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Current Medical Imaging 2022Zinner syndrome is a rare congenital abnormality defined by a clinical triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory...
INTRODUCTION
Zinner syndrome is a rare congenital abnormality defined by a clinical triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction.
CASE PRESENTATION
Most patients are asymptomatic, but if the cystic dilatation of the seminal vesicle becomes significant, it can result in urinary symptoms such as dysuria and urinary retention. This rare developmental anomaly related to mesonephric duct can also present with other abnormalities.
CONCLUSION
Here, we report our experience of Zinner syndrome with bladder outlet obstruction and an ectopic ureter remnant.
Topics: Cysts; Genital Diseases, Male; Humans; Kidney; Male; Seminal Vesicles; Syndrome; Ureter
PubMed: 34102980
DOI: 10.2174/1573405617666210608151618 -
Journal of Cancer Research and... 2022Mesonephric carcinoma is a rare type of carcinoma seen in the female genital tract. It arises from the mesonephric remnants situated in the broad ligament, lateral wall... (Review)
Review
Mesonephric carcinoma is a rare type of carcinoma seen in the female genital tract. It arises from the mesonephric remnants situated in the broad ligament, lateral wall of the cervix, vagina, and uterine corpus. Very few cases of mesonephric carcinoma have been reported so far in the literature. The sites mentioned in various literatures include the cervix, vagina, or uterus, but we could not find any literature that mentions posthysterectomy vault as a site for mesonephric carcinoma. Here, we report a case of 40-years-old hysterectomised female who presented in the hospital with nodular growth on the vault and complaints of bleeding per vaginum. Microscopy of the lesion did not show typical morphology of mesonephric carcinoma, but immunohistochemistry played a vital role in the diagnosis of this rare tumor.
Topics: Adenocarcinoma; Adult; Carcinoma; Cervix Uteri; Female; Humans; Hysterectomy; Immunohistochemistry; Uterine Cervical Neoplasms
PubMed: 35381800
DOI: 10.4103/jcrt.JCRT_168_19 -
Applied Immunohistochemistry &... Sep 2020Mesonephric carcinoma is a rare gynecologic neoplasm commonly mistaken for clear cell carcinoma, because of their overlapping morphologic features. Both tumors are...
Mesonephric carcinoma is a rare gynecologic neoplasm commonly mistaken for clear cell carcinoma, because of their overlapping morphologic features. Both tumors are negative for estrogen receptor and p16, magnifying this diagnostic dilemma. Recently, hepatocyte nuclear factor-1 beta (HNF-1β), a marker for clear cell carcinoma, has also been shown to be positive in mesonephric carcinomas. Other more recent markers for clear cell carcinoma, however, such as Napsin-A and alpha-methylacyl-CoA racemase (AMACR), have not yet been studied in mesonephric carcinomas. Here we examine HNF-1β, AMACR, and Napsin-A immunohistochemistry in 18 mesonephric and 55 endometrial/cervical clear cell carcinomas. HNF-1β was considered positive if nuclear staining was present in ≥70% of cells and at least moderate intensity; for Napsin-A and AMACR, any cytoplasmic staining was considered positive (≥1%). H-scores were determined by multiplying the intensity score by proportion score. HNF-1β was positive in a substantial portion of mesonephric carcinomas (9/18, 50%; H-score 98) and clear cell carcinomas (34/55, 62%; H-score 163) and did not distinguish between the 2 entities (specificity, 50%; P-value of H-score=0.08). Napsin-A and AMACR expression was significantly higher in clear cell [43/55 (78%) and 41/55 (75%), respectively] than mesonephric carcinomas [4/18 (22%) and 4/18 (22%) respectively], and helpful in this differential (specificity: 78% and 78%; P<0.05 for both). When Napsin-A and AMACR staining were seen in mesonephric carcinomas, staining was focal (≤5%), whereas staining in clear cell carcinomas was patchy/diffuse. In summary, Napsin-A and AMACR are helpful in distinguishing mesonephric carcinomas from clear cell carcinomas of the female genital tract, but HNF-1β is not.
Topics: Adenocarcinoma, Clear Cell; Adult; Aged; Aspartic Acid Endopeptidases; Cohort Studies; Endometrial Neoplasms; Female; Hepatocyte Nuclear Factor 1-beta; Humans; Immunohistochemistry; Middle Aged; Neoplasm Staging; Racemases and Epimerases; Tissue Array Analysis; Uterine Cervical Neoplasms
PubMed: 31361605
DOI: 10.1097/PAI.0000000000000801 -
International Journal of Gynecological... Mar 2023Ovarian combined serous borderline tumor/low-grade serous carcinomas (SBT/LGSC) and mesonephric-like adenocarcinomas (MLA) have been previously reported and the presence...
