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European Journal of Case Reports in... 2021Zinner syndrome is a developmental anomaly of the urogenital tract. This condition is defined by the triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst...
UNLABELLED
Zinner syndrome is a developmental anomaly of the urogenital tract. This condition is defined by the triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ipsilateral ejaculatory duct obstruction. The syndrome is due to malformation of the mesonephric duct during embryogenesis. The condition used to be rare but is now frequently encountered due to the advent of MRI and CT. MRI confirms the diagnosis by revealing the seminal vesicle cyst and its contents, and the ejaculatory duct obstruction, while CT confirms renal agenesis. We report the case of a young patient with Zinner syndrome.
LEARNING POINTS
Zinner syndrome consists of the triad of renal agenesis, seminal vesicle cyst and ejaculatory duct obstruction.Any insult during embryogenesis of the mesonephric duct in men can result in Zinner syndrome.Pelvic MRI is the gold standard to confirm the diagnosis of Zinner syndrome.
PubMed: 34268266
DOI: 10.12890/2021_002628 -
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 -
Journal of Mid-life Health 2021Congenital malformations of the seminal vesicles (SVs) are rare and are often associated with abnormalities of the ipsilateral upper tracts as embryologically both the...
Congenital malformations of the seminal vesicles (SVs) are rare and are often associated with abnormalities of the ipsilateral upper 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 the case of Zinner syndrome with hematuria as the mode of presentation. A 52-year-old gentleman presented with complaints of gross and painless hematuria for 3 months. An initial ultrasound revealed absent right kidney with a cystic structure abutting the urinary bladder. Cystoscopic examination revealed a high bladder neck. Magnetic resonance imaging of the abdomen revealed an absent right kidney and a large tubular structure in the region of the right ureter extending up to right SV with blood content and stones within. It was distally seen communicating with one of the cysts of the right SV. The cystic structure was removed with a robot-assisted laparoscopic approach. The console time was 110 min with minimal blood loss. Postoperative course was uneventful. Histopathology of the cyst wall revealed chronic inflammation. The patient is doing well on 6 months follow-up. This case was unique in terms of it presenting with a large intra-abdominal cyst with sharp stones within, probably first of its kind to be ever reported. Surgery is mandated for such symptomatic cysts and the daVinci robot with its minimally invasive approach offers the perfect platform for treating such challenging cases.
PubMed: 34188430
DOI: 10.4103/jmh.JMH_49_20 -
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 -
Archives of Pathology & Laboratory... Jan 2022Female adnexal tumor of probable Wolffian origin (FATWO) often is a diagnostic challenge given its rarity, histologic heterogeneity, and lack of specific immunoprofile.
CONTEXT.—
Female adnexal tumor of probable Wolffian origin (FATWO) often is a diagnostic challenge given its rarity, histologic heterogeneity, and lack of specific immunoprofile.
OBJECTIVE.—
To further understand the clinicopathologic and immunohistochemical features of this rare entity.
DESIGN.—
We studied the clinical, morphologic, and immunohistochemical features of a cohort of 11 FATWO cases from our institute.
RESULTS.—
Patients' age ranged from 25 to 76 years (mean, 55 years). Tumor size ranged from 0.5 to 18 cm (mean, 2.7 cm). Histopathologically, most tumors presented with low-grade cytologic features with low mitotic activity and lack of necrosis. Three main growth patterns were appreciated: solid, tubular, and sievelike patterns. Higher-grade nuclear atypia, increased mitotic activity, and focal necrosis were seen in 2 cases. These 2 cases were clinically considered malignant FATWO mainly because of their extra-adnexal involvement. Immunohistochemical studies found that tumor cells were positive for CD10 (11 of 11, 100%), AE1/3 (8 of 8, 100%), CAM 5.2 (4 of 5, 80%), and cytokeratin 7 (CK7; 7 of 10, 70%), and focally positive for calretinin (4 of 10, 40%), inhibin (4 of 10, 40%), epithelial membrane antigen (EMA; 3 of 9, 33%), and steroidogenic factor-1 (SF-1; 2 of 8, 25%). Lack of immunoreactivity to PAX8 and GATA3 in almost all cases indicates that FATWO is different from the tumors derived from the Müllerian or mesonephric origins. All patients with available follow-up had favorable prognosis.
