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Purpose: It has been suggested that symptomatic UA requires surgical excision. However, the management of asymptomatic urachus is still controversial. We aimed to evaluate the clinical presentation, the efficacy of current modalities used, and postoperative pathology in patients with UA. Material and Methods: We have performed a retrospective review of all patients diagnosed with UA and treated surgically or conservatively over 18 years. Demographic data, clinical presentation, imaging modalities, pathology, treatment, and postoperative complications were analyzed. Results: Twenty-five symptomatic patients (18 males and seven females) with a median age of 13 years (one month to 37 years) were identified. 15 (60%) were diagnosed with a urachal cyst, 4(16%) with sinus, 3(12%) with urachal diverticulum, and the remaining 3(12%) with patent urachus. Of those, 20(80%) underwent surgical repair, and the remaining five (20%) patients were managed conservatively. 4 (20%) underwent laparotomy, 7 (35%) laparoscopic incision, and the remaining 9 (45%) laparoscopic robotic-assisted surgery. Nine patients required bladder cuff excision. The median operative time was 75 minutes (42-140 min). One patient developed Clavien Dindo grade IIIA complication resulting in infected hematoma, which resolved after drainage. Another patient with a complication of grade IIIB needed reoperation as a result of recurrent events of an abscess. 13 (65%) demonstrated epithelium lining of the urachus on postoperative pathology.Conclusions: Our data show that most of the patients with UA presented with epithelial lining, which might lead to the later malignant transformation. It might cause a shift from the conservative management of asymptomatic patients to surgical intervention. Robotic-assisted surgery appears beneficial in these patients, especially when the bladder cuff excision is required.

Objective: The aim of this study was to improve understanding the clinical, pathologic, and prognostic features of urachal carcinoma (UrC), a retrospectively descriptive study was done in 2 clinical centers. Methods: After excluding the 2 missed patients, the clinical and pathological data of 59 patients with UrC, who were diagnosed or treated at 2 clinical centers between 1986 and 2019, was retrospectively analyzed. SPSS 22.0 (IBM) and GraphPad Prism 8.0.1 were used for statistics and data visualization. Survival data were analyzed by the Kaplan-Meier method and Log-rank tests. Cox proportional hazards regression were performed for find risk factors on predicting the prognosis. Results: Of all 59 patients, 47 were male and 12 were female. The median age at diagnosis was 51.6 years (range: 22–84 years). Gross hematuria was the most common symptom (79.66%). The majority of urachal neoplasms were adenocarcinomas (94.92%). Forty-two patients (72.41%) underwent extended partial cystectomy with en bloc resection of the entire urachus. The mean follow-up was 52 months (3–277 months). Median overall survival was 52.8 months (4–93 months). The 3-year cancer-specific survival (CSS) rate and 5-year CSS rate were 69.1% and 61.2%. There was no significant difference among localized T stage, tumor histologic grade and surgical procedures in determining prognosis by survival analyze. While patients with high-risk TNM stage (local abdominal metastasis, lymph node metastasis, or distant metastasis) (p = 0.003) and positive surgical margin (p < 0.001) had significantly worse prognosis. Conclusions: The results indicate that high-risk TNM stage and positive surgical margin are risk predictors of prognosis. Localized T stage, histologic grade, and surgical procedure cause no significant effect on patient prognosis. The extended partial cystectomy is the recommended surgical approach for patients with UrC. Active multimodal treatments may improve the survival of patients with recurrent and metastatic disease.

Urachus is a tubular connection between the umbilical cord and the bladder of developing foetus and tends to degenerate during perinatal period to form an impatent median umbilical ligament. Failure to degenerate results in patent canal between the bladder and the umbilicus called "patent urachus" which may lead to serious of symptoms such as umbilical discharge, dermatitis, umbilical infection, abdominal pain or recurrent urinary tract infections. The Tenckhoff catheter is a tube used to perform peritoneal dialysis that is inserted through abdominal wall into peritoneum either by open surgery, minilaparotomy, laparoscopy or needle-guidewire technique.A 57-years old man was admitted to the hospital after implantation of Tenckhoff catheter by percutaneous technique in order to start peritoneal dialysis treatment. His medical history was: endstage chronic kidney disease (diabetic nephropathy), type 2 diabetes and hypertension. After the infusion of dialysate the patient experienced sudden urine pressure and passed significant amount of urine. The CT scan showed the tip of catheter being placed inside the urinary bladder. The catheter was introduced through the abdominal wall into the canal of previously undiagnosed patent urachus. The decision about re-surgery was made to stitch urachal remnants and place new the Tenckhoff catheter. Awaiting the surgery patient temporary started hemodialysis. In ongoing observation patient did not present any complications associated with peritoneal dialysis treatment.

Objective To report a multicenter experience with the management of urachal abscess treatment in Japan. Methods This was a retrospective study of 263 cases of urachal abscess managed at 12 university hospitals in the Kyushu-Okinawa region over a 10-year period. Age, sex, abscess size, clinical symptoms, type of urachal remnants, and treatment were collected and analyzed. Results The average age was 29.8 ± 18.1 years, with males accounting for approximately two-thirds of the study population. The average abscess size was 1.7 cm (range 0-11 cm). The most common presenting symptom was umbilical secretion (66%), followed by abdominal pain (46%). A total of 127 patients (48.3%) were treated with antibiotics alone, whereas 136 patients (51.7%) received surgical treatment. The surgical approach was laparotomy in 75 patients (61.0%) and laparoscopic surgery in 48 patients (39.0%). Regarding the type of urachal remnant, the urachus sinus (180 patients) accounted for 68.4% of the total. Conclusions To our knowledge, this study represents the first report on urachal abscess treatment in Japan. Our data show that the clinical symptoms might vary depending on the type of urachus remnant. It should be noted that gross hematuria, a characteristic symptom of urachal cancer, is rare in patients with urachal abscess.

Purpose To discuss whether the dome or anterior wall of bladder adenocarcinoma (BAC) should be classified into urachal carcinoma (UrC) and the relationship of primary tumor location (PTL) as well as treatment with survival. Methods Surveillance, Epidemiology, and End Results 18 database was examined for eligible patients from 1975 to 2016. Patients were classified into adenocarcinoma originating from the urachus (UAC), the dome (D‐BAC), the anterior wall (A‐BAC), and the other sites adenocarcinoma of the bladder (O‐BAC). The clinicopathological features, treatment, and survival were compared among the groups. Results Comparable clinicopathologic features were obtained between UAC and D‐BAC, which were different from those of A‐BAC and O‐BAC; otherwise, the latter two had similar clinicopathologic features. Univariable and multivariable Cox regression analyses indicated that PTL was an independent predictor for survival. O‐BAC conferred the worst prognosis then followed by A‐BAC, D‐BAC, and UAC. For non‐metastatic UAC or D‐BAC, partial cystectomy (with an en bloc resection of the urachus and umbilicus) is optimal for survival. However, the worse survival of non‐metastatic D‐BAC (compared with UAC) suggested different modalities, maybe more intensive surgery approaches, should be considered for D‐BAC. Conclusion This study illustrates that PTL of UAC and BAC was an independent predictor for survival. A‐BAC had comparable characters and prognosis with O‐BAC and should not be classified into and treated as UrC. For non‐metastatic disease, non‐metastatic D‐BAC may need more intensive modality., Anterior wall of bladder adenocarcinoma (A‐BAC) had comparable characters and prognosis with other sites of bladder adenocarcinoma, while dome adenocarcinoma of the bladder is similar to urachal adenocarcinoma arising from the urachus. A‐BAC should be classified into and treated as BAC, instead of urachal carcinoma.

