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Introduction: Accessory cavitated uterine malformation (ACUM) is a relatively recent term used to describe a noncommunicating, accessory uterine cavity. ACUM have been published under different terms ranging from juvenile cystic adenomyosis to "uterus‐like mass". The objective of this study was to systematically identify all cases of ACUM and definitions described in the literature, regardless of label, and identify morphological, epidemiological, and clinical characteristics as well as management, while also highlighting knowledge gaps. Material and methods: A systematic literature search of three databases was performed, reviewing all records of cystic myometrial lesions. Cases that fitted common definitions for ACUM were included and clinical and imaging characteristics were documented in detail. This work was registered to PROSPERO and reporting followed PRISMA guidelines for scoping reviews. Results: A total of 53 articles were included, comprising 115 cases that met the minimal criteria for ACUM. The median age at onset of symptoms was 17 years, presenting with dysmenorrhea soon after menarche. A total of 19 women were parous. On ultrasound, ACUM appears as unilocular myometrial cysts, usually with ground‐glass content. Hemorrhagic content is also observed on magnetic resonance imaging (MRI), with high signal intensity on both T2 and T1‐weighted images. Ninety‐five (83%) cases were managed surgically, with a trend towards primary nonsurgical options. Although no adverse outcomes were reported, long‐term follow‐up on subsequent fertility and pregnancy was rare. Conclusions: Despite its increasing recognition as a clinical entity, ACUM often remains underdiagnosed as it shares similarities with other myometrial masses. We propose a unified terminology and definition for ACUM based on the data in this review. ACUM presents as a cavitated lesion, surrounded by a myometrial mantle, in continuity with the anterolateral uterine wall and located beneath the insertion of the round ligament and the interstitial portion of the fallopian tube. In contrast to other uterine abnormalities, a normal uterine cavity is visualized. Future studies are needed, using a clear definition for ACUM, and prospectively investigating management strategies, including long‐term follow‐up of patient‐reported symptoms, fertility, and pregnancy outcomes. [ABSTRACT FROM AUTHOR]

Anatomically, normal cells found in an abnormal site are known as choristoma. When any two of the three-cell lineage of the mullerian duct, that is endosalpinx, endocervix and endometrium, are found at an abnormal location, it is termed mullerian choristoma or mullerianosis. Mullerianosis histologically reveals glands of varying sizes lined by cervical, tubal and endometrial cells. Individual cell lineages like endometriosis of the ovary, endosalpingiosis and endocervicosis of the urinary bladder are common. But mullerianosis is quite rare, and as per literature, only about 20 cases have been reported. We report a mullerianosis involving the liver and lung in a 41-year-old female that mimicked metastatic biliary cystadenocarcinoma. It is the first case reported in literature where there is simultaneous involvement of the liver and lung by mullerianosis. The diagnosis was made with the help of histopathology and immunohistochemistry in the resected specimens. [ABSTRACT FROM AUTHOR]

Mullerianosis of the urinary bladder is a rare bladder lesion with fewer than 30 cases reported in the literature. It describes the ectopic presence of endocervical, endometrium, and endosalpingial tissues inside the urinary bladder. It is diagnosed when at least two of three Mullerian tissues, endometriosis, endocervicosis, and endosalpingiosis, are identified. Mullerianosis presents in females of the reproductive age group, especially after pelvic surgery. Treatment involves resection of the bladder mass and ruling out malignant pathology. Follow-up of Mullerianosis is vital, as recurrence is common. Medical therapy is also indicated when a diagnosis is established after the histopathological examination of the tumor. We report a 31-year-old female presenting with recurrent lower abdominal pain. Cystoscopy showed a mass of 3 × 3 cm in the urinary bladder. Transurethral resection of bladder tumor was resected, and histopathology suggested a combination of endometriosis, endocervicosis, and endosalpingiosis. Pain was resolved after surgery, but the lesion recurred at 3 months, which was subsequently resected. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to evaluate the clinical data on endosalpingiosis, which is only pathologically recognized, and which may accompany malignancies and endometriosis. Material and Method: We collected the data of patients who were pathologically diagnosed with endosalpingiosis between May 2017 and January 2023 in a tertiary health institution. The patient’s age, gravida, clinical complaints, imaging findings, presence of known risk factors for endosalpingiosis, the operation performed, and the location of the endosalpingiosis focus were recorded. The postoperative statuses of the patients were provided by hospital records and telephone interviews with the patients. Result: The data of six female patients between 34 and 62 years of age were analyzed. Two of the patients were in menopause, five had a history of previous abdominal surgery, one had endometriosis, and one patient had endosalpingiosis accompanying high-grade serose ovarian carcinoma. Conclusion: Considering the increase in synchronous malignancies, we believe that endosalpingiosis should be reported by pathology, especially in cases of endometriosis and those with a history of previous surgery, and the clinician should inform the patient of the presence of this diagnosis. [ABSTRACT FROM AUTHOR]

