A variant of Zinner syndrome with ectopic ureteral insertion into the seminal vesicle.

Zinner syndrome comprises a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ejaculatory duct obstruction, which can be accompanied by additional abnormalities of the genitourinary tract in some cases. Patients may be asymptomatic or present with urinary, reproductive, and/or local pain symptoms. Diagnosis is most commonly achieved via MRI. Here, we present the case of an 18-year-old male previously diagnosed with unilateral renal agenesis, who presented with testicular and penile pain, along with urinary urgency and frequency. MRI of the abdomen and pelvis revealed all three components of Zinner syndrome as well as an ectopic ureter emptying into the seminal vesicle. Our case adds to the existing limited literature on this rare syndrome and broadens the understanding of how this syndrome can present both clinically and radiologically.

A renal aplasia case mimicking radiologically as unilateral renal agenesis in a child with spina bifida, atresia ani and unilateral undescended testis: a case report.

BACKGROUND: As a result of the failure of embryogenic kidney formation, a condition can occur where not a single kidney appears and this phenomenon is known as unilateral renal agenesis (URA). Both aplastic and dysplastic kidney are different from renal agenesis, atrophy and renal hypoplasia. However, from this case report it can be seen that there are similarities, both radiologically and macroscopically, between cases of unilateral renal aplasia and renal agenesis. CASE PRESENTATION: A 2 year old Javanese boy came to the health facility with complaints of recurrent fever and urinary tract symptoms such as dysuria and straining. Computerized Tomography (CT) scan of the abdomen and urography showed agenesis of the left kidney and a probable spina bifida. Cystourethrography examination was done and showed grade 5 voiding, then retrograde pyelography was performed with the diagnosis of unilateral renal agenesis was made because there was no visible left side collecting system even though there was a duplication in the left ureter. The next examination was carried out by histopathology and immunohistochemistry after resection of the left side of the ureter and the diagnosis increasingly pointed towards renal aplasia after primitive renal structures were found. CONCLUSIONS: Renal agenesis and aplastic kidney are difficult to differentiate macroscopically and radiologically. Nevertheless, from this case report, we try to provide some interesting points to differentiate cases of unilateral renal agenesis from Renal Dysplasia which presents as unilateral renal aplasia.

Müllerian anomalies in girls with congenital solitary kidney.

BACKGROUND: The prevalence of Müllerian anomalies (MA) among patients with congenital solitary functioning kidney (SFK) is not well defined. A delay in diagnosis of obstructive MA can increase the risk of poor clinical outcomes. This study describes the prevalence of MA in patients with congenital SFK. METHODS: A retrospective review was performed of patients within the Nationwide Children's Hospital system with ICD9 or ICD10 diagnostic codes for congenital SFK defined as either unilateral renal agenesis (URA) or multicystic dysplastic kidney (MCDK) and confirmed by chart review. Patients with complex urogenital pathology were excluded. Renal anomaly, MA, reason for and type of pelvic evaluation, and age of diagnosis of anomalies were evaluated. RESULTS: Congenital SFK occurred in 431 girls due to URA (209) or MCDK (222). Pelvic evaluation, most commonly by ultrasound for evaluation of abdominal pain or dysmenorrhea, occurred in 115 patients leading to MA diagnosis in 60 instances. Among 221 patients ages 10 years and older, 104 underwent pelvic evaluation and 52 were diagnosed with an MA of which 20 were obstructive. Isolated uterine or combined uterine and vaginal anomalies were the most common MA. MA were five-fold more common in patients with URA compared to MCDK. In 75% of patients, the SFK was diagnosed prior to the MA. CONCLUSIONS: The prevalence of MA in patients with congenital SFK was 24% among those age 10 years or older, and 38% were obstructive. This justifies routine screening pelvic ultrasound in girls with congenital SFK to improve early diagnosis.

Herlyn-Werner-Wünderlich syndrome: Two case report. / Síndrome de Herlyn-Werner Wünderlich:reporte de dos casos.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.