Ovarian combined serous borderline tumor/low-grade serous carcinomas (SBT/LGSC) and mesonephric-like adenocarcinomas (MLA) have been previously reported and the presence of identical oncogenic somatic mutations in both components supports the concept that at least some of MLAs arise from a Müllerian origin. We report 2 cases of ovarian combined SBT/LGSC and mesonephric-like lesion. Case 1 was a 70-yr-old woman presented with a liver lesion and omental carcinomatosis. Histologic examination revealed biphasic tumors in bilateral ovaries consisting of conventional SBT and invasive MLA with extraovarian spread. The right ovary also had a component of cribriform variant of SBT/noninvasive LGSC. The SBT/LGSC component was diffusely positive for Pax8, WT-1, and ER, focally positive for PR, and negative for GATA3, while the MLA component was diffusely positive for GATA3 but negative for WT-1, ER, and PR. Molecular analysis revealed a KRAS G12V mutation in both the SBT/LGSC and MLA components, indicating their clonal origin. Case 2 was a 58-yr-old woman who presented with conventional type SBT in both ovaries. In addition, the left ovarian tumor demonstrated a few areas (each <5 mm) of mesonephric-like differentiation/hyperplasia in close proximity to the serous-type epithelium, with an immunophenotype of focal GATA3 expression, luminal pattern of CD10 staining and negative WT-1, ER, and PR staining. This phenomenon has been reported in endometrioid borderline tumor but not in any serous type lesions. The findings in case 1 provide further evidence to demonstrate the clonal relationship between these morphologically and immunophenotypically distinct components. It also supports the theory that, unlike cervical mesonephric carcinomas originating from mesonephric remnants, MLAs are derived from a Müllerian-type lesion with differentiation into mesonephric lineage. The presence of a hyperplastic mesonephric-like lesion/differentiation in case 2 indicates that a precursor lesion in the same lineage with the potential to develop into MLA exists in the ovary.
Topics: Female; Humans; Ovarian Neoplasms; Carcinoma; Mesonephros; Epithelium; Hyperplasia; Cystadenocarcinoma, Serous
PubMed: 35348533
DOI: 10.1097/PGP.0000000000000868 -
International Journal of Gynecological... Mar 2018Literature published between 1975 and 2015 was systematically reviewed to conduct a case-comparator study of tissue based, immunohistochemical biomarker expression among... (Review)
Review
Literature published between 1975 and 2015 was systematically reviewed to conduct a case-comparator study of tissue based, immunohistochemical biomarker expression among malignant glandular histotypes of the uterine cervix so as to identify differences that could have diagnostic utility. Of the 902 abstracts, 154 articles had a full review, and 52 were included. Biomarker positivity in cases of adenocarcinoma in situ (AIS) were compared with atypical lobular endocervical glandular hyperplasia and invasive histotypes grouped as mucinous, endometrioid, adenosquamous, serous clear cell, minimal deviation-gastric type, and mesonephric carcinomas (7 AIS case-comparators). The invasive histotypes were compared with each other (30 adenocarcinoma case-comparators). Biomarker positivity in all 37 case-comparators was calculated as weighted averages of histotype-specific estimates. Unsupervised hierarchical clustering examined differences in expression and were visualized via heatmaps and dendrograms. Of the 56 biomarkers tested, 1 or more of 15 showed a 50% or more difference in positive expression in 6 (86%) of the AIS and 21 (70%) of the adenocarcinoma case-comparators. There was no data on the comparison of serous clear cell to mesonephric carcinoma. AIS case-comparator biomarkers were HIK1083, alpha SMA, PAX8, VIL1, CEA, p53, p16, and CD10, and only alpha SMA had a difference of 100%. The adenocarcinoma case-comparator biomarkers were CEA, p53, Claudin18, HIK1083, p16, Calretinin, CD10, PR, Chromogranin, MUC6, Vimentin and p63, and none had a difference of 100%. Biomarker expression in the discrimination of AIS from invasive adenocarcinoma, and the invasive histotypes from each other is understudied. One or more of 15 biomarkers could have diagnostic utility.