Background: Endosalpingiosis is assumed to be the second most common benign peritoneal pathology after endometriosis in women. Although recent studies indicate a significant association with gynecologic malignancies, many underlying principles remain unclear. This work aimed to systematically describe the intraoperative appearance of endosalpingiosis. Methods: Data and intraoperative videos of patients with histologically verified endosalpingiosis were retrospectively reviewed. The main outcome measures were macroscopic phenotype and anatomical distribution. Additionally, a systematic review searching PubMed (Medline) and Embase was conducted. Results: In the study population (n = 77, mean age 40.2 years (SD 16.4)), the mean size of lesions was 3.6 mm and the main visual pattern was vesicular (62%). The most frequent localization was the sacrouterine ligaments (24.7%). In the systematic review population (n = 1174 (210 included studies overall), mean age 45.7 years (SD 14.4)), there were 99 patients in 90 different studies with adequate data to assess the appearance of the lesions. The mean size of the lesions was 48.5 mm, mainly with a cystic visual pattern (49.5%). The majority of the lesions affected the ovaries (23.2%), fallopian tubes (20.4%), or lymph nodes (18.5%). Comparing this study to the literature population, the main differences concerned the size (p < 0.001) and main visual patterns (p < 0.001) of lesions. Conclusions: The usual intraoperative findings of endosalpingiosis appeared less impressive than described in the literature. In our study population, lesions of a few millimeters in size with a vesicular appearance were mostly seen, most frequently in the sacrouterine ligament area. Intraoperative recognition by the gynecologic surgeon and histologic diagnosis should play an important role in further understanding this entity, scientifically and clinically. [ABSTRACT FROM AUTHOR]

Mullerianosis is a very rare condition and involves the coexistence of 2 or more different types of mullerian lesions such as endometriosis, endocervicosis and endosalpingiosis in the extrauterine area. It involves the bladder more frequently in the urinary system and is more common in women of reproductive age. In urinary system involvements, it may present clinically with hematuria, dysuria and pelvic pain. It can mimic malignant lesions of the bladder histopathologically and clinically. Therefore, although they are usually benign lesions, malignancy should be excluded. In this article, we presented a 41-year-old mullerianosis case who presented with gross hematuria and vaginal bleeding. [ABSTRACT FROM AUTHOR]

Through microscopy, early researchers identified the epithelium on the inner surfaces of the uterus, cervix and Fallopian tubes. The identification of ectopic epithelium was gradual, starting from the gross pathology study of unusual cystic lesions. Towards the end of the nineteenth century, attention focused on the epithelium as a critical component. The term ‘adenomyoma’ was coined around eighteen eighty to designate the majority of mucosa-containing lesions. Several theories were advanced to explain its aetiology. In the main, lesions were considered to arise from invasion from uterine epithelium; implantation of endometrium through retrograde menstruation; hematogenous or lymphatic spread; or from embryonic remnants. Although initially widely rejected, around 1920, an almost unanimous consensus formed on the endometrial nature of epithelial invasions. During the following years, adenomyosis and endometriosis came to be used to distinguished lesions within or outside the uterus. Adenomyosis was attributed to direct infiltration of uterine mucosa into the myometrium, and endometriosis to the implantation of endometrial cells and stroma into the peritoneal cavity through retrograde menstruation. Around the same time, ovarian lesions, initially described as ovarian hematomas or chocolate cysts, were regarded as a form of endometriosis. Three variants of endometriosis were thus described: superficial peritoneal, deep nodular and ovarian endometriomas. Ectopic epithelium has long been recognised as having similarities to tubal, or cervical epithelium. Lesions containing mixed epithelium are often termed Müllerianosis. This article demonstrates the stepwise evolution of knowledge, the role of the pioneers and the difficulties that needed to be overcome. It also demonstrates the value of collaboration and the inter-connected nature of the scientific endeavour.