The urachus is a remnant of the allantois. Failure to obliterate can result in one of four anomalies, urachal cyst being most common. Urachal cysts are relatively rare, especially in adults. This paper presents a patient with an umbilical hernia and a ruptured urachal cyst. A 39-year-old male presented with concern for umbilical hernia, but he also noted drainage. Computed tomography scan showed a urachal cyst and umbilical hernia. The urachal cyst was excised and umbilical hernia closed primarily. The incidence of an urachal cyst is unknown, but persists in roughly 2% of adults. Diagnosis is with ultrasound or CT scan. Management is excision due to risk of urachal carcinoma, which is present in over 50% of specimens. Review of literature did not reveal any other cases of a patient with both an urachal cyst and an umbilical hernia, thus making this case a unique presentation for this condition.

We describe our experience in 2 institutions handling bladder prolapse through a patent urachus (PU), together with a brief review of published literature. Case 1: A term neonate with congenital prolapsed bladder via PU. Ultrasound at 21 weeks gestation revealed a male fetus with a large midline pelvic cyst communicating with the bladder which disappeared on subsequent 27 weeks ultrasound. Case 2: A term female neonate with congenital prolapsed bladder via PU with no prenatal diagnosis. In both cases the bladder closure was undertaken during the newborns’ first days of life.

Urachal remnants are thought to have a low prevalence. However, recent studies indicate a higher rate. The aim of this study was to evaluate the prevalence of urachal remnants in children referred for imaging by abdominal/urinary/suprapubic ultrasonography in one calendar year. Files of children who underwent abdominal/urinary/suprapubic ultrasonography in one calendar year were retrospectively reviewed. Data regarding children ≤17 years that underwent ultrasonography of the ventral abdominal wall while being assessed for various reasons were collected. Anomalies detected, age, gender, reason for ultrasonography request, and diagnosis of the urachal remnant were noted. All ultrasonography assessments were performed by a single pediatric radiologist in a single institution. There were 4836 patients in the study (1919 boys). Median age of the whole cohort was 7 years. A total of 10 patients were found to have urachal remnants, which included eight urachal cysts (three boys) and two urachal diverticula (one boy).Conclusion: The prevalence of urachal remnants in our cohort was nearly 2 in every 1000 children. More specifically, the prevalence of urachal cysts was one in 600. What is Known: • Urachal remnants were believed to be rare. • Recent studies indicated a higher incidence than previous reports. What is New: • This study shows that urachal remnant may be observed in one every 500 children that undergo ultrasonography of the abdomen for various reasons. • Parental counselling and management of urachal remnants should be planned accordingly.

Purpose The identification of urachal remnants is occurring more in infancy. Despite evidence that nonoperative management is effective, operative management remains common and has a high complication rate. We sought to determine if the complication rate after urachal resection is associated with age. Methods Patients undergoing urachal remnant resection were identified from ACS NSQIP Pediatric from 2013 to 2017. Exclusion criteria included emergent operations, contaminated wounds, and any additional procedures. Patients were compared based on complication rates, need for reoperation or readmission, and length of stay. Results A complication occurred in 16 of 476 patients (3.3%), 6 (1.3%) had reoperation, and 11 (2.3%) were readmitted. The median age for patients requiring reoperation was lower (0.1 years) than those not (1.3 years; p = 0.004). The median age of those readmitted was lower (0.4 years) than those not (1.4 years, p = 0.03), and a weak trend of longer length of stay in younger patients was identified (ρ = − 0.16, p Conclusions Operative management of younger patients resulted in greater risk of reoperation, readmission, and longer length of stay. Given that nonoperative management is effective, it may be of benefit to delay resection of urachal remnants to after 1 year of age. Study type Treatment study. Level of evidence Level III.

A patent urachus is a rare congenital anomaly that atypically presents as an umbilical cord cyst or large umbilical cord. Here we describe a case of a giant umbilical cord cyst in a newborn diagnosed as a patent urachus.A male infant with a birth weight of 3260 g was transferred because of an antenatally diagnosed giant umbilical cord cyst accompanied by yellowish discharge and granulation in the umbilical cord after birth.Patent urachus.The patent urachus was treated by excision of the urachal remnant followed by partial cystectomy.Postoperative orchitis with pyocele occurred and was treated with a course of antimicrobial therapy; and no other complications developed.Newborns with a giant umbilical cord or umbilical cord cysts should be examined for possible accompanying urachal anomalies, even if antenatal ultrasound shows no other suspicious findings, to prevent delayed diagnosis and subsequent complications.

Urachal patency is an umbilical disease in newborn foals. Etiologically, either the closure of the urachus remains absent after birth (persistent urachus) or the urachus was already closed and reopens at a later time (patent urachus). The most common causes of patent urachus are congenital defects, tearing of the navel above the predilection site, trauma, increased intravesical or abdominal pressure and umbilical infections. Patent urachus occurs more frequently in premature or weak and immunocompromised foals. Colts are more often affected than fillies. Typical clinical signs are apparent immediately after birth (persistent urachus) or 7-14 days postpartum (patent urachus). These include either dripping or a stream of urine through umbilicus during micturition as well as a moist umbilical area. The diagnosis is usually unambiguous. Typical laboratory findings are not described for patent urachus. Conservative treatment such as obliteration, cauterization or cryosurgery may be employed in cases lacking an inflammation of the urachus or other umbilical structures and when the urachal lumen measures less than 6 mm. Surgical treatment is indicated in foals with large urachal lumen, an umbilical infection or sepsis as well as when no therapeutic success is attained after a conservative treatment of 5-7 days. In most cases, prognosis is good, however complications such as umbilical inflammation, disseminating infections, and secondary joint diseases may significantly impair prognosis.Die Urachusfistel ist eine Nabelerkrankung des neugeborenen Fohlens. Ätiologisch bleibt entweder der Schluss des Urachus nach der Geburt aus (Urachus persistens) oder der Urachus war bereits geschlossen und öffnet sich zu einem späteren Zeitpunkt wieder (Urachus patens). Häufige Ursachen für eine Urachusfistel sind kongenitale Defekte, das Abreißen des Nabelstrangs oberhalb der Prädilektionsstelle, Traumata, erhöhter intravesikaler oder abdominaler Druck und Nabelinfektionen. Die Urachusfistel wird des Öfteren bei prämaturen oder lebens- und immunschwachen Fohlen beobachtet. Hengstfohlen sind häufiger betroffen als Stutfohlen. Erkrankte Fohlen zeigen entweder direkt nach der Geburt (Urachus persistens) oder meist 7–14 Tage post natum (Urachus patens) typische klinische Symptome wie Harnträufeln während der natürlichen Miktion oder Harnabsatz im Strahl über den Hautnabel sowie eine feuchte Nabelumgebung. Die Diagnose lässt sich i. d. R. eindeutig stellen. Typische labordiagnostische Befunde sind für die Urachusfistel nicht beschrieben. Konservative Therapieversuche mittels Verödung, Kauterisation oder Kryochirurgie können erfolgen, solange keine entzündlichen Veränderungen des Urachus und der übrigen Nabelstrukturen vorliegen und das Urachuslumen bis zu 6 mm beträgt. Eine Nabelresektion ist dann indiziert, wenn ein sehr weites Urachuslumen, eine begleitende Nabelinfektion oder eine Sepsis vorliegt oder wenn sich nach 5–7 Tagen konservativer Behandlungsdauer kein Therapieerfolg einstellt. In den meisten Fällen besteht eine gute Prognose, doch können Komplikationen wie Nabelentzündungen, streuende Infektionen und sekundäre Gelenkerkrankungen die Prognose erheblich verschlechtern.

