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Year : 2020  |  Volume : 63  |  Issue : 4  |  Page : 627-629
Mullerianosis of urinary bladder: The great impersonator

1 Department of Pathology, Homi Bhabha Cancer Hospital, Sangrur, Punjab, India
2 Department of Surgical Oncology, Homi Bhabha Cancer Hospital, Sangrur, Punjab, India

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Date of Submission18-Sep-2019
Date of Decision22-Dec-2019
Date of Acceptance31-Dec-2019
Date of Web Publication28-Oct-2020


Müllerianosis of the urinary bladder is a rare entity with less than 30 cases reported. It encompasses a mixture of different types of Mullerian lesions like endometriosis, endocervicosis, and endosalpingiosis. It typically affects the women of reproductive age group. The symptoms vary from hematuria and dysuria, to pelvic pain, which may be associated with menstruation. It can mimic a malignant tumor clinically and radiologically; hence, it is important that a correct diagnosis is rendered in these cases to avoid unnecessary radical procedures. Herein we report a case of mullerianosis of the urinary bladder in a young female and discuss the important differential diagnosis and various treatment modalities.

Keywords: Bladder, endometriosis, mullerianosis

How to cite this article:
Sancheti S, Somal PK, Chaudhary D, Khandelwal S. Mullerianosis of urinary bladder: The great impersonator. Indian J Pathol Microbiol 2020;63:627-9

How to cite this URL:
Sancheti S, Somal PK, Chaudhary D, Khandelwal S. Mullerianosis of urinary bladder: The great impersonator. Indian J Pathol Microbiol [serial online] 2020 [cited 2021 Jul 25];63:627-9. Available from: https://www.ijpmonline.org/text.asp?2020/63/4/627/299324

   Introduction Top

Müllerianosis of the urinary bladder is a rare and complex tumor like lesion, which was first described in 1996 as an admixture of atleast two types of Mullerian derived tissues.[1],[2] The two main forms that have been described are endometriosis and endocervicosis followed by endosalpingiosis.[3] It usually occurs in women of reproductive age group, and a history of past pelvic surgery can also be elicited in some cases. The common location for mullerianosis is the dome and the posterior wall of the urinary bladder.[3] Histological examination reveals tubules and glands lined by different types of müllerian epithelium located in the lamina propria and muscularis propria.[3],[4]

   Case History Top

A 37-year-old female presented with a history of dysuria and burning micturition of long-standing duration with symptoms typically starting 10 days before menstruation. There was no history of hematuria, pyuria or a past pelvic surgery. Abdominal examination and per speculum examination were normal. Computed tomography (CT) scan revealed a 3.3 × 2.1 cm lesion in the left posterior wall of the urinary bladder [Figure 1]a. A biopsy was done to rule out bladder cancer. Biopsy revealed admixture of endometrial glands and stroma along with endocervical type of glands lined by ciliated cells in the lamina propria and muscularis propria [Figure 1]b and c]. There was no cytological atypia or evidence of malignancy in the glands. No urothelial differentiation was noted. On immunohistochemistry the glands were strongly positive for estrogen receptor [ER, [Figure 1] d] and the endometrial stroma was highlighted by CD 10 [Figure 2]e, [Figure 2]f, [Figure 2]h. The presence of the deeply seated benign glandular structures lined by different types of mullerian epithelium was consistent with mullerianosis.
Figure 1: CT scan showing the lesion in the left posterior wall of bladder (white arrow). (a) Endometrial (green arrow) and endocervical glands (black arrow) situated in the lamina propria and muscular layer. (b and c), Nuclear staining for estrogen receptors in the endometrial glands and negative staining in urothelium (red arrow) (d)

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Figure 2: Endometrial glands situated deep in the muscularis layer. (a and d) Endometrial stroma highlighted by CD 10. (b and c)

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   Discussion Top

Müllerianosis was first described by Young and Clement in 1996 as a benign ectopic lesion with tissue of Müllerian descent.[1],[2] Endometriosis, endosalpingiosis, and endocervicosis are benign proliferations of Mullerian epithelium, and coexistence of any 2 or all types constitutes mullerianosis.[3]

The exact pathogenesis of mullerianosis is still debatable with two main theories being put forward. The implantation theory supports the histogenesis of cellular seeding in patients with a prior history of pelvic surgery, but fails to account for cases with no pelvic surgery and involvement of distant sites.[4],[5] The metaplastic theory, which postulates the differentiation of müllerian epithelium towards tubal, endometrial or endocervical phenotype is more widely accepted. Further supporting this pathogenesis is the invariable occurrence of mullerianosis in the posterior wall and dome of the bladder, which corresponds to its peritoneal covering and is responsive to female hormones.[5]