CONCLUSION.—
The broad spectrum of clinical presentation, various morphologic features, and overlapping immunophenotype suggest that FATWO is a diagnosis of exclusion until it is further defined at the molecular and immunohistochemical levels.
Topics: Adenoma; Adnexal Diseases; Biomarkers, Tumor; Female; Humans; Neoplasms, Adnexal and Skin Appendage; Prognosis
PubMed: 34133728
DOI: 10.5858/arpa.2020-0432-OA -
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 -
Iranian Journal of Pathology 2021Mesonephric adenocarcinoma of the uterine cervix is an extremely rare tumor of the female genital tract which derives from the remnants of embryonic mesonephric ducts...
Mesonephric adenocarcinoma of the uterine cervix is an extremely rare tumor of the female genital tract which derives from the remnants of embryonic mesonephric ducts and its prognosis, diagnosis and treatment is rather challenging. We report a case of a 46-year-old woman with history of abnormal uterine bleeding and an enlarged uterine cervix on physical examination without obvious mass lesion. She was clinically underdiagnosed with cervical myoma and mesonephric hyperplasia. After simple hysterectomy, stage IB2 mesonephric adenocarcinoma was diagnosed. Despite adjuvant chemoradiation, she presented with peritoneal and locoregional recurrence in less than a year. So, in the presence of abnormal bleeding and cervical mass, mesonephric hyperplasia in cervical biopsy specimen should be suspected for adenocarcinoma. Radical hysterectomy and complete staging with or without salpingo-oophorectomy is the mainstay of treatment. Despite all ambiguities, due to the small number of reported cases, the overall prognosis seems to be less favorable than conventional cervical adenocarcinoma.
PubMed: 33936235
DOI: 10.30699/IJP.2020.125459.2375 -
Cells Apr 2021Mixed epithelial and stromal tumor of the kidney (MESTK), a benign rare tumor with malignant transformation potential, is thought to be derived from fetal or immature...
Mixed epithelial and stromal tumor of the kidney (MESTK), a benign rare tumor with malignant transformation potential, is thought to be derived from fetal or immature cells originating from the mesonephric and Müllerian ducts. However, due to its rarity, little is known about the anti-tumor immune responses in MESTK. Herein, we present five cases of MESTK and evaluate the population of tumor-infiltrating lymphocytes (TILs) using a freshly obtained MESTK sample. Microscopically, TILs were scattered or clustered in large aggregates in the stroma in all five cases; furthermore, three cases exhibited heavy, large lymphocytic aggregates with no well-organized tertiary lymphoid structures with germinal centers. Flow cytometric analysis of TILs in one freshly obtained MESTK sample revealed that >40% of CD3 T cells were effector memory FasCD28 γδ T cells expressing high levels of programmed cell death protein 1 and inducible T-cell co-stimulator, but low levels of CD44 and CD27. Most αß T cells exhibited a naïve phenotype. Additionally, we detected many activated class-switched CD21CD27 B cells as well as CD11cIgM marginal zone B-like and CD27CD21CD23 immunoglobulin (Ig)DIgM age-associated B-like cells. Collectively, for the first time, we report the immune microenvironment pattern of MESTK to oncogenic stress.
Topics: Adult; Epithelial Cells; Female; Humans; Kidney Neoplasms; Lymphocytes, Tumor-Infiltrating; Male; Middle Aged; Stromal Cells; Tomography, X-Ray Computed
PubMed: 33923583
DOI: 10.3390/cells10040917 -
Diagnostics (Basel, Switzerland) Apr 2021We present herein a rare case of uterine serous carcinoma with mesonephric-like differentiation (SC-MLD) initially misdiagnosed as mesonephric-like adenocarcinoma (MLA)....
