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Open Medicine (Warsaw, Poland) 2021During embryonic development in women, a regression of temporary embryonic structures - mesonephric (Wolffian) ducts occurs. Adnexal tumors of Wolffian duct origin...
BACKGROUND
During embryonic development in women, a regression of temporary embryonic structures - mesonephric (Wolffian) ducts occurs. Adnexal tumors of Wolffian duct origin (FATWO) are rare.
CASE REPORT
We presented the case of a 64-year-old female patient who was diagnosed with FATWO. After the surgical treatment, the uterus with bilateral adnexal structures was submitted for histopathological analysis. The left ovary was occupied by a tumor measuring 80 × 60 × 50 mm, with smooth, shiny, whitish surface. Tumor cells were medium-sized, relatively uniform, round, and polygonal, with eosinophilic cytoplasm and centrally laid nucleus with fine chromatin, organized into solid, trabecular, and tubular formations. Tumor cells were positive for pancytokeratin (CK), CK7, CD10, neuron-specific enolase (NSE), synaptophysin, calretinin, progesterone, estrogen, and epithelial membrane antigen (EMA).
CONCLUSION
This case adds a report of a rare tumor to the literature. We must think of it in the differential diagnostic algorithm to make an accurate diagnosis for selecting the best treatment modality.
PubMed: 34183995
DOI: 10.1515/med-2021-0306 -
BMJ Case Reports Jan 2021Congenital malformations of the seminal vesicles (SVs) are rare and are associated with abnormalities of the ipsilateral urinary tracts as embryologically both the...
Congenital malformations of the seminal vesicles (SVs) are rare and are associated with abnormalities of the ipsilateral urinary tracts as embryologically both the ureteral buds and SVs arise from the mesonephric ducts. The triad of SV cysts, ipsilateral renal agenesis and ejaculatory duct obstruction is known as the Zinner syndrome. We, herein, present three very rare presentations of Zinner syndrome. Case 1 presented with haematuria, and was found to have a large SV cyst with stones and underwent a robotic cyst excision. Case 2 presented with primary infertility, and was found to have a variant of Zinner syndrome. Case 3 was a known case of chronic kidney disease on maintenance haemodialysis who presented with fever and oliguria. He was found to have Zinner syndrome and underwent aspiration of SV abscess. To the best of our knowledge, such varying presentations of Zinner syndrome have been rarely reported thus far.
Topics: Adult; Congenital Abnormalities; Ejaculatory Ducts; Humans; Kidney; Kidney Diseases; Male; Middle Aged; Seminal Vesicles; Syndrome; Young Adult
PubMed: 33462046
DOI: 10.1136/bcr-2020-239254 -
Journal of the American Society of... Apr 2021Although zebrafish embryos have been used to study ciliogenesis and model polycystic kidney disease (PKD), adult zebrafish remain unexplored.
BACKGROUND
Although zebrafish embryos have been used to study ciliogenesis and model polycystic kidney disease (PKD), adult zebrafish remain unexplored.
METHODS
Transcription activator-like effector nucleases (TALEN) technology was used to generate mutant for , the homolog of the mammalian causative gene for Meckel syndrome type 3 (MKS3). Classic 2D and optical-clearing 3D imaging of an isolated adult zebrafish kidney were used to examine cystic and ciliary phenotypes. A hypomorphic strain or rapamycin was used to inhibit mTOR activity.
RESULTS
Adult zebrafish developed progressive mesonephric cysts that share conserved features of mammalian cystogenesis, including a switch of cyst origin with age and an increase in proliferation of cyst-lining epithelial cells. The mutants had shorter and fewer distal single cilia and greater numbers of multiciliated cells (MCCs). Absence of a single cilium preceded cystogenesis, and expansion of MCCs occurred after pronephric cyst formation and was inversely correlated with the severity of renal cysts in young adult zebrafish, suggesting a primary defect and an adaptive action, respectively. Finally, the mutants exhibited hyperactive mTOR signaling. mTOR inhibition ameliorated renal cysts in both the embryonic and adult zebrafish models; however, it only rescued ciliary abnormalities in the adult mutants.
