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International Urogynecology Journal Jun 2015Cystoscopy is frequently performed by gynecologists to ensure ureteral patency and to prevent bladder injury when performing concomitant gynecological procedures....
INTRODUCTION AND HYPOTHESIS
Cystoscopy is frequently performed by gynecologists to ensure ureteral patency and to prevent bladder injury when performing concomitant gynecological procedures. Generally, there are no additional findings on cystoscopy; however, when abnormalities arise, they may require either observation or intervention. Our aim was to create a visual library of benign, malignant, and foreign-body pathological conditions incidentally encountered on cystoscopy.
METHOD
Cystoscopic findings were videotaped at the time of routine surgical care. Regarding Institutional Review Board approval, individual consent was waived as the videos were de-identified and collected for educational purposes.
RESULTS
Benign pathological conditions: squamous metaplasia, duplicated ureteral orifice, ureterocele, Hutch diverticulum, bladder trabeculation, urachal cyst, interstitial cystitis with and without Hunner's lesion, endometriosis in the bladder, port-wine stain due to Klippel-Trénaunay-Weber syndrome, nephrogenic (mesonephric) metaplasia, and cystitis glandularis (intestinal metaplasia). Malignant pathological conditions: papillary urothelial neoplasm of low malignant potential (PUNLMP), carcinoma in situ (CIS), high-grade urothelial carcinoma, and urachal cancer. Foreign-body pathological conditions: edema from ureteral stents and stone-encrusted mesh.
CONCLUSION
This video is intended to educate the audience on some incidental bladder findings seen on female cystoscopy. Many pathological conditions can be biopsied or treated immediately during the procedure; hence, early urology consultation is encouraged for most abnormalities.
Topics: Cystitis, Interstitial; Cystoscopy; Female; Foreign Bodies; Gynecologic Surgical Procedures; Humans; Incidental Findings; Intraoperative Complications; Urethra; Urinary Bladder; Urinary Bladder Diseases; Urinary Bladder Neoplasms
PubMed: 25619539
DOI: 10.1007/s00192-014-2614-4 -
Strahlentherapie Und Onkologie : Organ... Sep 2016Mesonephric adenocarcinoma of the vagina is an extremely rare tumor of the female genital tract, with only a few cases reported so far worldwide. Consequently, there is... (Review)
Review
BACKGROUND
Mesonephric adenocarcinoma of the vagina is an extremely rare tumor of the female genital tract, with only a few cases reported so far worldwide. Consequently, there is no established standard treatment and limited knowledge about the prognosis and biologic behavior of vaginal mesonephric adenocarcinoma.
METHODS
This report documents a new case of vaginal mesonephric adenocarcinoma diagnosed in a 54-year-old woman, and analyzes this in the context of all previously published cases.
RESULTS
MRI demonstrated that the 2.5 × 1.8 cm tumor of the vaginal wall was invading urethra and bladder. Following surgical excision, histologic analysis determined mesonephric adenocarcinoma of the vagina, stage pT2 R1. In order to avoid the mutilating extended surgery which would be required to reach R0 and considerable impairment of quality of life, adjuvant radiochemotherapy was administered with external radiation and brachytherapy, including 5 cycles of cisplatin (40 mg/m²) for radiosensitization. After 4 years of continuous oncologic follow-up, the patient is alive and clinically free of disease.
CONCLUSION
In this case it was shown that adjuvant radiochemotherapy with radiation and brachytherapy was effective to manage the surgical R1 situation and maintain the patient's life quality. More published cases reports are needed to gradually substantiate optimal treatment strategies.
Topics: Brachytherapy; Chemoradiotherapy; Dose Fractionation, Radiation; Female; Humans; Middle Aged; Neoplasm Invasiveness; Radiotherapy, Conformal; Rare Diseases; Treatment Outcome; Vaginal Neoplasms; Wolffian Ducts
PubMed: 27349710
DOI: 10.1007/s00066-016-1004-x -
Medicine Nov 2020Mesonephric-like adenocarcinoma (MLA) from ovary is a very rare tumor which derives from mesonephric duct remnant of the female genital tract. Only six cases have been... (Review)
Review
RATIONALE
Mesonephric-like adenocarcinoma (MLA) from ovary is a very rare tumor which derives from mesonephric duct remnant of the female genital tract. Only six cases have been reported so far in the English literature.
PATIENT CONCERNS
A 29-year-old female patient was referred to the local hospital with a 20-day history of abdominal discomfort.