Introduction: Urachal remnants are relatively rare. Generally, urachal remnants are detected in young people, and the removed specimen is small, comprising a good indication for laparoscopic surgery. Laparoendoscopic single-site surgery (LESS) for urachal remnants is considered to be safe and result in an excellent cosmetic outcome. Therefore, we report our single-center experience with LESS for urachal remnants. Methods: We retrospectively reviewed 30 patients with urachal remnants who underwent LESS from January 2011 to December 2017. The patients’ characteristics, surgical data, postoperative pain, and cosmetic assessment results were retrospectively collected and analyzed. Results: Mean total operative time was 151 min, mean pneumoperitoneal surgery time was 83 min, and mean estimated blood loss was 5.0 mL. All patients were started on an oral diet and began ambulating on postoperative day 1. Mean hospital stay was 5.5 days. LESS was completed successfully in all patients, with no conversion to conventional or open surgery. Conclusions: LESS is a viable option for the surgical treatment of urachal remnants. This technique may result in less pain than conventional techniques. Further accumulation of surgical outcomes (especially regarding safety and cosmesis) is required for LESS to become an established treatment for urachal remnants.

Uroperitoneum is a typical disease in newborn foals. An accumulation of urine develops in the abdominal cavity in consequence to a congenital or acquired leakage in the urinary tract. Colts are more frequently affected than fillies. The most common cause of uroperitoneum is a rupture of the dorsal urinary bladder wall. The urinary bladder is affected in 73.1 %, the urachus in 21.6 % and the ureter in 5.2 % of cases. Typical clinical signs occur 2-5 days postpartum, and encompass reduced general condition, abdominal distention, mild colic symptoms and unphysiologic micturition. Ultrasound examination in conjunction with abdominocentesis is considered as the gold standard of diagnostics. Typical laboratory findings are azotemia, metabolic acidosis as well as electrolyte imbalances, particularly hyperkalemia, hyponatriemia and hypochloridemia. Surgical treatment is the only reasonable therapy. Preoperative metabolic disorders and electrolyte abnormalities should be corrected prior to surgery. Especially hyperkalemia leads to a high potential for complications during general anesthesia. Following effective perioperative stabilization, the presence of a defect in the bladder wall or the urachus carries a good prognosis. In total, 63.6 % of the affected foals are healed. Most common complication is a recurrence of uroperitoneum caused by disruption of the suture line or an incomplete closure of the defect.Das Uroperitoneum ist eine typische Erkrankung des neugeborenen Fohlens. Durch einen kongenitalen oder erworbenen Defekt im Harntrakt kommt es zur Ansammlung von Urin in der Bauchhöhle. Männliche Tiere sind öfter betroffen als weibliche. Am häufigsten entwickelt sich ein Uroperitoneum durch eine Zusammenhangstrennung in der dorsalen Harnblasenwand. Die Harnblase ist in 73,1 %, der Urachus in 21,6 % und die Ureteren in 5,2 % der Fälle betroffen. Erkrankte Fohlen zeigen meist 2–5 Tage post natum typische klinische Symptome wie reduziertes Allgemeinbefinden, umfangsvermehrtes Abdomen, milde Koliksymptome und unphysiologisches Harnabsatzverhalten. Die sonografische Untersuchung in Verbindung mit der Abdominozentese gilt als Goldstandard der Diagnostik. Typische labordiagnostische Befunde sind Azotämie, metabolische Azidose und Elektrolytabweichungen, insbesondere Hyperkaliämie, Hyponatriämie und Hypochloridämie. Die chirurgische Versorgung des Defekts stellt die einzige adäquate Behandlung dar. Vor dem chirurgischen Eingriff sollten stets die metabolische Entgleisung und die Verschiebungen im Elektrolythaushalt korrigiert werden, da vor allem die Hyperkaliämie das Komplikationspotenzial der Anästhesie erhöht. Bei guter perioperativer Stabilisierung und einem Defekt an der Blase oder am Urachus ist die Prognose gut. Insgesamt werden 63,6 % der betroffenen Fohlen geheilt. Zu den häufigsten Komplikationen gehören Rezidive durch Nahtdehiszenz oder einen inkompletten Verschluss der Defekte.

Urachal cancer is one of the rarest and aggressive malignant diseases of the bladder; its incidence was 0.2% of all bladder cancers in 2012. It occurs in advanced stages, among stage IV tumors 58% die at 22 months. It is originated from urachal, an embryological structure that is present in 32% of the adult population.To present a case series of urachal cancer in a third level medical facility.Case series. Clinical data from patients with of urachal cancer during 2011 to 2019 were analyzed. The variables included were age, gender, smoking, clinical stage, treatment and evolution. Descriptive statistics, measures of central tendency and dispersion were used.There were seven patients; three men and four women; the mean age was 54.4 years. The most frequent histological type was mucinous adenocarcinoma. Partial cystectomy was performed with bilateral pelvic lymphadenectomy in three (42.8%) patients, radical cystectomy plus bilateral pelvic lymphadenectomy in the remaining four (57.2% patients).Urachal cancer is rare, clinical manifestations occur late. Hematuria is the most frequent clinical sign. The medium term prognosis is bad.El cáncer de uraco es una de las enfermedades malignas de la vejiga más raras y agresivas. Su incidencia en 2012 era del 0.2% de todos los cánceres de vejiga. Se presenta en estadios avanzados, con una mortalidad del 58% a 22 meses para el estadio IV. Se origina a partir del uraco, una estructura embriológica que está presente en el 32% de la población adulta.Presentar las características clínicas de una serie de casos de cáncer de uraco en una unidad de tercer nivel de atención médica.Serie de casos. Se analizaron los resultados clínicos obtenidos de pacientes con cáncer de uraco de 2011 a 2019. Las variables incluidas fueron edad, sexo, tabaquismo, estadio clínico, tratamiento y evolución. Se utilizó estadística descriptiva, con medidas de tendencia central y dispersión.Fueron siete pacientes, tres hombres y cuatro mujeres, con una edad media de 54.4 años. El tipo histológico más frecuente fue adenocarcinoma tipo mucinoso. Se realizó cistectomía parcial con linfadenectomía pélvica bilateral a tres pacientes (42.8%), y cistectomía radical más linfadenectomía pélvica bilateral en los otros cuatro (57.2%).El cáncer de uraco es una enfermedad rara. Sus manifestaciones son tardías y el signo clínico principal es la hematuria. El pronóstico es malo a mediano plazo.

