ultrasound and\ncystoscopy.\nDiscussion\nMullerianosis of the urinary bladder is a rare entity with less\nthan 30 cases described in the literature. It is diagnosed when\nat least two of three Mullerian tissues like endometriosis,\nendocervicosis, and endosalpingiosis are identi fied inside\nthe urinary bladder. It usually affects women of the repro-\nductive age group, that is, from the second to fifth decade of\ntheir lives. The symptoms vary from hematuria, dysuria to\npelvic pain, which may be associated with menstruation. It\nmost commonly occurs as a polypoid mass in the posterior\nwall or dome of the urinary bladder. It can mimic a malignant\ntumor clinically and radiologically; hence, a correct diagnosis\ncan be made only after resection followed by histopatholog-\nical examination. Histologically, it consists of glands of\nvarying sizes lined by endometrial, endocervical, or tubal\nepithelium. Mullerianosis is indistinguishable from other\ntumors that occur in the urinary bladder, clinically or radio-\nlogically.\n1–3 Habiba et al in a literature review described 27\ncases of Mullerianosis in the urinary bladder and three cases\nin the lower ureter.\n4\nIn addition to classical endometriosis, glandular lesions\nmade up of endocervical type glands (endocervicosis) can\nFig. 1 (A and B) Contrast-enhanced computed tomography scan showing a mass arising from the posterior wall of urinary bladder. ( C)\nCystoscopy shows a solid mass in the posterior wall of urinary bladder. ( D) Recurrent lesion at 3 months after transurethral resection of bladder\ntumor.\nJournal of Health and Allied Sciences NU Vol. 14 No. 1/2024 © 2023. The Author(s).\nUrinary Bladder Mullerianosis Causing Recurrent Pain Abdomen Kumar et al.134\n\n\nalso occur in the bladder. Clement and young first described\nthe presence of cervical tissue in the urinary bladder. 1,2\nBefore this, only endometriosis had been discovered in the\nurinary bladder. Other Mullerian tissues, such as tubal\nepithelium in the bladder, were described under the name\nof endosalpingiosis in case reports.\n1,5 The term Mullerianosis\nis used when there is a combination of any two of the three\nMullerian-type lesions.\n1\nTwo theories have been purposed for the pathogenesis of\nMullerianosis. First is the implantation theory, in which\nMullerian epithelium gets implanted after pelvic surgeries.\nHowever, this theory fails to explain the diagnosis of Muller-\nianosis in patients with no history of pelvic surgery. Another\nwidely accepted theory is the metaplastic theory, which\npurposed that the Mullerian epithelium gets differentiated\ninto the endometrial, tubal, and endocervical epithelium.\nThis theory explains the occurrence of Mullerianosis in the\nposterior wall and dome of the urinary bladder. Peritoneum\ncells on the urinary bladder get differentiated into Mullerian\nepithelium on being stimulated by hormones estrogens and\nprogestins.\n3,6,7\nGuan et al reported cases of Mullerianosis in females from\n28 to 53 years with no history of prior pelvic surgery or\ncaesarean section. He also reported the use of cytology in\ndifferentiating it from other bladder tumors.\n8\nEndometriosis inside the urinary bladder is seen in females\nin their second to fifth decades of life, although it can also be\nseen in postmenopausal females taking estrogen therapy.\nAlthough rarely, endometriosis may also be seen in men\nwith prostatic carcinoma who have received exogenous\nestrogen therapy. It presents as a mass lesion on cystoscopy\nin the trigone, posterior wall, and dome of the urinary\nbladder. About one-third of these cases resemble a solid\nbladder tumor-like adenocarcinoma.\nThe differential diagnosis of Mullerianosis includes a\nvariety of lesions both neoplastic and non-neoplastic. Cysti-\ntis cystica, cystitis glandularis, urachal remnants, and malig-\nnant tumors can mimic Mullerianosis of the urinary bladder.\nTubular structures lined by mucinous epithelium suggest\nurachal remnants; however, they are seen at the dome of the\nbladder as incidental findings and are usually surrounded by\na loose peritubular fibromuscular tissue.\n3,9\nTreatment of Mullerianosis consists of medical and surgi-\ncal options. Medical treatment comprised contraceptive pills\ncontaining estrogens and progesterone, progestins, and go-\nnadotropin-releasing hormones. 10 Several case reports\nreported similar findings and treatment in patients with\nMullerianosis.\nUrinary bladder Mullerianosis is treated by transurethral\nresection of the mass and followed up with ultrasound and\ncystoscopy. Some patients may require medical therapy.\nConclusion\nMullerianosis of the bladder is a rare lesion, which presents\nas a mass lesion mimicking a malignant tumor. Resection of\nmass and ruling out malignant bladder tumors is the fore-\nmost priority. The possibility of Mullerianosis should always\nbe kept in mind in a young female in the reproductive age\ngroup presenting with recurrent pain abdomen and dysuria.\nFollow-up in the case of Mullerianosis is vital since the\nrecurrence of the disease is common.\nFunding\nNone.\nConflict of Interest\nNone declared.\nAcknowledgments\nThanks to Dr Kalpana Sharma for providing expert advise\nand technical help.\nReferences\n1 Young RH, Clement PB. Müllerianosis of the urinary bladder. Mod\nPathol 1996;9(07):731 –737\n2 Amir RAR, Taheini KM, Sheikh SS. Mullerianosis of the urinary\nbladder: a case report. Case Rep Oncol 2018;11(01):206 –211\nFig. 2 (A and B) Histopathology of the resected mass shows endometriosis, endocervicosis, and endosalpingiosis.\nJournal of Health and Allied Sciences NU Vol. 14 No. 1/2024 © 2023. The Author(s).\nUrinary Bladder Mullerianosis Causing Recurrent Pain Abdomen Kumar et al. 135\n\n\n3 Kudva R, Hegde P. Mullerianosis of the urinary bladder. Indian J\nUrol 2012;28(02):206 –207\n4 Habiba M, Brosens I, Benagiano G. Müllerianosis, endocervicosis,\nand endosalpingiosis of the urinary tract: a literature review.\nReprod Sci 2018;25(12):1607 –1618\n5 Branca G, Barresi V. Müllerianosis of the urinary bladder: a\nrare tumorlike lesion. Arch Pathol Lab Med 2014;138(03):\n432 –436\n6 Margulis V, Lemack GE, Molberg K, Saboorian MH. Bladder\n“Müllerianosis” in a woman with lower urinary tract symptoms\nand hematuria. J Urol 2001;165(6 Pt 1):1996 –1997\n7 Donné C, Vidal M, Buttin X, et al. Müllerianosis of the urinary\nbladder: clinical and immunohistochemical findings. Histopa-\nthology 1998;33(03):290 –292\n8 Guan H, Rosenthal DL, Erozan YS. Mullerianosis of the urinary\nbladder: report of a case with diagnosis suggested in urine cytology\nand review of literature. Diagn Cytopathol 2012;40(11):997–1001\n9 Nazeer T, Ro JY, Tornos C, Ordonez NG, Ayala AG. Endocervical\ntype glands in urinary bladder: a clinicopathologic study of six\ncases. Hum Pathol 1996;27(08):816 –820\n10 Almatrafi MH, Alhazmi AM, Almosaieed BN. Mullerianosis of the\nurinary bladder. Urol Case Rep 2020;33:101333\nJournal of Health and Allied Sciences NU Vol. 14 No. 1/2024 © 2023. The Author(s).\nUrinary Bladder Mullerianosis Causing Recurrent Pain Abdomen Kumar et al.136","source_license":"CC0","license_restricted":false}