Topics: Adenocarcinoma in Situ; Biomarkers; Cervix Uteri; Female; Humans; Immunohistochemistry; Neoplasms, Glandular and Epithelial; Uterine Cervical Neoplasms
PubMed: 28582347
DOI: 10.1097/PGP.0000000000000406 -
Anticancer Research Sep 2022The updated 2020 World Health Organization (WHO) classification divides endocervical adenocarcinomas (EACs) into human papillomavirus-associated (HPVA) and -independent... (Review)
Review
Endocervical Adenocarcinoma: Comprehensive Histological Review and Re-classification of 123 Consecutive Cases According to the Updated World Health Organization Classification of Female Genital Tumors.
BACKGROUND/AIM
The updated 2020 World Health Organization (WHO) classification divides endocervical adenocarcinomas (EACs) into human papillomavirus-associated (HPVA) and -independent (HPVI) tumors. The purpose of this study was to review our EAC cases and re-classify them according to the updated WHO classification.
PATIENTS AND METHODS
We reviewed the hematoxylin and eosin-stained slides of 123 EACs and reclassified them according to the updated WHO classification.
RESULTS
Eighty-one (65.9%) and 42 (34.1%) patients had HPVA and HPVI EACs, respectively. The usual (60/81; 74.1%) and gastric (31/42; 73.8%) types were the most common HPVA and HPVI EACs, respectively. Signet-ring cell (1/123; 0.8%), invasive stratified mucin-producing (10/123; 8.1%), clear-cell (4/123; 3.3%), mesonephric (3/123; 2.4%), and serous (1/123; 0.8%) types were uncommon. Unusual morphologies were seen, including microcystic, elongated, and fragmented patterns of stromal invasion, micropapillary growth patterns, and gastric-type adenocarcinoma in situ.
CONCLUSION
We successfully reclassified all the examined cases based on morphology alone. The numbers and relative proportions of EAC histotypes were variable. We found some uncommon histotypes, as well as unusual but clinically important histological features.
Topics: Adenocarcinoma; Alphapapillomavirus; Carcinoma; Female; Humans; Papillomaviridae; Papillomavirus Infections; Uterine Cervical Neoplasms; World Health Organization
PubMed: 36039458
DOI: 10.21873/anticanres.15967 -
Journal of Cancer Research and Clinical... Nov 2023Mesonephric-like adenocarcinomas (MLA) of the female genital tract represent a rare and relatively recently described neoplasm exhibiting characteristic morphologic and... (Review)
Review
Mesonephric-like adenocarcinoma of the female genital tract: possible role of KRAS-targeted treatment-detailed molecular analysis of a case series and review of the literature for targetable somatic KRAS-mutations.
PURPOSE
Mesonephric-like adenocarcinomas (MLA) of the female genital tract represent a rare and relatively recently described neoplasm exhibiting characteristic morphologic and immunohistochemical findings commonly associated with a KRAS-mutation. Most cases display an aggressive clinical behavior, but knowledge about treatment approaches is limited, especially for targeting KRAS.
METHODS
We report a series of eight cases with a detailed molecular analysis for KRAS. These cases as well as the data of previously published cases with detailed information regarding KRAS-mutational events were reviewed for a potential targeted approach and its prognostic impact.
RESULTS
Both the uterine and ovarian MLA harbor a somatic KRAS-mutation in about 85% of the reported cases, affecting the hotspot codons 12 and 13. 15.7% of the endometrial and 15.6% of ovarian MLA are wild type for KRAS. A p.G12A-alteration was seen in 5.6% (5/89) of the endometrial and in 6.2% (2/32) of the ovarian tumors, for p.G12C in 7.9% and 6.2%, for p.G12D in 32.6% and 34.5% and for p.G12V in 36% and 37.5%, respectively. Very limited data are available regarding the prognostic impact of different mutational sites within the KRAS-gene without significant prognostic impact.
CONCLUSION
Because of a specific p.G12C-KRAS somatic mutation, only the minority of MLA (7.9% with uterine and 6.2% with ovarian primary) are potentially targetable by sotarasib in that rare but aggressive subtype of adenocarcinoma of the female genital tract. Until now, the different location of a somatic KRAS-mutation is of no prognostic impact.