El síndrome de Herlyn-Werner Wünderlich, también llamado OHVIRA por sus siglas en inglés (obstructed hemivagina and ipsilateral renal anomaly), es una anomalía congénita mülleriana poco frecuente que se caracteriza por la asociación entre útero didelfo, hemivagina obstruida y agenesia renal ipsilateral. La presentación clínica más común es la masa abdominal secundaria a hematocolpos, dolor y dismenorrea. Se asocia a infertilidad, endometriosis, alteraciones menstruales y obstétricas. La ecografía es la técnica de elección para la evaluación inicial, mientras que la resonancia magnética sigue siendo el método más exacto para el diagnóstico. La septotomía vaginal es el tratamiento recomendado. Se describen 2 casos clínicos con el objetivo de destacar la importancia del diagnóstico temprano para evitar las posibles complicaciones futuras.

Pregnancy Outcomes in Patients with Obstructed Hemivagina Ipsilateral Renal Agenesis.

STUDY OBJECTIVE: Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) is a rare congenital Müllerian anomaly. We sought to explore the impact of OHVIRA on pregnancy outcomes. METHODS: A retrospective chart review identified 8 patients with an OHVIRA diagnosis and at least 1 documented pregnancy. Data collected included demographic characteristics, age at diagnosis and repair, gynecologic history, and pregnancy outcomes. Descriptive analysis was performed. RESULTS: Twenty-three pregnancies were identified among the 8 patients (range 1-5 pregnancies per patient), resulting in 9 term births, 6 preterm births, and 8 first-trimester spontaneous abortions. The most common pregnancy complications were fetal growth restriction (2/15) and preeclampsia with severe features (2/15). The average gestational age at delivery was 37 2/7 weeks; however, 60% (9/15) of live births were at term. Two patients delivered after going into preterm labor, one of whom also had preterm premature rupture of membranes. Of the 15 live births, there were 10 cesarean deliveries, 4 vaginal deliveries, and 1 forceps-assisted vaginal delivery. Three patients (30%) underwent primary cesarean deliveries due to fetal malpresentation. Five live births were repeat cesarean deliveries, and 2 were unscheduled cesareans, at 34 and 36 weeks, due to preeclampsia with severe features. CONCLUSION: This study is among the first to examine pregnancy outcomes in patients with OHVIRA. Among these patients, there were 23 total pregnancies, with higher incidences of spontaneous abortion, preterm birth, breech presentation, and cesarean delivery compared with the general population. This descriptive data set addresses a knowledge gap to help clinicians more effectively advise patients with OHVIRA about pregnancy outcomes.

Renal agenesis: A meta-analysis of its prevalence and clinical characteristics based on 15 641 184 patients.

Our objective was to analyse the newest relevant data on worldwide prevalence and associated symptoms of renal agenesis (RA). This meta-analysis builds on previous systematic reviews to include bilateral RA, its symptoms and data on gender, unilateral RA and anomaly location prevalence. Review of available data included records in English and other languages from PubMed, Embase, ScienceDirect, Web of Science, SciELO, BIOSIS, Current Content Connect Korean Journal Database and Russian Citation Index and Google. A total of 15 641 184 patients were analysed in relation to the prevalence of RA. The pooled prevalence of RA was 0.03% (95% CI: 0.03%-0.04%). Based on 500 subjects, a pooled prevalence of 47.96% (95% CI: 31.55%-64.58%) for unilateral and 52.04% (95% CI: 35.42%-68.45%) for bilateral RA has been set. Our study presents the newest generalized findings on bilateral RA. There appears to be universal disease and symptom prevalence with minor differences between world regions, although quality of future observational research should include genomic data. This will provide even further insight into the prognosis of various renal anomalies and their etiologies.

Pancreatic solid pseudopapillary neoplasm with concomitant left unilateral renal agenesis and bicornuate uterus: a case report.

A solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor of the pancreas. Concomitant SPN with urogenital anomalies is a very rare presentation. Case Presentation: A 16-year-old female presented with a chief complaint of abdominal pain 30 days back. Solid pseudopapillary neoplasm (SPN) of the pancreas was diagnosed with the aid of ultrasonography and contrast-enhanced computed tomography of the abdomen and pelvis. Incidentally, concomitant left unilateral renal agenesis and bicornuate uterus were also detected in radiological findings. The patient underwent spleen-preserving distal pancreatectomy, and SPN was confirmed with the histopathological report. Discussion: Symptomatic SPN patients present with an abdominal mass and pain or very rarely jaundice. Most of the SPNs are benign. Complete surgical excision results in more than 95% cure. SPN with concomitant urogenital anomalies is extremely rare, and their concurrent occurrence can be better attributed to Wnt signaling pathway owing to their similar pathogenic mechanism. Conclusion: The solid pseudopapillary tumor has an excellent prognosis if timely resected. Proper evaluation of the patient with imaging is necessary to suspect and diagnose SPN who has urogenital anomalies and vice versa.

Follow up of renal outcomes in children with solitary kidney.

BACKGROUND: Solitary kidney (SK) affects 1/1000 people worldwide, and there are controversies concerning renal outcomes in these patients. This study aimed to investigate clinical findings and renal outcomes in children with SK and to compare the results for congenital (CSK) and acquired SK (ASK) groups. METHODS: The study included patients that presented to our pediatric nephrology department with SK between January 2010 and January 2021. Demographic and clinical data were recorded retrospectively. RESULTS: Of the 101 patients with SK, 71 had CSK (55 had unilateral renal agenesis and 16 had a multicystic dysplastic kidney) and 30 had ASK (17 had previously undergone unilateral nephrectomy due to a renal tumor and 13 had urological structural anomalies). There were nine patients (9%) with renal injury. The serum uric acid level was significantly higher and the estimated glomerular filtration rate was significantly lower in the patients with ASK compared with those with CSK (p = 0.005 and p < 0.001, respectively). There was a positive correlation between renal injury and the uric acid level (p < 0.001, r = 0.45). CONCLUSION: In addition to the management of blood pressure and proteinuria, it is important to control uric acid levels in patients with SK, especially those with ASK, to prevent renal injury. The ASK group has a greater risk of renal injury than the CSK group. There is a need for new markers to predict early stage renal damage in SK.

Síndrome de cascanueces y agenesia renal derecha. Una rara asociación

Introducción: El síndrome de cascanueces se considera una anomalía infrecuente y poco pensada en la práctica médica; su incidencia no está bien establecida debido a su sintomatología variada. La agenesia renal unilateral se estima entre 1/2500 y 1/4000 nacidos vivos. Objetivos: Describir una paciente de 18 años con sospecha prenatal de agenesia renal derecha confirmada por imágenes después del nacimiento, que ingresó por proteinuria. Presentación del caso: Paciente de 18 años con agenesia renal derecha conocida, en la que un examen de orina detectó proteinuria desde los 13 años. En su estudio se clasificó como proteinuria ortostática y evolutivamente refirió dolor lumbar izquierdo ligero y transitorio. En el ultrasonido renal, realizado para valorar crecimiento del riñón único, se detectó dilatación de la vena renal izquierda. Se repitió el estudio ecográfico para precisar ángulo aorto-mesentérico y dilatación de vena renal izquierda, y se confirmó el síndrome de cascanueces. Conclusiones: La asociación entre agenesia renal derecha y síndrome de cascanueces, resulta extremadamente rara, y, cuando se presenta con proteinuria ortostática y dolor lumbar ocasional, debe seguirse en forma expectante, pero no se necesita tratamiento quirúrgico en la mayoría de los casos.

