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Disease Models & Mechanisms Jul 2023Little is known about the distal excretory component of the urinary tract in Danio rerio (zebrafish). This component is affected by many human diseases and disorders of...
Little is known about the distal excretory component of the urinary tract in Danio rerio (zebrafish). This component is affected by many human diseases and disorders of development. Here, we have undertaken multi-level analyses to determine the structure and composition of the distal urinary tract in the zebrafish. In silico searches identified uroplakin 1a (ukp1a), uroplakin 2 (upk2) and uroplakin 3b (upk3b) genes in the zebrafish genome (orthologues to genes that encode urothelium-specific proteins in humans). In situ hybridization demonstrated ukp1a expression in the zebrafish pronephros and cloaca from 96 h post-fertilization. Haematoxylin and Eosin staining of adult zebrafish demonstrated two mesonephric ducts uniting into a urinary bladder that leads to a distinct urethral opening. Immunohistochemistry identified Uroplakin 1a, Uroplakin 2 and GATA3 expression in zebrafish urinary bladder cell layers that match human urothelial expression. Fluorescent dye injections demonstrated zebrafish urinary bladder function, including urine storage and intermittent micturition, and a urethral orifice separate from the larger anal canal and rectum. Our findings reveal homology between the urinary tracts of zebrafish and humans, and offer the former as a model system to study disease.
Topics: Animals; Humans; Adult; Zebrafish; Membrane Glycoproteins; Uroplakin Ia; Uroplakin II; Urinary Bladder
PubMed: 37293698
DOI: 10.1242/dmm.050110 -
Asian Journal of Surgery Oct 2023
Topics: Female; Humans; Mesonephroma; Uterine Cervical Neoplasms; Adenocarcinoma
PubMed: 37230815
DOI: 10.1016/j.asjsur.2023.05.057 -
JNMA; Journal of the Nepal Medical... Mar 2023Herlyn-Werner-Wunderlich syndrome is a rare Mullerian and mesonephric ductal anomaly characterized by a triad of didelphys uterus, obstructed hemivagina, and ipsilateral...
UNLABELLED
Herlyn-Werner-Wunderlich syndrome is a rare Mullerian and mesonephric ductal anomaly characterized by a triad of didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis complex. This entity is also known as obstructed hemivagina and ipsilateral renal anomaly. We present a case of a 24-year-old nulliparous female with Herlyn-Werner-Wunderlich who presented with dysmenorrhea and intermenstrual bleeding. The diagnosis was initially made through ultrasound and confirmed on magnetic resonance imaging. The nonspecific nature of symptoms and variability in presentation depending on the classification and type of Herlyn-Werner-Wunderlich syndrome often leads to misdiagnosis or a delay in diagnosis. Therefore, a high index of suspicion is required.
KEYWORDS
case reports; mesonephric ducts; mullerian ducts.
Topics: Female; Humans; Young Adult; Adult; Vagina; Kidney; Uterus; Kidney Diseases; Abnormalities, Multiple
PubMed: 37203938
DOI: 10.31729/jnma.8096 -
Journal of Cancer Research and... Apr 2023Wilms' tumor (or nephroblastoma) is the most common renal malignancy in the pediatric population which consists of blastemal, epithelial, and stromal elements in...
Wilms' tumor (or nephroblastoma) is the most common renal malignancy in the pediatric population which consists of blastemal, epithelial, and stromal elements in variable proportions. The occurrence of renal cysts in children and infants is a rare phenomenon and is possibly an outcome of developmental aberrations in mesonephric blastema. The coincidental association of nephroblastoma with renal cysts is a very rare finding. Here, we describe two cases of Wilms' tumor with an unusual association between glomerulocystic kidney disease and multicystic dysplastic kidney.
Topics: Infant; Child; Humans; Wilms Tumor; Kidney; Kidney Neoplasms; Carcinoma, Renal Cell; Kidney Diseases, Cystic
PubMed: 37147970
DOI: 10.4103/jcrt.jcrt_275_22 -
Diagnostic Pathology Mar 2023Splenogonadal fusion (SGF) is a rare congenital malformation in which the spleen is abnormally connected to the gonads or to the mesonephric derivatives. There is no... (Review)
Review
BACKGROUND
Splenogonadal fusion (SGF) is a rare congenital malformation in which the spleen is abnormally connected to the gonads or to the mesonephric derivatives. There is no obvious causality between SGF and testicular neoplasm. However, cryptorchidism, which is a well-known risk factor of testicular germ cell tumors, are the most frequent malformations associated with SGF. To our knowledge, there are only four reported cases of SGF associated with testicular neoplasm so far. Herein, we reported a patient of this condition, and briefly reviewed the related literature.