DIAGNOSES
Pelvic ultrasound examination revealed a solid and cystic mass measuring 10 cm in diameter in the right adnexal area and a cystic mass measuring 5 cm in the left adnexal area. Postoperative pathology in the local hospital revealed suspected malignancy of the right ovary, and she was then transferred to our institution for definite diagnosis. The tumor mass was finally diagnosed as a primary MLA arising from the right ovary by histological and immunohistochemical examination in our institution.
INTERVENTIONS
The patient underwent laparoscopic right adnexectomy and removal of left ovarian cyst in the local institution. Then, she underwent a complete staging surgery including a total hysterectomy, left adnexectomy, pelvic plus para-aortic lymphadenectomy, and omentectomy in our hospital. In addition, she received four cycles of combination chemotherapy with carboplatin plus paclitaxel.
OUTCOMES
There is no evidence of recurrence with 13 months of follow-up till now, and we are still following-up this patient.
LESSONS
MLA is an extremely uncommon malignancy with difficult diagnosis, unclear treatment and poor prognosis. Familiarizing with the clinical features and optimal management of this rare tumor may increase awareness of the disease among clinicians and pathologists, thus avoiding the misdiagnosis and mistreatment.
Topics: Adenocarcinoma; Adult; Chemotherapy, Adjuvant; Female; Humans; Ovarian Neoplasms; Rare Diseases; Wolffian Ducts
PubMed: 33235131
DOI: 10.1097/MD.0000000000023450 -
Radiology Case Reports Jan 2023Zinner syndrome is a rare congenital malformation of the mesonephric duct comprising of seminal vesicle cyst, ipsilateral renal agenesis, and ejaculatory duct...
Zinner syndrome is a rare congenital malformation of the mesonephric duct comprising of seminal vesicle cyst, ipsilateral renal agenesis, and ejaculatory duct obstruction. Clinical presentation varies with perineal pain, painful ejaculation, hematospermia and infertility common presenting complaints. Here, we present a case of Zinner syndrome in a 35-year-old male with a rare clinical presentation of only abdominal discomfort. The purpose of this case report is to highlight the challenging clinical presentation of Zinner syndrome and the use of imaging modalities in diagnosing the condition.
PubMed: 36353249
DOI: 10.1016/j.radcr.2022.10.006 -
Development (Cambridge, England) Sep 2021During development of the mouse urogenital complex, the gonads undergo changes in three-dimensional structure, body position and spatial relationship with the...
During development of the mouse urogenital complex, the gonads undergo changes in three-dimensional structure, body position and spatial relationship with the mesonephric ducts, kidneys and adrenals. The complexity of genital ridge development obscures potential connections between morphogenesis and gonadal sex determination. To characterize the morphogenic processes implicated in regulating gonad shape and fate, we used whole-embryo tissue clearing and light sheet microscopy to assemble a time course of gonad development in native form and context. Analysis revealed that gonad morphology is determined through anterior-to-posterior patterns as well as increased rates of growth, rotation and separation in the central domain that may contribute to regionalization of the gonad. We report a close alignment of gonad and mesonephric duct movements as well as delayed duct development in a gonad dysgenesis mutant, which together support a mechanical dependency linking gonad and mesonephric duct morphogenesis.
Topics: Animals; Embryo, Mammalian; Female; Gestational Age; Gonads; Kidney; Male; Mesonephros; Mice; Mice, Inbred C57BL; Morphogenesis; Sex Differentiation; Wolffian Ducts
PubMed: 33795229
DOI: 10.1242/dev.199208 -
Medicine Sep 2021Mesonephric adenocarcinoma (MNAC) is a very rare tumor that originates from mesonephric duct remnants of the female genital tract. Only a few cases were reported in the...