Introduction: Nutcracker syndrome is considered an infrequent and poorly thought out anomaly in medical practice; its incidence is not well established due to its varied symptomatology. Unilateral renal agenesis is estimated to be between 1/2500 and 1/4000 live births. Objectives: To describe an 18-year-old female patient with suspected pre-natal imaging-confirmed right renal agenesis after birth, who was admitted due to proteinuria. Case presentation: An 18-year-old female patient with known right renal agenesis, in whom a urine test detected proteinuria from the age of 13. In the study it was classified as orthostatic proteinuria and evolutionarily she referred mild and transient left low back pain. Renal ultrasound, performed to assess single kidney growth, showed dilation of the left renal vein. The ultrasound study was repeated to specify aorto-mesenteric angle and left renal vein dilation, and nutcracker syndrome was confirmed. Conclusions: The association between right renal agenesis and nutcracker syndrome is extremely rare, and, when it presents with orthostatic proteinuria and occasional low back pain, it should be followed expectantly, but surgical treatment is not needed in most cases.

Zinner Syndrome with Papillary Neoplasm: A Rare Case Report.

One of the uncommon urogenital malformations in males, which presents late in the second or third decades of life, is the congenital malformation of the seminal vesicle. Zinner syndrome is a rare condition comprising a triad of unilateral renal agenesis, ipsilateral seminal vesicle obstruction, and ipsilateral ejaculatory duct obstruction. Very few cases in the literature describe the malignant change in seminal vesicle cyst in Zinner syndrome. To the best of our knowledge, this is the first report of a papillary neoplasm of the seminal vesicle cyst developed in a patient with Zinner syndrome.

Solitary kidney functional damage due to caesarean ureteric injury monitored for 2 years after acute management: A case report.

Caesarean section with associated ureteric injuries that damage kidney function is uncommon. Such injury in women with a solitary kidney has far-reaching effects if the diagnosis is delayed. The case report describes the successful acute management with stenting of ureteric damage after caesarean section in a woman with a solitary kidney. A 29-year-old woman presented with anuria, hypertension (174/101 mmHg) and flank pain 3 days after caesarean section. Physical examination was significant for peri-orbital oedema, left flank tenderness, pallor and delirium. A diagnosis of acute kidney injury was confirmed by elevated blood urea and creatinine levels. An ultrasound scan revealed a solitary hydronephrotic left kidney. She had successful acute management at a resource-deprived facility, with normal renal function at a 2-year follow-up. Proteinuria lasted for about three months after surgery. Recovery of solitary kidney function with acute kidney injury due to caesarean section ureteric injury may be associated with prolonged proteinuria without evidence of further functional deterioration.

Should we screen for Müllerian anomalies following diagnosis of a congenital renal anomaly?

INTRODUCTION: Despite the well-established embryological relationship in the development of renal and Müllerian structures, no clear guidelines exist regarding screening for Müllerian anomalies (MA) in the setting of a renal anomaly (RA). Delayed diagnosis of MA can have significant reproductive consequences. OBJECTIVE: To investigate the prevalence of coexisting MA in patients with congenital RA. STUDY DESIGN: This is a retrospective cohort study of females age 12-35 years with a diagnosis of RA, identified by diagnosis codes, who were followed for care between 2013 and 2020. Data were collected on demographics, medical history, clinical presentation, and imaging studies. Descriptive statistics were used to summarize the data. This study was IRB approved. RESULTS: A total of 465 patients were included in this study, of whom 326 patients (70.3%) had a pelvic evaluation during the study period. Of these 326 patients, 125 (38.3%) were found to have coexistent MA. About one-third of patients who underwent pelvic evaluation due to pain were found to have MA. For 69.6% (87/125) of patients with MA, the RA was diagnosed prior to the MA. The average age at time of RA diagnosis was 6.4 ± 8.8 years and the average age of MA diagnosis was 16.4 ± 6.9 years. Forty-eight (38.4%) patients had obstructive anomalies. Of the Müllerian obstructions, 93.8% were treated with urgent surgery and the remainder started on hormonal suppression. The prevalence of MA was dependent on the RA diagnosis (Figure). Of patients with a solitary kidney, 67.1% were diagnosed with MA. For other parenchymal RA, the prevalence of MA was 20-23%. In patients with solitary kidney, uterus didelphys was the most common MA (52.1%). Thirty percent of patients with a solitary kidney were diagnosed with an obstructive MA. CONCLUSIONS: In this study, 38% of patients with RA who underwent a pelvic evaluation were found to also have MA. Our study shows the strongest association between MA and solitary kidney, but also emphasizes a significant risk with other RA. Almost 40% of patients with diagnosed MA were found to have an obstruction that required urgent treatment. Delays in diagnosis and treatment of Müllerian obstructions can be detrimental for future reproductive health, due to risk of chronic pain, infertility, infection, and endometriosis. Given the high prevalence of MA in patients with RA, especially those with congenital solitary kidney, routine screening with pelvic ultrasound should be performed around the age of expected menarche.