Introduction: The urachus is an embryologic remnant which is formed from the obliteration of the allantois. Urachal abnormalities are caused when defective obliteration of the urachus happens. They are an infrequent condition. Incidence is estimated to be between 5,000 and 8,000 live births. Its diagnosis and management remain a challenge due to the lack of an specific clinical picture and the controversy about the management. Objective: The objective of this study is to assess the clinical presentation, diagnosis, therapeutic management, and outcomes of urachal anomalies in our health area. Materials and Methods: We performed a retrospective review of all cases of urachal anomalies recorded Tenerife (southern health area), La Gomera, and El Hierro Islands during a 5 year period. Results: Twenty-three cases of urachal pathology were included. The mean age of presentation was 32 years old. 73.9% were male. 65% were diagnosed in adults. In 30.3% of the cases, it was a casual finding. Symptoms included fever, umbilical exudate, hematuria, abdominal pain, and umbilical granuloma. The main diagnostic tests were ultrasound and computed tomography scan. Treatments were selected conservative management (43.5%), selective resection, partial cystectomy, and radical cystectomy. All patients had a good evolution. However, 2 cases where benign tumors were suspected, clinically, had a final histology of cancer in the specimen. Conclusions: Due to the lack of a specific clinical picture and undefined findings in image tools, diagnosis is difficult and it may be inaccurate. Despite more data are needed, our results suggest that the systematic excision of urachal lesions could result in safer outcomes since cases where a benign lesion is clinically suspected might result in malignant tumors.

Urachal anomalies are rare in the adult population and when diagnosed, are typically malignant. Herein, we report a case of a 61-year-old male who underwent an excision of a urachal mass for a presumed malignancy. Pathologic evaluation demonstrated a neurofibroma. Neurofibromas are benign peripheral nerve sheath tumors that mostly appear as localized skin tumors and rarely involve the genitourinary system. Neoplastic transformation of neurofibromas is rare but not unheard. To our knowledge, this is the first description of a neurofibroma originating from the urachus.

To evaluate the surgical feasibility and postoperative cosmesis of a novel transumbilical laparoendoscopic single-site (LESS) surgical approach involving Y-shaped incisions and three-flap umbilicoplasty in urachal remnant patients. Patients with symptomatic urachal remnants who underwent either conventional laparoscopic surgery (CL group, n = 21) or LESS surgery with Y-shaped incisions and three-flap umbilicoplasty (LESS group, n = 36) between May 2010 and September 2019 were retrospectively assessed. Perioperative factors and postoperative esthetic outcomes were compared between the groups using univariate and multivariate analyses. Esthetic outcomes were assessed using the body image questionnaire consisting of the body image scale (BIS) and the cosmetic scale (CS); a higher score indicated a better outcome. The median operative time was greater and the insufflation time was shorter in the LESS group than in the CL group. The estimated blood loss and postoperative hospital stay and surgical site infection rate did not differ significantly between the groups. While the BIS score also did not differ significantly between the groups, the CS score was greater in the LESS group than in the CL group. Multivariate analysis revealed that the surgery type (LESS surgery) was an independent predictor of greater postoperative esthetic satisfaction. Transumbilical LESS surgery with Y-shaped incisions and subsequent three-flap umbilicoplasty is feasible in patients with symptomatic urachal remnants. Regarding postoperative cosmesis, the higher CS score suggests that this technique is superior to CL surgery. Furthermore, the selection of this procedure was an independent predictor of good postoperative esthetic outcomes.

Background This study aimed to evaluate the diagnosis and treatment of pediatric patients with urachal remnants. Methods Medical records of patients diagnosed with urachal remnants between 2015-2019 were evaluated retrospectively. Age, gender, admission complaints, diagnosis, size of cysts, the complications that developed, and treatment modalities were recorded. Results The study population consisted of 15 cases including seven girls. Most of the cases were asymptomatic. The urachal remnants were detected by ultrasonography. The most common symptom in symptomatic patients was abdominal pain and umbilical discharge. Three patients required surgery and most of them were conservatively followed up. Complication, infection, and bladder rupture were observed in one case. Conclusions Follow-up of patients with urachal remnants can be performed conservatively. The type of complaint and the presence of clinical and radiological regression (if any) are important for deciding the treatment during the follow-up. However, conservative follow-up is more prominent today, as shown in this study.

We relied on the most contemporary Surveillance, Epidemiology, and End Results (SEER) database and tested the hypothesis that chemotherapy may improve survival in metastatic urachal carcinoma (m-UraC).Within the SEER database (2004-2016), we identified m-UraC patients aged ≥ 18 years. Propensity score matching (PSM: cystectomy status, age and sex), Kaplan-Meier plots, cumulative incidence plots, Cox regression models and competing risks regression (CRR) models addressed overall mortality (OM) and cancer-specific mortality (CSM).Overall, 274 m-UraC patients were identified with a median age of 70 years. Most were male (66%) and Caucasian (72%). Overall, 32% received chemotherapy. Chemotherapy-exposed patients were younger (62 vs. 73 years, p0.001) and more frequently underwent cystectomy (19 vs. 8%, P = 0.014). In 274 m-UraC patients, median OM and CSM were 6 (4 -10) months and 8 (6 -14) months, respectively. After 1:1 PSM, chemotherapy-exposed patients exhibited lower OM (median 16 vs. 3 months; multivariable HR 0.38, P0.001) and lower CSM (median 17 vs. 4 months; multivariable CRR HR 0.52, P = 0.001). The association between chemotherapy and better survival was even stronger in younger (≤70 years) patients (OM HR: 0.23, P0.001; CSM CRR HR: 0.42, P = 0.001), but not in older (≥71 years) patients (OM HR: 0.61, P = 0.2; CSM CRR HR: 1.02, P = 1), after PSM and multivariable adjustments.Overall, we validated the very aggressive nature of UraC, when distant metastases are present, and observed that m-UraC patients exposed to chemotherapy exhibited lower OM and CSM.

Combination of posterior urethral valves and urachus remnants has been described as rare occurrence. We report a case of a baby boy with normal antenatal scans, in whom the presence of large urachal cyst and posterior urethral valves causing high pressure bladder and chronic kidney disease was found. The patient underwent ablation of posterior urethral valves at 23 days of life and urachal cyst removal at age of 4 and a half months.

Background Umbilical remnant infection and patent urachus are an important cause of morbidity for the equine neonate. Left untreated, fatal complications can develop. Identifying risk factors for post-operative complications after surgery are important for estimating prognosis. Objectives To identify risk factors associated with post-operative complications in foals undergoing umbilical remnant resection due to patent and/or infected umbilical remnants. Study design Retrospective case series. Methods Foals undergoing umbilical remnant resection due to patent urachus or infected remnants at the Marion duPont Scott Equine Medical Center from 2004 to 2016 were included in the study. Data were obtained from medical records and associations between outcomes and post-operative complications, and pre or intraoperative clinical variables were assessed using bivariable and multivariable analyses. Results Eighty-two foals underwent umbilical remnant resection of which 73 (89.0%) survived to discharge. The urachus was the most commonly affected structure being patent and/or infected in 84.1% of cases. Concurrent diseases were present in 60.6% of foals prior to surgery, with diarrhoea and septic arthritis being most common. The presence of preoperative septic arthritis and/or physitis were significantly associated with nonsurvival (OR 33; 95% confidence interval 1.1-985.2; P = 0.04). Longer anaesthesia time (OR 1.4; 95% confidence interval 1.1-1.7; P = 0.02) and failure of passive transfer of immunoglobulins (OR 5.9; 95% confidence interval 1.2-29.04; P = 0.03) were associated with increased odds for post-operative complications. Main limitations It is not known if medical treatment alone would have been successful in foals that did not receive preoperative medical treatment. Conclusions Overall survival is high after surgical excision of umbilical remnants. The presence of preoperative septic arthritis and/or physitis was associated with decreased survival and failure of passive transfer of immunoglobulins and longer anaesthesia times were associated with increased odds for post-operative complications.