CONCLUSIONS
Adult zebrafish mutants offer a new vertebrate model for renal cystic diseases, in which cilia morphology can be analyzed at a single-nephron resolution and mTOR inhibition proves to be a candidate therapeutic strategy.
PubMed: 33574160
DOI: 10.1681/ASN.2020070991 -
Diagnostic Pathology Jun 2019Endometrial mesonephric-like adenocarcinomas exhibit classical histologic features of mesonephric carcinoma; however, it remains unclear whether these tumors represent...
BACKGROUND
Endometrial mesonephric-like adenocarcinomas exhibit classical histologic features of mesonephric carcinoma; however, it remains unclear whether these tumors represent mesonephric (Wolffian) carcinoma or endometrioid (Müllerian) carcinomas that closely mimic mesonephric carcinoma.
CASE PRESENTATION
A 32-year-old Japanese primigravida presented with atypical vaginal bleeding. An endometrial biopsy suggested low-grade endometrioid carcinoma, and she was administered medroxyprogesterone acetate. Her tumor recurred 6 years later, and she underwent hysterectomy, salpingo-oophorectomy, and omentectomy, at which point she was diagnosed with mesonephric-like adenocarcinoma of the uterine endometrium. Retrospective pathological review of the initial biopsy confirmed coexisting low-grade endometrioid carcinoma and mesonephric-like adenocarcinoma of the uterine endometrium. On immunohistochemistry, the endometrioid carcinoma component was diffuse positive for estrogen and progesterone receptors but negative for thyroid transcription factor 1. However, the mesonephric-like adenocarcinoma component exhibited a mixture of estrogen receptor- and thyroid transcription factor 1-positive cells within the same glands.
CONCLUSIONS
We encountered a patient with coexisting endometrial mesonephric-like adenocarcinoma and low-grade endometrioid carcinoma, which was treated using medroxyprogesterone acetate therapy, resulting in recurrence of mesonephric-like adenocarcinoma alone. These clinicopathological findings support the prevailing notions that mesonephric-like adenocarcinoma is a Müllerian adenocarcinoma exhibiting mesonephric differentiation.
Topics: Adenocarcinoma; Adult; Antineoplastic Agents, Hormonal; Biomarkers, Tumor; Carcinoma, Endometrioid; Cell Differentiation; Endometrial Neoplasms; Endometrium; Female; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Medroxyprogesterone Acetate; Mullerian Ducts; Retrospective Studies; Wolffian Ducts
PubMed: 31174566
DOI: 10.1186/s13000-019-0830-4 -
Oncology Letters Nov 2017Female Wolffian adnexal tumor (WAT) is a rare neoplasm arising from the remnants of the mesonephric duct and <100 cases have been reported globally. The present case...
Female Wolffian adnexal tumor (WAT) is a rare neoplasm arising from the remnants of the mesonephric duct and <100 cases have been reported globally. The present case report describes a 73-year-old female patient with WAT in the left ovary which, to the best of our knowledge, is the largest benign WAT tumor to be reported. In addition, the present case report reviewed previous studies on the clinical characteristics and therapy for WAT and the surgery methods for female WAT of ovary were summarized. WATs are typically benign; however, a number factors may increase the risk of malignancy.
PubMed: 29142599
DOI: 10.3892/ol.2017.6859 -
World Journal of Clinical Cases Jun 2021Mesonephric adenocarcinoma (MNAC) is an extremely rare malignancy in the female genital tract. Only a few cases have been reported in the literature, and most of them...
BACKGROUND
Mesonephric adenocarcinoma (MNAC) is an extremely rare malignancy in the female genital tract. Only a few cases have been reported in the literature, and most of them occurred in the cervix, with extremely rare cases in the uterine body and ovary. MNAC has never been reported to arise in the fallopian tube.