Abstract Background Müllerianosis is a very rare neoplasm composed of two or three Müllerian derived tissues (endosalpinx, endometrium and endocervix). We report the first case of concurrent müllerianosis of the urinary bladder and the umbilicus presenting with umbilical bleeding. Case presentation A 43-year-old Asian premesopausal female, gravida 1, para 1, presented with intermittent umbilical bleeding. An umbilical nodule and a bladder tumor on the posterior wall of the urinary bladder were identified. She underwent transurethral resection of the bladder tumor and excision of the umbilical nodule successively. Diagnosis of müllerianosis was confirmed by the histological and immunological features. No tumor recurrence was noted at 6 months of follow-up. Conclusions Müllerianosis is extremely rare and mainly reported in the urinary bladder, and generally affects women of reproductive age. Despite the common presentations of müllerianosis of the urinary bladder including irritative voiding symptoms, abdominal/pelvic pain and gross hematuria, our rare case had no symptom except umbilical bleeding. The possibility of concurrent bladder müllerianosis should be considered when müllerianosis is found at other location. We suggest a surgical intervention to establish the correct pathological diagnosis because it is essential to exclude malignant neoplasms of the urinary bladder. The majority of patients have a favorable prognosis.

Bladder müllerianosis is defined by the presence of Müllerian epithelium (endometrial, endocervical or endosalpinx) in the bladder. It is a rare benign disease that affects women and presents a non-specific clinical presentation that poses a broad differential diagnosis. We present the case of a 49-year-old woman who presented with recurrent urinary tract infections, urinary discomfort and abdominal pain. The approach is carried out by ultrasound and urethrocystoscopy that reveal the presence of a 5mm polypoid lesion that is removed. The histological study revealed bladder müllerianosis together with the complementary finding of glandular cystitis and cystic cystitis., (Copyright © 2024 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.)

Müllerianosis of the urinary bladder is a rare entity characterized by the presence of an admixture of at least two types of müllerian tissue in the muscularis propria of the bladder. We report the case of a 43-year-old male patient presented for urological evaluation due to episodes of total gross hematuria. Physical examination revealed ambiguous external genitalia. Abdomiopelvic CT scan revealed a tumor at the level of the bladder floor. Transurethral resection of the bladder lesions was done and histopathological studie confirmed the diagnosis of müllerianosis. Karyotype analysis showed a 46 XX male syndrome.

Endosalpingiosis refers to the ectopic presence of tubal epithelium. However, this incidental finding received little attention, although it is the second most common benign peritoneal pathology in women following endometriosis. In contrast to endometriosis, endosalpingiosis shows an increase in prevalence with age beyond the menopause. Furthermore, it does not appear to be chronically inflammatory and, according to research to this date, does not cause chronic pain or infertility. Recent epidemiological and molecular pathological studies show a significantly higher incidence of ovarian and endometrial tumors in women with endosalpingiosis. These correlations have not been conclusively clarified. Generally accepted clinical recommendations for the detection of endosalpingiosis do not yet exist. In order to better understand the disease value of endosalpingiosis and its oncological correlations, this entity should be brought to the attention of surgical gynecology and involved pathology. [ABSTRACT FROM AUTHOR]

Mullerianosis is a term used to describe a pathologic entity comprised of at least 2 types of Mullerian-type epithelia (tubal, endocervical, or endometrial) at non-gynecologic sites. This is an uncommon occurrence in the urinary tract. To the best of our knowledge, only 3 instances of Mullerianosis involving the ureter have been reported. We present a fourth report and describe the clinical, radiological, and histopathologic findings. Awareness of this rare process is crucial to avoid misdiagnosis. [ABSTRACT FROM AUTHOR]