Mesonephric adenocarcinoma (MNAC) is a very rare tumor that originates from mesonephric duct remnants of the female genital tract. Only a few cases were reported in the literature, and most of them occurred in the cervix, extremely rare in the uterine body and ovary. MNAC was rarely reported to arise in the uterine corpus, but never was reported in the ovary. Mesonephric-like adenocarcinomas are recently suggested to describe these neoplasms arising from the uterine corpus and ovary. Due to the rareness of the disease, little is known regarding clinical characteristics, pathological diagnosis, prognosis, and optimal management strategy of MNAC in the female reproductive system. We report a series of MNACs arising from the vagina, cervix, uterine corpus, ovary, and fallopian tube, to summarize the clinical characteristics, pathological diagnosis, treatment, and prognosis.We retrospectively analyzed all MNACs in the female genital tract derived from our institute from January 2010 till January 2020. Patients' clinical details and follow-up were obtained from hospital records and scans were obtained from picture archiving and communication system.A total of 11 patients were included. The median age of onset of symptoms was 52 years. All patients underwent total hysterectomy and bilateral salpingo-oophorectomy, and lymph node dissections were performed in 7/11 (63.6%) patients. Two/eleven (18.2%) received neoadjuvant chemotherapy before surgery and 7/11 (63.6%) received adjuvant chemotherapy after primary surgery. Of the 11 patients, only 1 patient received adjuvant radiation therapy. One patient died at the end point of this study, 9 patients (81.8%) survived and 1 patient was lost to follow-up. The mean follow-up duration was 33.5 months.Although there is no consensus for the optimal treatment of this rare disease, radical surgery is considered to be the initial choice for localized lesion. Given the high malignancy, the majority of MNAC or mesonephric-like adenocarcinoma patients who underwent adjuvant chemotherapy received 4 to 8 cycles of carboplatin/paclitaxel as a first-line treatment after primary surgery with a median progression-free survival of 12 months. Treatment for recurrent disease in these patients included gemcitabine, carboplatin, and paclitaxel. Radiation was very limited in the treatment of the disease.
Topics: Adenocarcinoma; Adult; Aged; China; Female; Genital Neoplasms, Female; Genitalia, Female; Humans; Mesonephroma; Middle Aged; Retrospective Studies
PubMed: 34477176
DOI: 10.1097/MD.0000000000027174 -
Diagnostics (Basel, Switzerland) Nov 2021Mesonephric-like adenocarcinoma (MLA) of the uterine corpus is a rare but distinct malignant tumor of the female genital tract, demonstrating a characteristic morphology...
Mesonephric-like adenocarcinoma (MLA) of the uterine corpus is a rare but distinct malignant tumor of the female genital tract, demonstrating a characteristic morphology and unique immunohistochemical profiles and molecular alterations. We conducted immunohistochemical staining (IHC) to make precise differential diagnoses of uterine MLAs from common histological subtypes of endometrial carcinomas. We collected 25 uterine MLAs and performed IHC for GATA3, TTF1, CD10, ER, PR, p16, p53, and HER2. Seventeen cases (68.0%) showed at least moderate nuclear GATA3 immunoreactivity in ≥25% of tumor cells. Most cases expressed TTF1 (17/21, 81.0%) and CD10 (luminal; 17/21, 81.0%). Heterogeneous TTF1 expression was noted in 12 cases. An inverse pattern of GATA3 and TTF1 staining was observed in eight cases (32.0%). Three cases (12.0%) showed moderate-to-strong ER expression in ≥25% of tumor cells, and two cases (8.0%) showed moderate-to-strong PR expression in ≥5% of tumor cells. These hormone receptor-positive MLAs varied in intensity and proportion of GATA3 staining. None of the 25 cases exhibited either diffuse and strong p16 expression or aberrant p53 expression. Five cases (20.0%) showed equivocal HER2 immunoreactivity (score 2+), but FISH confirmed that none of them exhibited gene amplification. In summary, a small subset of uterine MLAs displayed atypical IHC results: focal but strong expression of ER or PR, the complete absence of GATA3 immunoreactivity, the concurrent expression of mesonephric and hormone receptors, and the inverse pattern of GATA3 and TTF1 staining. These unusual immunophenotypes may complicate the differential diagnosis of MLA. Moreover, pathologists should be encouraged to interpret the IHC results cautiously.
PubMed: 34829389
DOI: 10.3390/diagnostics11112042 -
American Journal of Medical Genetics.... Oct 2021WNT9B plays a key role in the development of the mammalian urogenital system. It is essential for the induction of mesonephric and metanephric tubules, the regulation of...