CASE SUMMARY
A 45-year-old woman was referred to our institution with a history of abdominal pain. Ultrasound revealed a cystic and solid mass in left adnexal region. The patient underwent complete staging surgery when intraoperative pathological examination demonstrated that the mass was malignant. The final histological and immunohistochemical results confirmed the diagnosis of MNAC originating from the fallopian tube. Then she received four cycles of combination chemotherapy with carboplatin plus paclitaxel. The tumor recurred with hepatic metastases 4 mo after initial surgery, and second resection of the tumors in the liver plus partial hepatectomy was performed. She was supplemented with five courses of a new combination chemotherapy with gemcitabine plus carboplatin, and there was no evidence of recurrence within the 22-mo follow-up period after the second surgery.
CONCLUSION
MNAC originating from the fallopian tube is an extremely rare and high malignancy with a poor prognosis. It can be very aggressive, even at early stage. Little is known about the clinical characteristics, pathological diagnosis, prognosis, and optimal management strategy of MNAC originating from the fallopian tube. Herein we report the first case of primary MNAC deriving from the fallopian tube.
PubMed: 34222441
DOI: 10.12998/wjcc.v9.i18.4741 -
International Journal of Gynecological... May 2024Immunohistochemical markers shown to be useful in identifying/confirming mesonephric/mesonephric-like differentiation (MLD markers) include thyroid transcription factor...
Immunohistochemical markers shown to be useful in identifying/confirming mesonephric/mesonephric-like differentiation (MLD markers) include thyroid transcription factor (TTF1), GATA-binding protein 3 (GATA3), and cluster of differentiation 10 (CD10). Only a few studies have examined the expression levels of MLD markers in endometrial endometrioid carcinomas (EECs). This study aimed to analyze the frequency and pattern of MLD marker expression in low-grade EECs. We performed immunostaining for the detection of TTF1, GATA3, and CD10 expression in 50 low-grade EEC tissue samples and evaluated their staining proportion and intensity. Nine tumors (18.0%) expressed at least one MLD marker in varying proportions and intensities, and 2 of these tumors were positive for 2 MLD markers (TTF1/GATA3 and GATA3/CD10, respectively). Three (6.0%) tumors showed moderate-to-strong nuclear TTF1 immunoreactivity in ≤5% of the tumor cells. Five tumors (10.0%) had at least moderate nuclear GATA3 staining, and three of them displayed a staining proportion of ≥15%. Three tumors (6.0%) were focal (mean proportion, 15%) but strongly positive for CD10. Our findings indicate that a subset of EEC can express one or more MLD markers with varying staining proportions and intensities. Given that a diagnosis of uterine mesonephric-like adenocarcinoma should be established based on a combination of characteristic histologic features, unique immunophenotypes, and confirmed molecular findings, pathologists should not exclude EEC based only on the presence of focal immunoreactivity for MLD markers. Awareness of the atypical expression patterns of MLD markers in EEC helps pathologists avoid misdiagnosing EEC as a uterine mesonephric-like adenocarcinoma.
Topics: Female; Humans; Carcinoma, Endometrioid; Mesonephros; Uterus; Adenocarcinoma; Biomarkers, Tumor; Endometrial Neoplasms
PubMed: 37566876
DOI: 10.1097/PGP.0000000000000976 -
Cureus Sep 2022Zinner syndrome is a rare congenital triad of mesonephric duct abnormality encompassing unilateral renal agenesis or dysgenesis, ipsilateral seminal vesicle cyst, and...