Endometriosis is the ectopic presence of endometrial tissue outside of the uterus. Bladder involvement of these lesions is not uncommon and has been reported and studied extensively. Mullerianosis is a term that describes the ectopic presence of tissues from the endocervix and endosalpinx in extrauterine sites. Only a few cases of Mullerianosis involving the urinary tract system have been reported in literature. We report a case of a 32-year-old lady with bladder mass and periodic hematuria and dysuria. She underwent TURBT 1and on histopathology was found to have bladder Mullerianosis. Patient was later treated with oral contraceptives with improvement.

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.

Background: Müllerianosis is a very rare neoplasm composed of two or three Müllerian derived tissues (endosalpinx, endometrium and endocervix). We report the first case of concurrent müllerianosis of the urinary bladder and the umbilicus presenting with umbilical bleeding.Case Presentation: A 43-year-old Asian premesopausal female, gravida 1, para 1, presented with intermittent umbilical bleeding. An umbilical nodule and a bladder tumor on the posterior wall of the urinary bladder were identified. She underwent transurethral resection of the bladder tumor and excision of the umbilical nodule successively. Diagnosis of müllerianosis was confirmed by the histological and immunological features. No tumor recurrence was noted at 6 months of follow-up.Conclusions: Müllerianosis is extremely rare and mainly reported in the urinary bladder, and generally affects women of reproductive age. Despite the common presentations of müllerianosis of the urinary bladder including irritative voiding symptoms, abdominal/pelvic pain and gross hematuria, our rare case had no symptom except umbilical bleeding. The possibility of concurrent bladder müllerianosis should be considered when müllerianosis is found at other location. We suggest a surgical intervention to establish the correct pathological diagnosis because it is essential to exclude malignant neoplasms of the urinary bladder. The majority of patients have a favorable prognosis. [ABSTRACT FROM AUTHOR]

Mullerianosis is a rare entity consisting of an admixture of 2 or more of the following tissues: endometriosis, endocervicosis, and endosalpingiosis. It most commonly affects the urinary bladder and affects females of fertile age. It presents clinically as hematuria, dysuria, and pelvic pain which may be associated with menstruation. Radiologically and macroscopically, it typically presents as a polypoid mass in the dome or posterior wall of the bladder. Histologically, it consists of glands of varying size lined by endometrial, endocervical, or tubal epithelium. Mullerianosis clinically and histologically mimics other benign and malignant lesions. Herein we report a case of mullerianosis of the urinary bladder. This is a rare lesion with less than 20 cases reported in the literature thus far. We believe raising awareness of this poorly recognized entity is of utmost significance in order to avoid misdiagnosis and the following unnecessary radical procedures.

Mullerianosis is a rare, complex, benign tumor most commonly found in the bladder and often mistaken for a neoplastic lesion.  Herein, we report a case of mullerianosis in a 65-year-old woman who presented with an incidental 2 cm bladder mass found on cross-sectional imaging.  A mixed cystic and solid tumor was identified on cystoscopy and a transurethral resection of the suspected tumor was performed with histopathology confirming a final diagnosis of mullerianosis.  While an unusual diagnosis, mullerianosis of the urinary bladder needs to be correctly identified to provide appropriate treatment and avoid misdiagnosis.

Endosalpingiosis is a benign condition characterized by the presence of tubal-type epithelial cells outside the Fallopian tube. It may rarely involve the uterus and present as a cystic or tumor-like mass. We report an unusual case of cystic uterine endosalpingiosis in a postmenopausal female with carcinoma endometrium. Preoperative and intraoperative diagnosis of this condition is challenging. Awareness about this condition in clinicians may help in preventing misdiagnosis and overtreatment. [ABSTRACT FROM AUTHOR]