BackgroundThe prognostic value of the primary neoplasm responsible for pseudomyxoma peritonei (PMP) remains poorly studied. The aim of this study was to determine the prognosis for patients with extra-appendicular PMP (EA-PMP) treated optimally with complete cytoreductive surgery (CCRS) and hyperthermic intraperitoneal chemotherapy (HIPEC).MethodsAll patients treated for PMP with CCRS and HIPEC between 1994 and 2016 were selected retrospectively from a French multicentre database. Patients with EA-PMP had pathologically confirmed non-neoplastic appendices and were matched in a 1 : 4 ratio with patients treated for appendicular PMP (A-PMP), based on a propensity score.ResultsSome 726 patients were identified, of which 61 (EA-PMP group) were matched with 244 patients (A-PMP group). The origins of primary tumours in the EA-PMP group included the ovary (45 patients), colon (4), urachus (4), small bowel (1), pancreas (1) and unknown (6). The median peritoneal carcinomatosis index was comparable in EA-PMP and A-PMP groups (15·5 versus 18 respectively; P = 0·315). In-hospital mortality (3 versus 2·9 per cent; P = 1·000) and major morbidity 26 versus 25·0 per cent; P = 0·869) were also similar between the two groups. Median follow-up was 66·9 months. The 5-year overall survival rate was 87·8 (95 per cent c.i. 83·2 to 92·5) per cent in the A-PMP group and 87 (77 to 96) per cent in the EA-PMP group. The 5-year disease-free survival rate was 66·0 (58·7 to 73·4) per cent and 70 (53 to 83) per cent respectively.ConclusionOverall and disease-free survival following treatment with CCRS and HIPEC is similar in patients with pseudomyxoma peritonei of appendicular or extra-appendicular origin.

The urachus is a fibrotic remnant of the allantois, a canal that collects liquid waste and exchanges gases from the fetal bladder with the umbilical cord. Urachal cancer is a rare and aggressive cancer that often presents as adenocarcinoma at the dome of the bladder.If urachal carcinoma is found early and confined to the urachus, cystectomy with en bloc resection of the urachal ligament and umbilicus can be curative. For unoperable patients, chemotherapy has a low rate of efficacy.In this case we explore the effects of Gem-FLiP (gemcitabine, 5-fluorouracil, leucovorin, and cisplatin) in the treatment of urachal carcinoma, a regimen that is undergoing clinical trials. A 76-year-old man with a long history of genitourinary disease presented with gross painless hematuria. A flexible cystoscopy showed a sessile tumor at the dome of the bladder. A computed tomography (CT) urogram of the abdomen and pelvis confirmed a mass at the dome of the bladder. A CT-guided biopsy revealed adenocarcinoma consistent with urachal cancer.After beginning treatment with Gem-FLiP, he developed pedal edema as well as drainage of the umbilicus. A CT scan of the abdomen and pelvis showed an anterior extension through the abdominal wall just below the umbilicus with central hypoattenuation within the mass; this represented a fistula between the bladder and umbilicus, along the tract of the urachal ligament, therefore confirming the diagnosis of urachal cancer.This case illustrates a clinical scenario in which urachal carcinoma was confirmed when the entire cancerous urachus fistulized in response to treatment with systemic chemotherapy. It is highly unusual that a response to chemotherapy confirms a diagnosis.

Symptomatic urachal remnants are rare benign anomalies affecting young adults. For these patients, both curative treatment and acceptable postoperative aesthetic outcomes are important. Here, we describe a novel transumbilical approach, using Y-shaped incisions and three-flap umbilicoplasty techniques, to improve repair and aesthetic outcomes. The clinical and perioperative data of 16 patients (11 men; median age, 25 years; range 16–46 years) who underwent LESS surgery and three-flap umbilicoplasty (LESS group) between December 2013 and March 2017 were analyzed. For comparison, the perioperative data of those who underwent conventional laparoscopic surgery (CL group) between May 2010 and November 2013 at the same institutions were investigated. The median operative time of the LESS group was longer than that of the CL group (146.5 vs 107 min; p = 0.009). The estimated blood loss and postoperative hospital stay were not significantly different between the two groups. The two perioperative complications were injury to the transverse colon serosa and minor surgical site infection. Both complications were resolved with appropriate management. This novel umbilical approach is useful for patients with symptomatic urachal remnants, with the reduction in port-site scarring resulting in good postoperative cosmesis.

Urinary bladder hemangiomas are rare, especially in children and adolescents. We present a case of a 17-year-old young man with persistent gross hematuria for 1 month. Computed tomography revealed a 3.6 cm mass on the superior anterior wall of the urinary bladder, which was highly suspected as an urachal tumor. We carried out an en bloc resection of the urachus and bladder tumor. The pathologic report indicated a cavernous hemangioma of the urinary bladder. No tumor recurrence or bleeding was found during the 2-year follow-up. Urinary bladder hemangioma is an important differential diagnosis in young patients with hematuria.

Introduction: Urachal cyst is an exceptionally rare disease in children caused by the incomplete obliteration of the urachal remnant. Urachal cysts seldom cause symptoms unless a secondary infection occurs. The symptoms of an infected urachal cyst are nonspecific and may be similar to acute appendicitis or other acute abdominal conditions. However, complications attributable to a delayed diagnosis can endanger the life of a patient. Patient concerns: A 5-year-old boy presented with a 3-day history of severe intermittent lower abdominal pain. Diagnosis: Infected urachal cyst. Interventions: The patient was treated with surgical resection of the urachus, followed by intravenous antibiotics during the hospitalization. Outcomes: The patient was discharged without incident 7 days after the operation. With his follow-up in our out-patient department, he recovered well without any sequelae in the 6 months post-surgery. Conclusion: We suggested using the abdominal echo scan to differentiate the urachal cyst because of its high sensitivity and nonradioactive characteristic, and computed tomography is a typical diagnostic tool for urachal cysts. The mainstream management of an infected urachal cyst remains surgical excision. Complete excision of urachal cysts is relatively easy in a pediatric patient and the risk of subsequent infection is low; however, patients tend to have a low, although possible, risk of potential malignant transformation over their lifetimes.

Objective To determine whether surgical removal of urachal anomalies improves the outcomes of dogs with recurrent lower urinary tract disease (LUTD) and bacterial urinary tract infection (BUTI). Study design Retrospective study. Animals Thirty-three dogs with urachal anomalies and recurrent LUTD or BUTI. Methods Medical records of dogs with LUTD or BUTI and a diagnosis of urachal anomaly treated by partial cystectomy were reviewed. A minimum follow-up of 9 months was required for inclusion. Results Median age at onset of clinical signs was 12 months (range, 1 month to 10 years). Urachal anomalies were detected with histopathology in 20 of 28 (71%) dogs. At a median follow-up of 22 months (range, 9-114), 21 of 28 (64%) dogs were free of signs of LUTD. Nine (27%) dogs exhibited reduced signs of LUTD; in three (9%) dogs, no clinical improvement was observed. Among the 25 dogs with confirmed preoperative BUTI, 22 clinically improved with surgery. Conclusion Partial cystectomy reduced the long-term severity of clinical signs and risk of recurrence of LUTD or BUTI in dogs with confirmed or suspected urachal anomalies. Clinical significance Partial cystectomy should be considered as an adjunct to the treatment of LUTD and BUTI in dogs.