Zinner syndrome is a rare congenital triad of mesonephric duct abnormality encompassing unilateral renal agenesis or dysgenesis, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction. Literature has reported 214 cases, with the most common presentation being lower urinary tract symptoms and abdominal pain. Most cases are incidentally diagnosed, and MRI has been the choice of radiological diagnosis. We report the case of an 81-year-old male who presented with a three-month history of a fungating elbow lesion, elbow pain, and weight loss. Imaging revealed an ipsilateral seminal vesicle cyst, absent kidney, and ejaculatory duct obstruction, i.e., Zinner syndrome with bone metastasis. A bone biopsy revealed a urothelial primary, and cyst aspiration and cytology revealed spermatozoa and malignant cells representing an adenocarcinoma. This patient was managed with symptom control, radiotherapy to the elbow, and palliative chemotherapy, but later succumbed to the condition.
PubMed: 36237803
DOI: 10.7759/cureus.28949 -
Gynecologic Oncology Reports Aug 2016A mesonephric adenocarcinoma of the cervix is a very rare tumor deriving from remnants of the mesonephric duct. Differential diagnosis from other cervical carcinomas is...
A mesonephric adenocarcinoma of the cervix is a very rare tumor deriving from remnants of the mesonephric duct. Differential diagnosis from other cervical carcinomas is difficult and little is known regarding its biological behavior, prognosis, and the optimal management strategy. We present a case of a mesonephric adenocarcinoma of the cervix with a comprehensive review of the existing literature. In this case a 66-year-old woman presented with postmenopausal vaginal bleeding. She was diagnosed with a FIGO stage IIB mesonephric adenocarcinoma of the cervix and treated with neoadjuvant chemoradiotherapy and a Wertheim hysterectomy. The recovery from surgery was uneventful and the patient remains with no evidence of disease with 2 years of follow-up.
PubMed: 27354991
DOI: 10.1016/j.gore.2016.05.002 -
Indian Journal of Pathology &... Jun 2024Clear cell adenocarcinoma (CCAC) of cervix is a rare subtype of endocervical adenocarcinoma that accounts for 4% of all cervical adenocarcinoma with many morphological...
Clear cell adenocarcinoma (CCAC) of cervix is a rare subtype of endocervical adenocarcinoma that accounts for 4% of all cervical adenocarcinoma with many morphological mimickers. Retrospectively study cases of cervical clear cell adenocarcinoma of the cervix. Clinical profile and pathological features of CCAC of the cervix diagnosed between 2018-2022 were retrospectively analyzed.The database of the Department of Pathology of our institute was systematically searched for patients diagnosed with clear cell adenocarcinoma of the cervix during 2018-2022.A total of 19 patients were studied with the mean age of patients being 53.72 years (range 25 -84 yrs,standard deviation-25.9) and median tumor size being 5.6cm. Lymph node metastasis was identified in 33.3% and distant metastasis were seen in 20% of the cases. Staging could not be done in 4 cases.FIGO staging of the cases included IB1(2 cases), IB2(2 cases), IIB (3 cases),IIIA (1 case)IIIB(4 cases),and IV(3 cases). On histopathological evaluation, heterogeneous architectural pattern comprising of tubulocystic, solid, and papillary patterns were seen in 13 cases (13/19,68.4%). Pure tubulocystic (3/19,15.7%), pure papillary (2/19,10.5%), and pure solid patterns (1/19,5.3%) were also identified. Tumor cells with clear cytoplasm ranged from 5% to 95%. Nuclear atypia was moderate to marked in all the cases (19/19,100%). Mitotic activity varied from 1/10hpf to 20-22/10hpf. By immunohistochemistry, tumor was positive for Napsin A in all the cases,p16INK4a was negative in majority of cases (15/19,78.9%) and ER was negative in 14 cases (14/19,73.7%) .p53 showed wild type staining except for one case . Clear cell adenocarcinoma being a rare subtype of cervical adenocarcinoma, needs to be differentiated from other Human Papilloma Virus(HPV) independent adenocarcinomas (gastric and mesonephric types) and benign entities such as endocervical glandular Arias-Stella reaction. Judicious use of a panel of immunostains is often helpful.
PubMed: 38847204
DOI: 10.4103/ijpm.ijpm_518_23