Introduction and Hypothesis: We reviewed reported cases of endocervicosis, endosalpingiosis, and müllerianosis of the bladder or ureters in women in order to highlight these conditions as distinct from endometriosis or adenomyosis. Methods: Medline and Scopus searches of all cases published till the October 1, 2017. All retrieved references were searched manually to identify relevant articles. Results: Twenty-six articles reported endocervicosis. These included 37 cases described by the authors as endocervicosis of the bladder. We identified 7 case reports of bladder endosalpingiosis. There were 26 publications including 27 cases of müllerianosis of the bladder and 3 cases affecting the lower ureter. Literature descriptions are limited but available accounts do not point to features that uniquely distinguished these conditions. Affected women tended to be older, and in many cases, there was a possible relation to past surgery and particularly to hysterectomy and caesarean sections. Many cases were reported in postmenopausal women. Local excision was sufficient in most cases. Conclusion: The etiology of these conditions remains speculative, but case reports raise a possible link to pelvic surgery. There is a need for more detailed accounts of these lesions, which should be considered in the differential diagnosis of pre- and postmenopausal women with pelvic pain, dyspareunia, lower abdominal pain or discomfort, dysuria, frequency, or hematuria. [ABSTRACT FROM AUTHOR]

Bladder endocervicosis is a benign disease, characterized by the presence of ectopic endocervical tissue in the bladder wall, with few cases described in the literature. A case of bladder endocervicosis in a 35-year-old female, with the previous history of cesarean section, subtotal hysterectomy, and right oophorectomy is reported here. The patient was asymptomatic and the bladder lesion was detected on pelvic ultrasonography. Transurethral bladder resection was performed and the histopathological analysis revealed bladder endocervicosis. 12 months of follow-up have passed without evidence of clinical or radiological recurrence. Clinical presentation, pathological features, and treatment are discussed.

Müllerianosis is the term used to designate lesions composed of an admixture of two or three types of müllerian-derivation glands in heterotopic location. In this report, we describe a case of incidental vaginal müllerianosis in a 59-year-old woman who underwent rectosigmoidectomy for rectal adenocarcinoma. In the vaginal cuff removed for neoplastic invasion, a separate multilocular mass measuring 1.5 cm was found. The microscopic examination of the vaginal wall revealed endosalpingeal, endocervical and endometrial dilated or cystic glands with predominance of the endosalpingeal epithelium. Müllerian epithelium showed positivity for cytokeratins 7 and 8/18, high molecular weight cytokeratin, estrogen receptor alpha, and androgen receptor. The periglandular stroma was condensed and reactive for smooth-muscle actin, h-caldesmon, and CD10. To the best of our knowledge, a case of vaginal müllerianosis has not been previously reported. This lesion should be differentiated form vaginal adenosis and primary well-differentiated vaginal adenocarcinoma. The vagina should be added to the list of locations in which müllerianosis can be observed. [ABSTRACT FROM AUTHOR]

Objective: To describe the anatomical distribution and intraoperative morphology of endosalpingiosis.Design: Retrospective observational video study.Setting: Data and intraoperative videos were reviewed by two independent reviewers at one referral center. The study was approved by the local ethics committee.Patient(s): A total of 77 patients with histologically proven endosalpingiosis from 2007-2020.Intervention(s): None.Main Outcome Measure(s): The primary endpoints were anatomical distribution and macroscopic phenotype. The secondary endpoints were demographic and clinical characteristics as well as associated diseases.Result(s): Of the 77 patients with endosalpingiosis, the mean age was 40.2 years (standard deviation, 16.4 years), mean body mass index 24.1 kg/m2 (standard deviation, 5.7 kg/m2), 59.7% (n = 46) were nulligravide, 70.1% (n = 54) nulliparous, 22.1% (n = 17) suffered of infertility, and 53.2% (n = 41) had at least one previous abdominal or vaginal surgery. Endometriosis was associated in 53.2 % (n = 41) and malignancies in 28.6% (n = 22, 7 endometrial cancers, 1 uterine carcinosarcoma, 8 borderline ovarian tumors, 5 epithelial ovarian cancers, and 1 yolk sac tumor of the ovary). Anatomic distribution and varying intraoperative phenotypes were demonstrated in the video presentation.Conclusion(s): In the majority of this population, endosalpingiosis was located in the pelvis. The higher prevalence of specific gynecologic tumors is consistent with previous results. In phenotype, most lesions appear to be less spectacular than prominent in the literature. For further studies on the relevance as a risk factor for malignancy and consequently clinical recommendations, sound knowledge about endosalpingiosis of laparoscopists as initial diagnosticians is crucial. [ABSTRACT FROM AUTHOR]