Zinner Syndrome: A Case Report of a Rare Etiology of Infertility.

Infertility is a common medical issue with different etiologies. It can be related to female factors, male factors, or factors related to both partners. We report the case of a 35-year-old male patient who presented with primary infertility for 10 years. He was otherwise healthy with unremarkable past medical history. Physical examination revealed normal external genitalia with both testes were normal in size. Basic semen analysis revealed decreased sperm volume and sperm count. Abdominal ultrasound examination revealed an absent right kidney. Subsequently, the patient under a computed tomography scan that confirmed the right renal agenesis and demonstrated a well-defined right seminal vesicle cyst. Such findings were consistent with the diagnosis of Zinner syndrome. He underwent aspiration of the cyst that resulted in improvement in the sperm parameter of basic semen analysis. The case demonstrated a rare etiology of male infertility that was successfully managed conservatively. Despite its rarity, physicians should consider the developmental anomalies of the genitourinary system when encountering patients with infertility.

Clinical Management of Children with a Congenital Solitary Functioning Kidney: Overview and Recommendations.

CONTEXT: A congenital solitary functioning kidney (cSFK) is a common developmental defect that predisposes to hypertension and chronic kidney disease (CKD) as a consequence of hyperfiltration. Every urologist takes care of patients with a cSFK, since some will need lifelong urological care or will come with clinical problems or questions to an adult urologist later in life. OBJECTIVE: We aim to provide clear recommendations for the initial clinical management and follow-up of children with a cSFK. EVIDENCE ACQUISITION: PubMed and EMBASE were searched to identify relevant publications, which were combined with guidelines on related topics and expert opinion. EVIDENCE SYNTHESIS: Initially, cSFK diagnosis should be confirmed and risk factors for kidney injury should be identified using ultrasound. Although more research into early predictors of kidney injury is needed, additional congenital anomalies of the kidney or urinary tract and absence of compensatory kidney hypertrophy have repeatedly been associated with a worse prognosis. The role of voiding cystourethrography and antibiotic prophylaxis remains controversial, and is complicated by the exclusion of children with a cSFK from studies. A yearly follow-up for signs of kidney injury is recommended for children with a cSFK. As masked hypertension is prevalent, annual ambulatory blood pressure measurement should be considered. During puberty, an increasing incidence of kidney injury is seen, indicating that long-term follow-up is necessary. If signs of kidney injury are present, angiotensin converting enzyme inhibitors are the first-line drugs of choice. CONCLUSIONS: This overview points to the urological and medical clinical aspects and long-term care guidance for children with a cSFK, who are at risk of hypertension and CKD. Monitoring for signs of kidney injury is therefore recommended throughout life. Large, prospective studies with long-term follow-up of clearly defined cohorts are still needed to facilitate more risk-based and individualized clinical management. PATIENT SUMMARY: Many children are born with only one functioning kidney, which could lead to kidney injury later in life. Therefore, a kidney ultrasound is made soon after birth, and other investigations may be needed as well. Urologists taking care of patients with a solitary functioning kidney should realize the long-term clinical aspects, which might need medical management.