Müllerianosis of the bladder is an extremely rare entity with fewer than 30 cases reported.[1] Few cases have also been reported in spinal cord, inguinal nodes, ureter, and mesosalpinx.[4] It typically occurs in the women of reproductive age group with symptoms ranging from lower abdominal pain to dysuria, Polyuria and hematuria. Symptoms are often cyclical and coincide with the menstrual cycle.[3] On cystoscopy it usually presents as polypoidal mass like lesions or darkly colored cyst covered by hyperemic mucosa ranging from 1.0 to 4.5 cm located in the dome or posterior wall of the bladder.[3],[5] There may be associated scarring and fibrosis with distortion of the bladder wall, which raises suspicion for a malignancy.[3]

Few cases of mullerianosis have also been reported in males, especially with a history of receiving hormonal therapy for prostatic cancer. These typically affect the lower genitourinary tract. Persistent rudimentary Mullerian tissue present in prostatic utricle has been postulated as the likely cause. Another rare cause is the persistent Mullerian duct syndrome, wherein patients retain structures derived from Mullerian duct, but have normal external male genitalia.[6]

Müllerianosis assumes significance due to its ability to mimic a neoplasm based on history, radiological and cytological findings. The differential diagnosis spans an entire gamut of benign and neoplastic conditions including cystitis glandularis, urachal remnants, nephrogenic adenoma, bladder adenocarcinoma, and direct spread from cervix adenocarcinoma.[5],[7]

In contrast to mullerianosis, cystitis cystica, cystitis glandularis, and nephrogenic adenoma are superficially located in the lamina propria of the bladder wall with preservation of the muscularis propria, and do not stain for estrogen and progesterone receptors.[3],[5] Urachal remnants are frequently observed near the dome of bladder and can be differentiated from mullerianosis by the demonstration of endometrial stroma through CD10 immunostaining in the latter.[5] Possibility of the bladder wall infiltration by adenocarcinoma of cervix can be ruled out by examination and imaging of cervix.[7] Bladder adenocarcinoma can also have an infiltrative growth pattern with involvement of muscularis propria, however, in contrast to mullerianosis, the clinical presentation is usually in an older individual, and the glands show significant nuclear atypia, mitotic activity, and stromal reaction.[3],[5]

The preferred treatment modality for bladder mullerianosis depends upon the age of the patient, the location within the bladder, the size, number and depth of infiltration.[3] Pharmacologic treatment is the preferred line of management for certain sites such as spine where surgical intervention is not feasible. Nonetheless, for mullerianosis of bladder extensive endoscopic transurethral resection may be preferable with strict follow up. In cases with recurrence, repeat cystoscopy and resection may be required.[3],[5] Malignant transformation of bladder mullerianosis is extremely rare with only 1 reported case of endometriod carcinoma in literature.[8]

It is imperative to keep this rare entity in mind to avoid misdiagnosis, which has great therapeutic implications on the patient.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Patel A, Desai P, Malczewski F, Stephens D. Müllerianosis of the urinary bladder: A rare and problematic bladder tumour. BMJ Case Rep 2017;2017:bcr2016218772.  Back to cited text no. 1
Batt RE, Smith RA, Buck Louis GM, Martin DC, Chapron C, Koninckx PR, et al. Müllerianosis. Histol Histopathol 2007;22:1161-6.  Back to cited text no. 2
Olivia Vella JE, Nair N, Ferryman SR, Athavale R, Latthe P, Hirschowitz L. Müllerianosis of the urinary bladder. Int J Surg Pathol 2011;19:548-51.  Back to cited text no. 3
Amir RAR, Taheini KM, Sheikh SS. Mullerianosis of the urinary bladder: A case report. Case Rep Oncol 2018;11:206-11.  Back to cited text no. 4
Branca G, Barresi V. Müllerianosis of the urinary bladder: A rare tumor like lesion. Arch Pathol Lab Med 2014;138:432-6.  Back to cited text no. 5
González RS, Vnencak-Jones CL, Shi C, Fadare O. Endomyometriosis (”Uterus-like mass”) in an XY Male: Case report with molecular confirmation and literature review. Int J Surg Pathol 2014;22:421-6.  Back to cited text no. 6
Kudva R, Hegde P. Mullerianosis of the Urinary Bladder. Indian J Urol 2012;28:206-7.  Back to cited text no. 7
  [Full text]  
Guan H, Rosenthal DL, Erozan YS. Mullerianosis of the urinary bladder: Report of a case with diagnosis suggested in urine cytology and review of literature. Diagn Cytopathol 2012;40:997-01.  Back to cited text no. 8

Correspondence Address:
Sankalp Sancheti
Department of Pathology, Homi Bhabha Cancer Hospital, Sangrur, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_726_19

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