“Endosalpingiosis” refers to the presence of fallopian tube-like epithelium outside fallopian tubes. It merits attention both by Clinicians and Pathologists since it is a benign, non- neoplastic condition which maybe easily misjudged as carcinoma mainly due to the rarity of its occurrence and lack of awareness owing to paucity of its documentation in literature. Further, salpingiosis can undergo massive cystic change thereby mimicking a tumour when it is referred to as cystic salpingiosis. We present one such extremely rare case of cystic salpingiosis of uterine parametrium in a 40-year-old lady along with an extensive review of literature which revealed that about 10 cases have been documented till date.

Endocervicosis is a rare benign condition characterized by the presence of endocervical-type mucinous glands. Urinary bladder endocervicosis forms an elevated lesion in the posterior wall of the urinary bladder and is sometimes misdiagnosed as a malignant tumor clinically and pathologically. Herein we describe the first case of adenocarcinoma arising in urinary bladder endocervicosis. The patient, a 58-year-old woman, presented with asymptomatic hematuria. Cystoscopy revealed a nodular mass measuring 4 cm in diameter in the posterior wall, and total cystectomy was performed. Histology revealed that the elevated lesion of the bladder wall was composed of haphazard proliferation of cystic glands lined by benign endocervical-type epithelium. An adenocarcinoma arose at the center of this endocervicosis. Mucin histochemistry revealed the presence of sulfomucin in both the endocervicosis and adenocarcinoma components. Immunohistochemically, the endocervicosis was positive for cytokeratin ( CK) 7, AE1/ AE3, CAM5.2, HBME1, CA19-9, and estrogen receptor ( ER), and negative for CK20, CDX2, progesterone receptor ( PR), MUC5 AC, and β-catenin. The adenocarcinoma showed similar immunohistochemical results, except for loss of ER expression and a slight increase in the ratio of Ki-67-positive cells. This case indicates that endocervicosis, known as a benign lesion, harbors the possibility of malignant transformation. [ABSTRACT FROM AUTHOR]

Introduction: Endometriosis is a condition in which endometrial tissue exists outside the uterine cavity. Urinary tract lesions are present in 1 % of women with this condition. Case Presentation: A 34-year-old woman was presented to our clinic with a 3-year history of dysuria and frequency. Her past medical history was significant for cesarean sections, performed 3 years earlier. In our center, the patient underwent cystoscopy and transurethral resection of a lesion found in the bladder wall, which on histopathology examination appeared to be endometriosis. A course of six-month hormonal therapy with luteinizing hormone-releasing hormone (LHRH)-agonist (Dipherelin™ 3.75 IM per 28 day) was planned for her. One month later, trans-vaginal ultrasound revealed a nodule posterior to the bladder, which had grown 1cm in the preceding month. Therefore, Hysterectomy, bilateral oophorectomy, and partial cystectomy were performed. The pathology report was Mullerianosis. During two years of follow up, the patient remained asymptomatic. Conclusions: Endometriosis and endocervicosis should be regarded as important differential diagnoses in patients with gross hematuria and those suspected to have urothelial carcinomas. [ABSTRACT FROM AUTHOR]

Bladder endocervicosis is a benign disease, characterized by the presence of ectopic endocervical tissue in the bladder wall, with few cases described in the literature. A case of bladder endocervicosis in a 35-year-old female, with the previous history of cesarean section, subtotal hysterectomy, and right oophorectomy is reported here. The patient was asymptomatic and the bladder lesion was detected on pelvic ultrasonography. Transurethral bladder resection was performed and the histopathological analysis revealed bladder endocervicosis. 12 months of follow-up have passed without evidence of clinical or radiological recurrence. Clinical presentation, pathological features, and treatment are discussed. [ABSTRACT FROM AUTHOR]