Topics: Humans; Female; Proto-Oncogene Proteins p21(ras); Adenocarcinoma; Mutation; Prognosis; Genitalia, Female
PubMed: 37668797
DOI: 10.1007/s00432-023-05306-9 -
Abdominal Radiology (New York) Jun 2024Zinner syndrome is a rare congenital urological entity, secondary to an alteration in embryogenesis between 4th and 13th weeks of gestation, specifically because of... (Review)
Review
Zinner syndrome is a rare congenital urological entity, secondary to an alteration in embryogenesis between 4th and 13th weeks of gestation, specifically because of abnormalities in the development of the distal mesonephric duct. It is characterized by the triad of unilateral renal agenesis, cystic dilatation of the ipsilateral seminal vesicle and ipsilateral ejaculatory duct obstruction. The aim of this article is to provide the reader with all the necessary information to be able to suspect the presence of this syndrome, reviewing its physiopathology, clinical manifestations and the imaging techniques that enable its diagnosis, emphasizing those radiological findings by MRI that should lead us to think about it. This work is illustrated with representative radiological images of cases belonging to our institution, including patients with different variants of Zinner syndrome. We also include an overview of the embryology of the male urogenital system, to remember the role of the mesonephric duct and the ureteral bud in the formation of the different urogenital structures, as well as a differential diagnosis that allows us to differentiate seminal vesicle cysts from other pelvic cystic lesions.
PubMed: 38900322
DOI: 10.1007/s00261-024-04430-5 -
International Journal of Gynecological... Mar 2022Endometrial mesonephric-like carcinoma (ML-CA) is a recently recognized subtype of aggressive endometrial adenocarcinoma that is morphologically and immunophenotypically...
Endometrial mesonephric-like carcinoma (ML-CA) is a recently recognized subtype of aggressive endometrial adenocarcinoma that is morphologically and immunophenotypically similar to mesonephric carcinoma but not typically associated with mesonephric remnants. Here, we report a case of 58-yr-old female who had a past medical history of fibroids and of irregular menstrual bleeding for ~20 yr who presented with visual disturbance. On further investigation, she was found to have a large choroidal peri-papillary tumor of the right eye. A presumptive diagnosis of choroidal melanoma was made. Right eye enucleation was performed, and microscopy revealed moderately differentiated metastatic adenocarcinoma. Further work up was advised. A uterine mass was identified on imaging followed by endometrial biopsy that showed a morphologically and immunohistochemically similar tumor to that in the eye. A hysterectomy was carried out and a malignant neoplasm with varying morphologic patterns including gland formation, solid sheets of tumor cells, cribriform, glomeruloid, spindled and papillary areas was seen. The immunohistochemical profile showed diffuse strong positivity for AE1/AE3, TTF1, P16, and vimentin. CD56, GATA3, Napsin A, and CD10 were focally positive. The neoplastic cells were negative for the following markers ER, PR, WT1, calretinin, and synaptophysin. PDL-1 was negative and mismatch repair protein was proficient. An identical KRAS mutation was detected in both the uterine corpus and ocular tumors. The findings are in keeping with a uterine mesonephric-like adenocarcinoma with an ocular metastasis. An Oncomine Focus-Mutation profile, Thermo-Fisher Scientific Inc., a 60 gene oncologic panel, performed on the ocular tumor, revealed no further mutations.
Topics: Adenocarcinoma; Biomarkers, Tumor; Endometrial Neoplasms; Female; Humans; Mesonephros; Uterine Neoplasms
PubMed: 33935158
DOI: 10.1097/PGP.0000000000000781 -
European Journal of Gynaecological... 2016Malignant mesonephric tumor (MMT) is a relatively uncommon malignancy of the female genital tract. The diagnosis of metastatic MMT is difficult because cytological,... (Review)
Review
Malignant mesonephric tumor (MMT) is a relatively uncommon malignancy of the female genital tract. The diagnosis of metastatic MMT is difficult because cytological, pathological, immunohistochemical characteristics of MMT are under-recognized. The authors present a 55-year-old female with metastatic pulmonary nodules. The bronchial washing cytology revealed three dimensional clusters of bland epithelial cells with slight nuclear grooves. A corresponding lung histology had ductal or tubular clusters of epithelial cells with intraglandular eosinophilic materials. These epithelial cells were positive for immunohistochemical stain of CD10, suggesting metastasis from MMT. The cervical smear showed clusters of bland, gland-forming epithelial cells with intraglandular eosinophilic materials. On histologic examination, mesonephric adenocarcinoma with papillary and solid proliferation was identified in the uterine cervix. A review of the literature for 72 cases of MMT is also included. Clinical and cytopathological features of MMT are herein made available.
Topics: Biopsy; Bronchoalveolar Lavage Fluid; Female; Humans; Lung Neoplasms; Mesonephroma; Middle Aged; Multimodal Imaging; Multiple Pulmonary Nodules; Positron-Emission Tomography; Tomography, X-Ray Computed; Uterine Cervical Neoplasms
PubMed: 27172762
DOI: No ID Found