Diverticular disease is a common clinical condition among Western populations, which increases with age. It can present in a variety of manners and has myriad of potential disease complications. We present a rare case of an adult patient with an extraperitoneal complications of a diverticular perforation presenting with facial swelling due to a colo-urachal fistula associated with a patent urachal remnant. Perforation should be considered in patients presenting with surgical emphysema with background of diverticular disease.

Urachal remnants (UR) represent a failure in the obliteration of the allantois, which connects the bladder to the umbilicus, at birth. Surgical management of UR in children is controversial. The traditional surgical approach involves a semicircular intraumbilical incision or a lower midline laparotomy. Recently, many reports have supported the laparoscopic approach (LA) for removing UR. However, there is a paucity of data comparing the benefits of LA those of the open approach (OA). We retrospectively reviewed all children (aged ≤16 years) with UR who underwent surgical procedures. Age at surgery, sex, operative time, intraoperative or postoperative complications, total wound length, and length of hospital stay length after operation were analyzed. Overall, 30 children aged between 9 months and 16 years (mean 9.0 years) underwent surgical procedures: 15 were treated by OA and 15 were treated by LA. The only statistically significant variable was the operative time. Furthermore, we reanalyzed the age distributions of the older children (aged ≥10 years). In this group, no significant difference in the operative time between OA and LA was observed; however, there was a statistically significant difference in the total wound length. Our review indicated that LA required longer operative time than OA without any cosmetic advantage. However, in older children (aged ≥10 years), the difference in the operative time was not significant; moreover, LA provided greater cosmetic advantage. LA is recommended for older children (aged ≥10 years) because of its cosmetic advantage.

Objectives To identify the clinicopathological features of malignant urachal tumor in Japan, and analyze the 5-year overall survival of malignant urachal tumor patients. Methods We used the hospital-based cancer registry data to extract malignant urachal tumor cases that were diagnosed in 2008-2009 and 2012-2015, histologically confirmed, and received the first course of treatment. We analyzed the 5-year overall survival using the 2008-2009 cohort's data. Results We identified 456 patients, and malignant urachal tumor accounted for 0.4% of all malignant bladder cancers. The median age was 61 years (range 2-97), and 66% were men. The most common histology was adenocarcinoma (80%), followed by urothelial carcinoma (11%) and squamous cell carcinoma (3%). The proportions of patients were: 19% Ta/Tis/T1N0M0, 55% T2-3N0M0, 13% T4/N+ and 13% M+. Regarding the initial treatment, the proportions of surgery alone were 79% and 33% in cases of T3 or less N0M0 and T4/N+, respectively. The proportion of combination therapy including surgery and chemotherapy were 13% and 44% in T2-3N0M0 and T4/N+, respectively. Radiation therapy was not common at any stage. In the 2008-2009 cohort, the 5-year overall survival rate in Ta/Tis/T1N0M0, T2-3N0M0, T4/N+ and M+ were 60%, 64%, 63% and 12%, respectively. Conclusions Malignant urachal tumors are quite rare in Japan, and most of those without metastasis are likely to be treated by surgery alone, even at advanced stages. A standard of care must be established for malignant urachal tumor patients at advanced stages or with metastasis, as the prognosis of these patients can be poor.

Background Ex vivo drug screening refers to the out-of-body assessment of drug efficacy in patient derived vital tumor cells. The purpose of these methods is to enable functional testing of patient specific efficacy of anti-cancer therapeutics and personalized treatment strategies. Such approaches could prove powerful especially in context of rare cancers for which demonstration of novel therapies is difficult due to the low numbers of patients. Here, we report comparison of different ex vivo drug screening methods in a metastatic urachal adenocarcinoma, a rare and aggressive non-urothelial bladder malignancy that arises from the remnant embryologic urachus in adults. Methods To compare the feasibility and results obtained with alternative ex vivo drug screening techniques, we used three different approaches; enzymatic cell viability assay of 2D cell cultures and image-based cytometry of 2D and 3D cell cultures in parallel. Vital tumor cells isolated from a biopsy obtained in context of a surgical debulking procedure were used for screening of 1160 drugs with the aim to evaluate patterns of efficacy in the urachal cancer cells. Results Dose response data from the enzymatic cell viability assay and the image-based assay of 2D cell cultures showed the best consistency. With 3D cell culture conditions, the proliferation rate of the tumor cells was slower and potency of several drugs was reduced even following growth rate normalization of the responses. MEK, mTOR, and MET inhibitors were identified as the most cytotoxic targeted drugs. Secondary validation analyses confirmed the efficacy of these drugs also with the new human urachal adenocarcinoma cell line (MISB18) established from the patient’s tumor. Conclusions All the tested ex vivo drug screening methods captured the patient’s tumor cells’ sensitivity to drugs that could be associated with the oncogenic KRASG12V mutation found in the patient’s tumor cells. Specific drug classes however resulted in differential dose response profiles dependent on the used cell culture method indicating that the choice of assay could bias results from ex vivo drug screening assays for selected drug classes.

Introduction: Urachal remnant with heterotopic sinus is an extremely rare congenital anomaly, and usually coexists with other congenital anomalies. We report the case of a 32-year-old adult male with urachal remnant with heterotopic sinus. Patient concerns: A 32-year-old adult male presented with purulent secretion in the heterotopic sinus on the dorsal side of the normal external urethral orifice and pain in the balanus since 5 months. Diagnosis: The computed tomography scan demonstrated a 4 cm cystic mass next to the anterior wall of the urinary bladder. Retrograde urethrography was performed, which demonstrated that this mass communicated with the heterotopic sinus on the dorsal side of the normal external urethral orifice. Cystoscopy showed that there was no communication between the mass and the bladder. Pathology results confirmed that this mass was urachal tissue. Interventions: The patient underwent a laparoscopy surgery to undertake the cystic mass, part of the anterior wall of urinary bladder and the epithelium of channel which communicated with the cystic mass. Outcomes: The patient was discharged without any complications after 6 months and follow-up was continued in the clinic. Conclusions: Urachal remnant with heterotopic sinus is rare, and we recommend that urachal remnant should be considered when a patient presents with a mass in the retropubic space.