Síndrome de Zinner: monorrenos especiales / Zinner syndrome: special single kidney patients

El síndrome de Zinner es una rara entidad urológica caracterizada por la dilatación quística unilateral de una vesícula seminal junto con atrofia o agenesia renal ipsilateral. Los pacientes permanecen asintomáticos en la mayoría de los casos y su diagnóstico es incidental. El tratamiento de elección es quirúrgico cuando presentan sintomatología o se ve afectada la fertilidad del paciente. Presentamos el caso de un paciente de 13 años, monorreno de nacimiento, asintomático, sin otros antecedentes personales de interés, al que, durante un examen rutinario de su patología de base, se le realiza ecografía abdominal en la que se visualiza un probable quiste ureteral con una dilatación de la vesícula seminal compatible con un probable síndrome de Zinner.(AU)

Zinner syndrome is a rare urological entity characterized by unilateral cystic dilation of a seminal vesicle together with atrophy or ipsilateral renal agenesis. Patients remain asymptomatic in most cases and their diagnosis is incidental. The treatment of choice is surgical when symptoms occur or affect the patient´s fertility. We report the case of a 13-year-old asymptomatic boy born with a single-kidney with no other personal history of interest. During a routine examination of his underlying pathology, abdominal ultrasound was performed where a probable ureteral cyst with seminal vesicle dilation compatible with probable Zinner syndrome was observed.(AU)

Retrospective evaluation of children with unilateral renal agenesis.

BACKGROUND: Children born with unilateral renal agenesis (URA) are thought to have a risk of developing hypertension, proteinuria, and progressive chronic kidney disease (CKD). The present study aimed to evaluate the long-term prognosis and clinical characteristics of children with URA. METHODS: The study included 171 patients aged < 18 years diagnosed as URA who were followed-up for ≥ 1 year and 121 healthy controls matched for age, gender, and BMI. RESULTS: Median age at diagnosis was 2 years (IQR: 1 month-16 years) and the incidence of URA in males (65.4%) was higher than in females. Among the patients, 21 (12.2%) had other urinary system anomalies. It was noted that 2.3% of the patients had proteinuria, 15.2% had hyperfiltration, and 2.9% had CKD. Hypertension based on ambulatory blood pressure monitoring (ABPM) was diagnosed in 18 (10.5%) of the patients, of whom 10 had masked hypertension. Diastolic blood pressure in the URA patients was significantly higher than in the healthy controls. The incidence of hypertension and CKD was significantly higher in the patients with other urinary system anomalies. CONCLUSIONS: Patients with a single functional kidney should be periodically evaluated throughout their lifetime for urine protein, blood pressure, and kidney functions. The most remarkable finding of this study is the importance of the use of ABPM for evaluating blood pressure in pediatric URA patients, especially for the detection of masked hypertension and the non-dipper phenomenon, which cannot be achieved with office blood pressure measurement.

Renal growth slope in children with congenital and acquired solitary functioning kidneys.

PURPOSE: This study aimed to analyze the renal growth slope in children with congenital and acquired solitary functioning kidneys. METHODS: This retrospective study included all renal ultrasonography examinations performed in children in the agenesis, multicystic dysplastic kidney (MCDK), or nephrectomy group between September 2002 and February 2019. We reviewed the images and recorded the contralateral kidney size only when there was no focal lesion. Linear mixed model or piecewise linear mixed model analyses with a time point of 24 months of age were performed. RESULTS: There were 132 patients, including 26 patients in the agenesis group, 35 in the MCDK group, and 71 in the nephrectomy group. The nephrectomy group showed the largest baseline kidney size (7.4 cm vs. 5.3 cm in the agenesis group [P<0.001] and 5.2 cm in the MCDK group [P<0.001]) and the smallest overall growth slope (0.04 cm/mo vs. 0.06 cm/mo in the agenesis group [P=0.004] and 0.07 cm/mo in the MCDK group [P<0.001]). However, considering the time point of 24 months for reaching adult renal function, there were significant changes in slope, from 0.1 cm/mo before 24 months of age to 0.03 cm/mo after 24 months of age in all three groups (P<0.001), without a significant difference among the groups. CONCLUSION: Significant changes were found in the renal growth slope before and after 24 months of age, with no significant difference between congenital and acquired solitary functioning kidneys.

A case of dorsal agenesis of pancreas associated with unilateral renal agenesis, unicornuate uterus, and ovarian ectopia: A brief review and learning points.