We are reporting a case of Mullerianosis, which presented as a multiloculated cystic mass on the serosal surface of the fundus of the uterus. Clinically and radiologically, this was interpreted as an ovarian tumor. Mullerianosis is a very rare benign tumor-like lesion. Awareness of this lesion is necessary to avoid misdiagnosis by clinicians, radiologists, and pathologists. [ABSTRACT FROM AUTHOR]

Purpose: Endometriosis is a common disease; however, ectopic müllerian tissue within the spine is a rare entity with the potential for producing significant neurological compromise. There are several postulated etiologies for this phenomenon, and only a few case reports are available in the world literature. Knowledge of this rare phenomenon is of paramount importance, since early diagnosis can lead to lessened neurological morbidity. Methods: In this manuscript, we present a case report, discuss gynecological and neurosurgical perspectives relating to the treatment strategies for managing this entity, and propose an alternative explanation for such an occurrence from a neurogenetic standpoint. Results: We present a case of spinal müllerianosis within the conus medullaris which was managed symptomatically for several years with an intracystic drain and subcutaneous reservoir. Over the years, it became clear that there was a cyclical presentation to her clinical malady, which at times was severe. Ultimately, she required surgical resection which aided in her diagnosis and subsequent treatment. Conclusion: Intraspinal müllerianosis is a rare location for an otherwise common disease in women and has the potential to create significant neurological morbidity by creating a mass lesion. Although the exact etiology remains unclear, the histogenic theories of embryologic origin appear most plausible. Treatment strategies for this condition may include hormonal therapy, obstetrical surgery, or open spinal surgery. This unusual and poorly understood disease should be considered in the differential diagnosis for intraspinal lesions presenting with hemorrhage in the clinical context of cyclical neurological symptoms. [ABSTRACT FROM AUTHOR]

The theory of müllerianosis predicts that embryonic müllerian tissue, misplaced during organogenesis, results in the formation of 4 benign müllerian diseases—developmental adenomyosis, endometriosis, endosalpingiosis, and endocervicosis—(developmental müllerian diseases) that will be identified in human female fetuses, infants, children, adolescents, and adults. Direct evidence is presented to support the existence of developmental adenomyosis, developmental endometriosis, and developmental endocervicosis in human female fetuses along with strong circumstantial evidence supporting the existence of all 4 developmental müllerian diseases in human female infants, children, adolescents, and adults. This evidence throws light upon the pathogenesis of rare müllerian lesions whose pathogenesis remains inexplicable by classical and modern theories. Furthermore, this research has scientific and clinical relevance: scientific relevance because it opens up a new field of comparative research—the 4 developmental müllerian diseases complement the 4 acquired müllerian diseases; clinical relevance because it identifies rare müllerian diseases curable by complete surgical excision. [ABSTRACT FROM AUTHOR]

Müllerianosis of the urinary bladder is a rare condition that encompasses 3 histological entities (endometriosis, endocervicosis, and endosalpingiosis). The authors report 2 patients with bladder müllerianosis, one of whom had endometriosis and the other a history of past pelvic surgery, describe the histological and cystoscopic features, and review the literature on this condition. Mucosal hyperemia and submucosal nodules or cysts with associated fibrosis and distortion of the bladder wall may mimic malignancy on cystoscopy, as may the infiltrative growth pattern sometimes evident histologically. Recognition of this complex diagnostic entity is important to avoid misdiagnosis and inappropriate investigation. There is a clinical association with endometriosis and past pelvic surgery. [ABSTRACT FROM PUBLISHER]