CASE PRESENTATION
A 48-year-old man was diagnosed with bilateral cryptorchidism 30 years prior, and only underwent a right orchiopexy for the left testicle could not be explored during the operation. At that time, doctors failed to realize the possibility of SGF due to the lack of sufficient knowledge of this condition. This time, the patient was treated for a left abdomen mass that was diagnosed as stage III metastatic seminoma. Then, a right orchiectomy, robot-assisted laparoscopic left retroperitoneal tumor resection, and left retroperitoneal lymph node dissection was performed after four cycles of BEP (bleomycin + etoposide + cisplatin) systemic chemotherapy in our center. The final diagnosis of SGF was made by postoperative pathology. The patient was re-examined in our center at 3 months and 6 months after the operation, and no obvious abnormalities were found.
CONCLUSIONS
Surgeons should always bear in mind the possibility of association between bilateral cryptorchidism and splenogonadal fusion to avoid malignant transformation caused by delayed treatment.
Topics: Humans; Cryptorchidism; Male; Middle Aged; Seminoma; Antineoplastic Combined Chemotherapy Protocols; Orchiectomy; Splenic Diseases; Testicular Neoplasms; Gonads; Spleen; Treatment Outcome
PubMed: 36998078
DOI: 10.1186/s13000-023-01332-w -
Folia Medica Cracoviensia Dec 2022Hutch Diverticulum (HD) is defined as the protrusion of the mucosal and submucosal layer through the muscle bundles of the underlying detrusor muscle. HD is located at... (Review)
Review
Hutch Diverticulum (HD) is defined as the protrusion of the mucosal and submucosal layer through the muscle bundles of the underlying detrusor muscle. HD is located at the vesicoureteral junction with a backward direction from the homolateral ureteral orifice. As far as its etiology is concerned, HD is caused either by a congenital muscle wall defect at the level where the Waldeyer's fascia occupies the clefts between the vesical part of the homolateral ureter and the detrusor, or is associated with abortive ureteral duplication or defective incorporation of mesonephric duct into the bladder at the site of ureteral hiatus or finally is associated with the development of transient urethral obstruction. HD is usually unilateral and more common in male patients. It may be associated with the Ehlers-Danlos, Williams-Elfin and Menkes syndromes. HD usually occurs in childhood and rarely during adulthood. It is found in 0.2-13% of all children presenting with urinary tract infection. Through this short review article, we attempt to present in detail the most recent bibliographic data concerning this entity, focusing on pathophysiology, diagnostic approach, and treatment strategy.
Topics: Child; Humans; Male; Adult; Urinary Bladder; Fascia; Diverticulum
PubMed: 36854087
DOI: 10.24425/fmc.2022.144083 -
Asian Journal of Surgery Jul 2023
Topics: Female; Humans; Uterine Cervical Neoplasms; Mesonephroma; Cervix Uteri; Adenocarcinoma
PubMed: 36841625
DOI: 10.1016/j.asjsur.2023.02.012 -
Modern Pathology : An Official Journal... Apr 2023Endometrial carcinoma (EC) can be divided into 4 prognostic molecular subtypes, and no specific molecular profile (NSMP) type is the most commonly occurring type...
Endometrial carcinoma (EC) can be divided into 4 prognostic molecular subtypes, and no specific molecular profile (NSMP) type is the most commonly occurring type (∼50%). Although described as having an intermediate to favorable prognosis, this subtype encompasses pathologically and molecularly diverse tumors. We aimed to identify factors associated with outcomes within the NSMP ECs that might be used to stratify prognosis and direct treatment. Clinicopathologic, immunohistochemical, and genetic features of a large series of NSMP EC were used to identify parameters that could identify the subset associated with a very favorable outcome (disease-specific death rate <5% at 5 years, termed low-risk NSMP). A total of 1110 NSMP ECs were profiled. In a univariate analysis, stage, grade, lymphovascular invasion, estrogen receptor (ER) and progesterone receptor (PR) expression, L1CAM overexpression, and mutations in PIK3CA were associated with disease-specific survival. Two critical features, grade and ER expression, identified a low-risk NSMP subset (grade 1-2, ER-positive [>1%], 84% of cases), which showed a 5-year disease-specific death rate of 1.6% across all stages and 1.4% within stage I. The remaining cases (high-risk NSMPs, grade 3, and/or ER-negative status) were responsible for most of the disease-specific deaths (disease-specific death rate at 5 years, 22.9%; hazard ratio compared with that of low-risk NSMPs: 16.3; 95% CI, 8.4-31.7). Within NSMP EC, the low-risk and high-risk categories were of prognostic significance independent of the stage on a multivariate analysis. Low-grade and ER-positive NSMP ECs are a homogeneous low-risk group associated with an exceptionally favorable prognosis in which de-escalation and/or endocrine therapy strategies can be applied. Grade 3 and/or ER-negative status identifies a high-risk NSMP subset, including rare high-grade histotypes (eg, clear cell, dedifferentiated, and mesonephric-like), responsible for most NSMP-related deaths. Subclassification of NSMPs allows for the category of low-risk EC molecular subtypes to be dramatically expanded because it now includes both POLEmut and the much more common low-risk NSMP EC.
Topics: Female; Humans; Receptors, Estrogen; Endometrial Neoplasms; Prognosis; Risk Factors; Biomarkers, Tumor; Carcinoma, Endometrioid
PubMed: 36788084
DOI: 10.1016/j.modpat.2022.100085