Dorsal agenesis of the pancreas is a rare entity, with about 100 cases reported. It can be overlooked on ultrasound due to the non visualization of the body and tail of the pancreas. This is due to overlying gas in the stomach, which offers a poor acoustic window and obscures visualization. Renal agenesis and Mullerian duct anomalies are uncommon associations of dorsal agenesis of the pancreas because of the separate embryological origin of the pancreas and genitourinary organs. Here, we present a case of a 17-year-old patient who had dorsal agenesis of the pancreas, associated with unilateral renal agenesis, unicornuate uterus, and ectopic ovary. We describe the anomalies and discuss the radiological differential diagnosis and potential pitfalls. We provide a brief review of the literature with few radiological teaching points and possible genetic implications of the case.

IMRT and HDR-ICBT for Locally Advanced Clear Cell Adenocarcinoma of the Cervix in Uterus Didelphys Associated With Unilateral Renal Agenesis.

Clear cell adenocarcinoma of the cervix (CCAC) with genitourinary malformations is rare. Here, we report a case of CCAC in uterus didelphys (UD) associated with unilateral renal agenesis (URA) that was treated with intensity-modulated radiotherapy (IMRT) and high-dose rate intracavitary brachytherapy (HDR-ICBT). We also retrospectively reviewed the medical records of CCAC cases with genitourinary malformations treated at the National Cancer Center/Cancer Hospital (Beijing, China) between December 2006 and June 2017. Eight cases of this rare condition were identified by pathologic diagnosis. Seven patients received surgical treatment including radical hysterectomy (n = 4), modified radical hysterectomy (n = 1), and total hysterectomy (n = 2). Five patients received adjuvant radiotherapy and chemotherapy after surgery. One patient with CCAC in UD associated with URA was treated with radical IMRT and adjuvant chemotherapy. The eight patients were followed up for an average of 7.9 years; in seven cases, there was no evidence of disease recurrence, while one patient relapsed and died after 1.5 years of treatment. On the basis of these findings, locally advanced CCAC in UD associated with URA can be effectively treated with radical IMRT.

Carcinoma of the lower female genital tract in patients with genitourinary malformations: a clinicopathologic analysis of 36 cases.

Background: Lower genital tract carcinomas that coexist with genitourinary malformations are rare. The aim of this study was to investigate the clinicopathologic characteristics and outcomes of patients with this rare joint condition. The aim of this study was to investigate the clinicopathologic characteristics and outcomes of these patients to analyze the associations among clinicopathologic features in this rare entity. Methods: The medical records of patients with carcinoma of lower genital tract combined with genital tract malformations who were treated at Peking Union Medical College Hospital (Beijing, China) between January 1995 and December 2017 were retrospectively reviewed and a comprehensive literature review was performed. The correlations between variables were determined using Fisher's exact test. Results: Thirty-six patients were identified, among whom 22 had cervical carcinoma, 10 had vaginal carcinoma, and two had carcinomas of both the cervix and vagina. The most common genital tract anomaly was uterus didelphys (n=17), and 63.9% of patients had with urinary tract malformations. Twenty-five (69.4%) had pathologically confirmed adenocarcinoma, 10 (27.8%) had squamous cell carcinoma (SCC), and 1 had adenosquamous carcinoma. Patients with urinary tract malformations developed adenocarcinoma more frequently than those without malformations (95.7% vs. 30.8% P<0.001). Compared with patients with SCC, patients with adenocarcinoma presented at a younger age (36.1 vs. 47.0 years, P=0.011), had more advanced stages disease (57.7% vs. 10.0% were in stage II-IV, P=0.022), and tended to have poorer five-year overall survival rates (75.6% vs. 100.0%, P=0.279). Conclusions: Patients with simultaneous malformations of the genital and urinary tracts had a high probability of developing adenocarcinoma. It is recommended that rigorous gynecologic exams with Pap smears and imaging analyses be performed periodically in women with genital malformations, as they may be at a risk of genital malignancies.