Several endometriosis classifications were proposed, based on the assumption that endometriosis is a progressive disease, and designed to score severity of visible lesions. In addition, several specific classifications, e.g., for deep endometriosis, were proposed. None of these classifications however, have been validated to be predictive for diagnosis, treatment prognosis, recurrence, progression or for the associated infertility or pain. The difficulties derive from the fact that pathophysiology and the natural history are still uncertain. A classification should avoid assumptions. It seems established beyond reasonable doubt that endometriosis presents as subtle, typical, cystic, and deep lesions and that severity of each lesion is related to size or volume. By pathology, these four lesions present as active, burnt-out, inactive, and active lesions, respectively. Besides this, there are many uncertainties. It is unclear whether endometriosis is one disease progressing ultimately into severe endometriosis or whether typical, cystic, and deep endometriosis represents three different diseases, each being an end stage. It is unclear whether endometriotic cells are different from endometrial cells or whether only the environment is different. It is unclear how adenomyosis, Müllerianosis, and peritoneal pockets should be considered. We therefore suggest a descriptive classification with the severity of Subtle, Typical, Cystic, Deep, Adenomyotic, and peritoneal pocket lesions, estimated by their area or volume. This classification should permit to evaluate the actual uncertainties in order to build subsequently a validated classification. The similarity of the classes for superficial and cystic lesions with the rAFS classification is considered an advantage. It is discussed why adhesions need not to be scored. In conclusion, a simple classification scoring separately severity of subtle, typical, cystic, deep, adenomyotic, and peritoneal pocket lesions is suggested. This will permit to confirm or reject statistically many of the actual uncertainties on endometriosis and to evaluate what the predictive power of the severity of each type of lesion is, both essential elements for a validated endometriosis classification. [ABSTRACT FROM AUTHOR]

Abstract: Arias-Stella reaction is a hormone-induced atypical endometrial change that may involve endometrial, extraendometrial, or endometriotic sites. Establishing a correct diagnosis of Arias-Stella reaction may sometimes be problematic when seen outside of the uterine corpus, particularly in a limited biopsy sample when the pathologist is not aware of the clinical history. We report an Arias-Stella reaction in bladder endometriosis, which presented as a bladder mass in a 25-year-old pregnant woman. Clinical information of intrauterine pregnancy (week 16) was not initially provided when the biopsy was performed. We also discuss endometriosis, endocervicosis, and müllerianosis of the bladder, which are benign glandular lesions that may mimic tumors within the bladder wall. [Copyright &y& Elsevier]

Mullerianosis of the urinary bladder is a rare and morphologically complex tumor-like lesion, composed of several types of mullerian lesions like endometriosis, endocervicosis, and endosalpingiosis. This disease occurs in women of reproductive age group. Implantative and metaplastic origins have been suggested in the pathogenesis.

Mullerianosis is a sporadic condition of the urinary bladder. The study aims to present a case of mullerianosis with a brief literature review. A 52-year-old lady presented with hematuria for one-year duration. A general urine examination showed microscopic hematuria. Abdominal and transvaginal ultrasonography revealed a soft tissue mass (13 * 10 mm) within the base of the urinary bladder. Cystoscopy showed 1.0 * 1.3 cm benign-looking mass bulging into the posterior bladder wall covered by a normal bladder mucosa. Transurethral resection was done. The histopathological examination was consistent with mullerianosis. Two hypotheses have been proposed; the first one (implantation theory) believing that at the time of pelvic surgery and caesarian delivery Mullerian tissues become implanted inside the bladder. The second theory (metaplastic theory) holds the probability of differentiation of Mullerian epithelium to endometrial, endocervical, and tubal components and proliferation in the bladder. mullerianosis is a very infrequent entity that mainly affects the urinary bladder and is mainly reported in females during the reproductive period, medical and surgical treatment are both regarded as the management options. • Mullerianosis is an extremely rare condition of the urinary bladder. • Mullerian-derived components are endosalpinx, endometrium, and endocervix. • Clinical symptoms vary from dysuria, abdominal pain, hematuria and recurring renal colic. • In this study a case of mullerianosis is discussed. [ABSTRACT FROM AUTHOR]

We report a case of mullerianosis involving the urinary bladder. A 45-year-old woman presented with complaints of chronic pelvic pain, dysmenorrhoea, irritative lower urinary tract symptoms and cyclical haematuria. Cystoscopy and transurethral biopsy suggested mullerianosis involving the posterior bladder wall; she subsequently had surgery including partial cystectomy with complete excision of the lesion. Histology reported presence of endometrial, endocervical and endosalpingeal tissues within the bladder mass confirming mullerianosis. She made satisfactory postoperative recovery. Awareness of this lesion is necessary for proper diagnosis and appropriate treatment. [ABSTRACT FROM AUTHOR]