Immunoglobulin G4 related disease (IgG4-RD) is a fibro-inflammatory disease of unknown etiology, characterized by lesions in the form of tumors, elevated serum IgG4 levels, plasma cells with significant IgG4 infiltration, accompanied by phlebitis obliterans and fibrosis. This disease usually has multiorgan disease, including pancreas, biliary tract, salivary glands, peri orbital tissues, kidneys, lungs, lymph nodes and retro peritoneum. IgG4-RD mainly affects men with a predominance of age by young adults until old age. The clinical manifestations of IgG4-RD, depend mainly on the organs affected and the response to steroids. His forecast is not yet clear. Within the affected urogenital organs can be observed kidney, retroperitoneum, ureter, bladder, urachus, testis/epididymis, paratesticular region, prostate and urethra.La enfermedad relacionada con la inmunoglobulina G4 (ER-IgG4) es una enfermedad fibroinflamatoria de etiología desconocida, la cual se caracteriza por presentar lesiones en forma de tumoraciones, concentraciones séricas aumentadas de IgG4 y células plasmáticas con una infiltración importante de IgG4, junto con flebitis obliterante y fibrosis. Esta enfermedad suele tener afección multiorgánica, incluyendo el páncreas, el tracto biliar, las glándulas salivares, los tejidos periorbitarios, los riñones, los pulmones, los ganglios linfáticos y el retroperitoneo. La ER-IgG4 afecta principalmente a hombres, con un predominio de edad por los adultos jóvenes y hasta la vejez. Las manifestaciones clínicas de la ER-IgG4 dependen principalmente de los órganos afectados y de la respuesta a los esteroides. Su pronóstico aún no es del todo claro. Dentro de los órganos urogenitales afectados pueden incluirse el riñón, el retroperitoneo, el uréter, la vejiga, el uraco, el testículo/epidídimo, la región paratesticular, la próstata y la uretra.

The aim of this study was to evaluate and compare the cosmetic outcomes of laparoscopic single-site surgery (LESS) and conventional laparoscopy (CL) in the treatment of ureteropelvic junction obstruction (UPJO) and urachal remnant removal. LESS is thought to produce better cosmetic results than CL; however, patients' perception of their scars has not been assessed. This study compared the subjective body image and cosmesis ratings of patients who had undergone LESS or CL for UPJO and urachal remnant removal.Fifty patients who underwent LESS or CL for UPJO or urachal remnant removal between June 2008 and June 2015 were included. Cosmetic outcomes were evaluated using the Body Image Questionnaire (BIQ) and Photo-Series Questionnaire (PSQ).The body image and cosmetic scores were significantly higher for patients who underwent LESS than for those who underwent CL, for both pyeloplasty and urachal remnant removal (p .05 each). When performed for either a pyeloplasty or urachal remnant removal, significantly greater BIQ and PSQ scores were observed in females after LESS compared to CL, but not in males.LESS for UPJO and urachal remnant removal has better self-reported body image and cosmesis ratings than CL. This trend is particularly strong in female patients.

Between 2012 and 2014, nine consecutive patients with symptomatic urachal remnants were treated at Roskilde Hospital, Denmark, with a robot-assisted laparoscopic en bloc resection of their urachal remnants with partial cystectomy and umbilectomy with primary umbilicoplasty. The objective of this study is to report surgical results and give recommendations for the management of urachal remnants.The patient group consisted of four women and five men with a median age of 59 years. Indications for surgery included bothersome umbilical secretion in two patients, and suspicion of malignancy in seven patients with unexplained haematuria. Three patients also suffered from recurrent urinary tract infections. Urachal remnants were confirmed on computed tomography scans in all patients. The median surgical time was 120 min with no significant intraoperative problems.The surgical approach led to cosmetically satisfactory results in all patients. However, three patients suffered subsequent rupture of the fascia and one required an exploratory laparotomy to secure haemostasis of a bleeding spleen. Histology revealed a well-differentiated adenocarcinoma with negative surgical margins in one patient while no signs of malignancy were observed in the remaining eight patients. Umbilical secretion, haematuria and urinary infections subsided in all patients after the surgery.Based on these findings, it would be reasonable to offer surgery to patients with symptomatic urachal remnants. However, one should be aware that there is a significant risk of complications and that the risk of cancer seems to be limited.

// Orsolya Modos 1 , Henning Reis 2 , Christian Niedworok 3 , Herbert Rubben 3 , Attila Szendroi 1 , Marcell A. Szasz 4 , Jozsef Timar 4 , Kornelia Baghy 5 , Ilona Kovalszky 5 , Tomasz Golabek 6 , Piotr Chlosta 6 , Krzysztof Okon 7 , Benoit Peyronnet 8 , Romain Mathieu 8 , Shahrokh F. Shariat 9 , Peter Hollosi 5,10 , Peter Nyirady 1 and Tibor Szarvas 1,3 1 Department of Urology, Semmelweis University, Budapest, Hungary 2 Institute of Pathology, University of Duisburg-Essen, Essen, Germany 3 Department of Urology, University of Duisburg-Essen, Essen, Germany 4 2nd Department of Pathology, Semmelweis University, Budapest, Hungary 5 1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary 6 Department of Urology, Jagiellonian University, Krakow, Poland 7 Department of Pathomorphology, Jagiellonian University, Krakow, Poland 8 Department of Urology, Rennes University Hospital, Rennes, France 9 Department of Urology, Medical University of Vienna, Vienna General Hospital, Vienna, Austria 10 Tumor Progression Research Group, Hungarian Academy of Sciences, Budapest, Hungary Correspondence to: Tibor Szarvas, email: // Keywords : urachal carcinoma, urachal cancer, urachus, mutation, EGFR, Pathology Section Received : April 08, 2016 Accepted : May 29, 2016 Published : June 05, 2016 Abstract Purpose: Targeted therapy represents an attractive alternative for rare tumors such as urachal carcinoma (UrC). The aim of this study was to assess the mutations of the most commonly affected 5 genes in the targetable EGFR-pathway in UrC and comapre their frequencies to those of found in urothelial and colorectal cancer. Materials and Methods: Mutational hot-spots of selected genes were tested in 22 UrC samples by pyrosequencing. Mutational patterns were compared to those published for colorectal and urothelial cancers. Furthermore, we sought correlations between mutations and clinicopathological and follow-up data. Results: We found 11 mutations in 10 of 22 (45%) patients. The most frequently mutated gene was KRAS (27%) followed by BRAF (18%) and NRAS (5%), while no mutations were detected in the EGFR and PIK3CA genes. No correlation was found between the mutation status and clinicopathological parameters (Sheldon/Mayo stage, tumor grade, metastases). Furthermore, none of the mutations correlated with progression-free or overall survival. Conclusions: The mutation pattern of UrC is more similar to colorectal than to urothelial cancer. However, the mutation characteristics of UrC seems to be unique suggesting that clinical decision-making for UrC cannot be simply adopted from urothelial or colorectal carcinoma. The high occurence of EGFR-pathway mutations warrants the testing for KRAS and BRAF mutations when considering anti-EGFR therapy in UrC.

The urachus is a fibrous tube extending from the umbilicus to the anterosuperior bladder dome that usually obliterates at week 12 of gestation, becoming the median umbilical ligament. Urachal pathology occurs when there is incomplete obliteration of this channel during foetal development, resulting in the formation of a urachal cyst, patent urachus, urachal sinus or urachal diverticulum. Patients with persistent urachal remnants may be asymptomatic or present with lower abdominal or urinary tract symptoms and can develop complications. The purpose of this review is to describe imaging features of urachal remnant pathology and potential benign and malignant complications on ultrasound, CT, positron emission tomography CT and MRI.