Serous Carcinoma of the Endometrium with Mesonephric-Like Differentiation Initially Misdiagnosed as Uterine Mesonephric-Like Adenocarcinoma: A Case Report with Emphasis on the Immunostaining and the Identification of Splice Site Mutation.
We present herein a rare case of uterine serous carcinoma with mesonephric-like differentiation (SC-MLD) initially misdiagnosed as mesonephric-like adenocarcinoma (MLA). A 51-year-old woman underwent total hysterectomy for a uterine tumor. Histologically, the tumor exhibited various architectures, including papillary, glandular, tubular, cribriform, and cystic. On the basis of this architectural diversity accompanied by intraluminal eosinophilic secretions and intermediate-grade nuclear atypia, the initial diagnosis was MLA. However, the tumor was diffusely and strongly positive for the expression of p16 and negative for the expression of GATA-binding protein 3 (GATA3). Furthermore, we identified a pathogenic tumor protein 53 () mutation affecting an acceptor splice site in intron 9, despite a wild-type p53 immunostaining pattern. The observations of diffuse and strong p16 expression, lack of GATA3 expression, pathogenic mutation, and wild-type Kirsten rat sarcoma viral oncogene homolog indicate that this tumor was not an MLA but an SC-MLD. Both uterine SC and MLA can exhibit various histological growth patterns. Our comprehensive clinicopathological and molecular analyses can serve to improve the understanding of this rare condition and help pathologists in making an accurate diagnosis.
PubMed: 33919505
DOI: 10.3390/diagnostics11040717 -
Medicine Apr 2021Female adnexal tumors of probable Wolffian origin (FATWOs) are rare gynecologic neoplasms arising from the mesonephric duct remnants. Less than 90 cases have been... (Review)
Review
RATIONALE
Female adnexal tumors of probable Wolffian origin (FATWOs) are rare gynecologic neoplasms arising from the mesonephric duct remnants. Less than 90 cases have been reported in the English literature. Although most cases of FATWO are considered benign, recurrence and metastasis may occur in very few cases during the course of the disease. Due to the small number of recurrent and metastatic FATWO cases, there are no clear recommendations regarding optimal treatment.
PATIENT CONCERNS
A 75-year-old postmenopausal woman, who underwent a mass excision of the right broad ligament three years ago, was found to have a right adnexal mass during a regular postoperative physical examination.
DIAGNOSES
Vaginal ultrasound examination revealed a cystic and solid mass approximately 3.6 × 4.4 × 3.8 cm on the right side of the uterus. Three years ago, the mass of the right broad ligament was diagnosed with FATWO in the local hospital. Following extensive immunohistochemistry analysis and after reviewing the histology slides from the primary tumor, the final diagnosis of the mass on the right side of the uterus was recurrent and metastatic FATWO.
INTERVENTIONS
The patient underwent laparoscopic mass excision, hysterectomy and resection of the metastatic lesion in the small intestine, and then she received 6 cycles of docetaxel and carboplatin-based chemotherapy.
OUTCOMES
The disease has recurred three years after the first surgery in the local hospital. After the second surgery followed by systemic chemotherapy, there is no evidence of recurrence with 24 months of follow-up till now.
LESSONS
FATWO is considered a benign entity. However, a few FATWOs have been shown to behave aggressively. Due to only a few reported cases, there are no comprehensive recommendations regarding the optimal clinical management of recurrent and metastatic FATWOs. Complete surgical resection followed by combination chemotherapy is considered to be the most effective therapy for recurrent and metastatic FATWOs. Chemotherapy with docetaxel plus carboplatin, which is most commonly used in malignant cases, may be effective in the treatment of recurrent and metastatic FATWOs.
Topics: Adenoma; Adnexal Diseases; Aged; Antineoplastic Combined Chemotherapy Protocols; Broad Ligament; Chemotherapy, Adjuvant; Female; Humans; Hysterectomy; Intestinal Neoplasms; Intestine, Small; Laparoscopy; Neoplasm Recurrence, Local; Tomography, X-Ray Computed; Treatment Outcome; Ultrasonography
PubMed: 33787642
DOI: 10.1097/MD.0000000000025377