WNT9B plays a key role in the development of the mammalian urogenital system. It is essential for the induction of mesonephric and metanephric tubules, the regulation of renal tubule morphogenesis, and the regulation of renal progenitor cell expansion and differentiation. To our knowledge, WNT9B has not been associated with renal defects in humans; however, WNT9B mice have renal agenesis/hypoplasia and reproductive tract abnormalities. We report four individuals from two unrelated consanguineous families with bilateral renal agenesis/hypoplasia/dysplasia and homozygous variants in WNT9B. The proband from Family 1 has bilateral renal cystic dysplasia and chronic kidney disease. He has two deceased siblings who presented with bilateral renal hypoplasia/agenesis. The three affected family members were homozygous for a missense variant in WNT9B (NM_003396.2: c.949G>A/p.(Gly317Arg)). The proband from Family 2 has renal hypoplasia/dysplasia, chronic kidney disease, and is homozygous for a nonsense variant in WNT9B (NM_003396.2: c.11dupC/p.(Pro5Alafs*52)). Two of her siblings died in the neonatal period, one confirmed to be in the context of oligohydramnios. The proband's unaffected brother is also homozygous for the nonsense variant in WNT9B, suggesting nonpenetrance. We propose a novel association of WNT9B and renal anomalies in humans. Further study is needed to delineate the contribution of WNT9B to genitourinary anomalies in humans.
Topics: Animals; Child; Congenital Abnormalities; Female; Homozygote; Humans; Infant; Kidney; Kidney Diseases; Kidney Tubules; Male; Mice; Pregnancy; Urinary Tract; Urogenital Abnormalities; Wnt Proteins
PubMed: 34145744
DOI: 10.1002/ajmg.a.62398 -
Journal of Endourology Case Reports 2020A 17-year-old male with Zinner syndrome, a right seminal vesicle cyst, and a solitary left kidney presented with chronic pelvic pain. Previous surgeons had attempted...
A 17-year-old male with Zinner syndrome, a right seminal vesicle cyst, and a solitary left kidney presented with chronic pelvic pain. Previous surgeons had attempted robot-assisted laparoscopic seminal vesicle cyst aspiration and transurethral resection of the ejaculatory duct. Neither surgery provided sustained symptom relief. Abdominal and pelvic MRI showed a cystic structure lodged between the prostate and bladder. The right seminal vesicle, kidney, and ureter were not observed. A robot-assisted laparoscopic seminal vesiculectomy was planned. Dissection distal to the right vas deferens and between the bladder neck and prostate revealed a cystic seminal vesicle-like structure. Attached to this was a tubular structure coursing deep to the vas deferens from the right renal fossa. This was presumed to be a dysplastic ureter. The dysplastic ureter was transected from the seminal vesicle and the seminal vesicle was marsupialized to the deep pelvis. Proximally, the dysplastic ureter was transected and left open. Histologic assessment of the specimen revealed an ∼12.1 cm tubular mesonephric remnant. The postoperative course was uncomplicated. At 6 months follow-up, the patient remains free of symptoms with preserved ejaculatory volume. Mesonephric duct abnormalities and symptoms present on a spectrum. We present a safe and effective resection of a mesonephric duct remnant from a 17-year-old male with Zinner syndrome. A robotic approach localized to the right allowed for excellent observation without compromising left-sided genitourinary anatomy. In males presenting with renal agenesis and pelvic symptoms, clinicians should be suspicious of Zinner syndrome and other mesonephric abnormalities.
PubMed: 33102726
DOI: 10.1089/cren.2020.0020 -
Diagnostics (Basel, Switzerland) May 2021Mesonephric adenocarcinomas are rare tumors of the female genital tract, thought to arise from embryonic mesonephric remnants, primarily in the cervix and vagina....
Mesonephric adenocarcinomas are rare tumors of the female genital tract, thought to arise from embryonic mesonephric remnants, primarily in the cervix and vagina. Conversely, endometrial and ovarian mesonephric adenocarcinomas may have a different pathogenesis, probably originating from transdifferentiated Müllerian carcinomas, as demonstrated by the association of these neoplasms with endometriosis and ovarian serous tumors. For this reason, in the endometrium and in the ovary, they are defined as "mesonephric-like adenocarcinomas". Some cases of mesonephric carcinomas of the female genital tract have been reported to show a sarcomatous component and have been defined as "mesonephric carcinosarcomas", characterized by poor prognosis and high metastatic behavior, but this entity has never been described in the ovary. The case herein presented is of a 74-year-old female with abdominal discomfort and a complex ovarian mass. Histological and immunohistochemical analysis showed features of ovarian mesonephric-like carcinoma combined with a low-grade serous component, in support of the theory of a Müllerian origin of these neoplasms. The tumor also revealed foci of chondrosarcomatous differentiation, never before reported in the ovary, showing a similar immunohistochemical profile to the mesonephric-like elements. This work thus describes the first reported case of ovarian mesonephric-like carcinosarcoma.
PubMed: 34063676
DOI: 10.3390/diagnostics11050827