To determine the ideal management of uncomplicated adult urachal remnants and to confirm if conservative management with repeated imaging is acceptable.This is a retrospective review of clinical notes and imaging of 45 patients with a mean age of 54.6 years who had been diagnosed with urachal anomalies at a single urology tertiary centre from January 2005 to December 2016. Patients who underwent surgical intervention and findings from patients managed non-operatively, with a mean follow up of 31 months, were evaluated.Thirty (66.7%) patients had incidental findings, while 15 (33.3%) were symptomatic. Eight underwent excision, one underwent an incision and drainage of abscess, while 34 of the 36 remaining patients elected to undergo surveillance with repeated ultrasound imaging. Two were lost to follow-up. Malignancy was confirmed in three patients. There was interval stability of the urachal remnant in all the patients in the non-operative cohort. The small sample size, limited follow-up, and retrospective nature of the study are recognised limitations.It was found that simple and asymptomatic lesions can be monitored with ultrasound, but effort must be made on initial diagnosis to ensure that malignancy is excluded using CT imaging and flexible cystoscopy where possible. Long-term follow-up of this cohort is required to assess the natural history of observed urachal anomalies.

Urachal carcinoma (UrC) is an exceedingly rare neoplasm that develops from the urachus, an embryologic remnant of the urogenital sinus and allantois. The most commonly encountered histologic subtype is adenocarcinoma. The aim of this study is to characterize a series of UrC by morphology, immunohistochemistry, and molecular analysis. We retrospectively investigated seven cases of UrCs and assessed patient symptoms, imaging, histologic features, immunohistochemical profile, molecular characteristics, pathologic stages, and type of treatment. Immunostaining for CK7, CK20, Muc-2, CDX2, GATA3, β-catenin, and CK34βE12 was carried out on each neoplasm and on seven non-neoplastic urachal remnants as the control group. Additionally, a mutational analysis was performed using the QIAact Actionable Insights Tumor Panel Kit, which analyzes KRAS, NRAS, KIT, BRAF, PDGFRA, ALK, EGFR, ERBB2, PIK3CA, ERBB3, ESR1, and RAF1. Our cohort comprised five females and two males with a mean age of 64 years. UrCs consisted of two mucinous cystadenocarcinomas and five invasive, non-cystic adenocarcinomas. Carcinoma antigen expression profile was positive for CK20 and negative for CK34βE12 and GATA3 in all cases. Five of seven cases stained positively for Muc-2 and CDX2. On the contrary, non-neoplastic urachal remnants were immunoreactive for CK34βE12, CK7, and GATA3. Mutational analysis gave a positive result in four out of seven (57.1%) cases. All four positive tumors showed RAS mutation and one an additional mutation in PIK3CA. Urachal tumors exhibit peculiar morphologic, immunohistochemical, and molecular features. Due to the advanced stage at presentation, individualized treatment should be undertaken.

Benign urachal remnants can be encountered in the adult urinary bladder and it is recognized that these can uncommonly give rise to urachal urothelial carcinoma. However, urachal remnants containing urothelial carcinoma incidentally encountered in cystectomies for bladder cancer has not been previously described. Herein, we present 8 adult bladder cancer cystectomies with incidental urachal remnants containing urothelial carcinoma. All 8 incidental urachal remnants with tumor were located at the dome that varied from small tubular to tubulocystic structures and contained urothelial carcinoma in situ (CIS) (6), noninvasive high-grade papillary urothelial carcinoma (PUC) (1), and coexistent noninvasive high-grade PUC and urothelial CIS (1). Six of the 8 urachal remnants with tumor also showed benign urothelial cells (2), mixed urothelial and glandular cells (2), and cuboidal cells (2). The bladder mucosa directly above the remnant showed urothelial CIS (4), PUC (1), concomitant PUC and urothelial CIS (1), invasive urothelial carcinoma (1), and benign urothelium (1); only 1 remnant intermingled with invasive urothelial carcinoma nests. Two remnants with tumor were at a region away from the main bladder tumor including the one overlaid by benign urothelium. The remnant with tumor extended into the upper half (5) or lower half (3) of muscularis propria (MP) and if misinterpreted as MP invasion, 5 of 8 bladder tumors will be overstaged. In conclusion, urachal remnant can have an early involvement by urothelial CIS or PUC similar in the bladder proper lumen. Urothelial carcinoma involving the urachus can be divided into a: (a) contiguous spread from a bladder urothelial carcinoma, (b) separate (noncontiguous) focus concomitant to bladder urothelial carcinoma, and (c) primary urachal urothelial carcinoma. Caution is warranted not to over interpret urachal remnant involvement by noninvasive urothelial carcinoma as an invasive tumor focus which could lead to overstaging.

Urachal neoplasms are uncommon and represent a minor portion of bladder tumors. According to the recently updated World Health Organization classification (2016), these tumors are classified as adenomas, adenocarcinomas, nonglandular neoplasms, and mixed carcinomas. The mucinous cystic neoplasms represent a small percentage of urachal tumors with morphologic spectrum ranging from benign mucinous cystadenoma to borderline mucinous cystic tumor of low malignant potential and to malignant mucinous cystadenocarcinoma. Benign urachal mucinous cystic adenomas are exceedingly rare, and only a few cases have been reported in the literature to date. The goal of this review is to summarize the clinical features, histopathologic characteristics, treatment, and prognosis of urachal mucinous cystadenoma in light of differentiating them from mucinous cystic tumor of low malignant potential and mucinous cystadenocarcinoma.

The purpose of the study was to report the outcomes of our modified techniques of laparoscopic urachal resection, followed by novel umbilical-plasty using dermal regenerative grafts for symptomatic urachal remnants. Between March 2014 and December 2016, laparoscopic urachal resection was carried out on 45 patients with complaints of urachal remnants. After extraction of the specimen and closure of the fascia at the umbilical site, a 1-cm square section of dermal regeneration sheet (TERUDERMIS®, Olympus) was suture retained between the fascia and the skin. Patient satisfaction with cosmesis was scored prospectively using the visual analogue scale (VAS). Perioperative records were reviewed to assess surgical outcomes. Laparoscopic urachal resection was successfully conducted via the transperitoneal approach. Postoperative bleeding was experienced in one patient (2.2%), which was subsequently controlled laparoscopically. Infection subsided with symptomatic relief in all patients. No symptomatic recurrences were encountered. Over 80% of patients (37/45) rated the cosmetic outcome of the plasty as good to excellent, with the median scores of 8 (range 5–10). Laparoscopic urachal resection is safe and curative with reasonable risk of complications. Novel umbilicoplasty using dermal regenerative graft is very simple technique and offers satisfactory cosmetic outcomes. Further follow-up and cosmesis evaluation are required.

A 41-year-old man with no medical history presented with 2 weeks of nausea, vomiting, a new palpable abdominal mass, constipation and a 14kgweight loss. On admission, CT abdomen and pelvis demonstrated a 6.9×3.7 cm soft-tissue abdominal mass deep to and invading the lower anterior abdominal wall with tethering of the urinary bladder and potential involvement of the urachus. Subsequently, a biopsy demonstrated a low-grade spindle cell neoplasm compatible with inflammatory myofibroblastic tumour with immunostain positive for smooth muscle actin and desmin and negative for CD21, CD117, DOG-1, TKE-1, mdm2, CD34 and ALK. One week following admission, he underwent en bloc excision of the mass including abdominal wall (umbilicus, portions of rectus sheath and muscle), bladder dome, right colon and a segment of small bowel. Final pathology of the mass confirmed an inflammatory myofibroblastic tumour, and his postoperative course was uneventful.