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Abstract: Urachal adenocarcinoma is an unusual and aggressive form of bladder cancer that arises from urachus, a midline fibrous remnant of allantois. Experience with diagnosing them is limited and differentiating urachal adenocarcinoma from other urachal pathologies like infected urachal cysts may be difficult at times. Differentials of urachal anomalies can be narrowed down by proper assessment of patient demographics, clinical details, lesion morphology, and imaging findings. With this case series of five patients of urachal adenocarcinoma, we have tried describing their clinical manifestation and imaging appearances., (Copyright © 2023 Copyright: © 2023 Journal of Cancer Research and Therapeutics.)

Background: Urachal remnants are a rare congenital defect resulting from failure of obliteration of a fibrous tube that connects the umbilicus to the bladder dome during embryological development. Oftentimes a urachal remnant will go undiagnosed, but occasionally a patient may present with a variety of symptoms, ultimately leading to the identification of the remnant. Given its rarity, there is very limited literature available on the management of symptomatic urachal remnants, especially in adults. Surgical resection has been the first-line management of urachal remnants for years, especially given the risk of the development of urachal adenocarcinoma secondary to recurrent infection, persistent irritation, and urinary stasis associated with some urachal remnants., Aim: We present our experience in the management of symptomatic urachal remnants in adults at our institute and perform a brief literature review of the same., Methods: A retrospective review of all cases who underwent surgical management of symptomatic urachal remnants between December 2015 and January 2022 was performed. Seven cases of urachal remnant excision in total were identified over the time period. Patient characteristics and perioperative parameters were analysed. Post-operative complications were measured in accordance with the Clavien-Dindo grading system., Result: In total, 7 cases of urachal remnants were treated at our institute over the study period. Four patients were treated with a TURBT and 3 patients were treated with a laparoscopic partial cystectomy. There were no intraoperative complications and one post-operative complication requiring readmission for intravenous antibiotics. There was one mortality but this was not as a direct result of the operative procedure. Mean length of stay was 1.71 days. Two of patients had histologically confirmed urachal adenocarcinoma and the remaining five patients had benign histology. Each patient was seen in the outpatients department 6 weeks post-operatively for clinical review and review of histology. No further follow-up was required for the patients with benign histology given resolution of symptoms and follow-up for the malignant histology was arranged appropriately following MDM., Conclusion: There is a paucity of data available on the management of urachal remnants in the adult population; however, an endoscopic or laparoscopic approach is a safe and effective method of excising symptomatic urachal remnants., (© 2023. The Author(s).)

Background: A urachal mass is a relatively rare presentation to the urologists' practice, often requiring radical surgical excision for a definitive diagnosis. Xanthogranulomatous inflammation of the urachus is an extremely rare entity with few cases reported worldwide, and to the best of our knowledge, no cases reported in the western world., Case Presentation: In this case, a 55-year-old male patient presented with bothersome lower urinary tract symptoms and computed tomography findings demonstrating a urachal mass that was worrisome for urachal carcinoma. Following surgical intervention, histopathology revealed urachal xanthogranuloma. Post-operatively, the patient recovered well, and eventually, he had symptomatic and radiologic improvement., Conclusion: This case brings awareness to a rare presentation of a urachal mass-urachal xanthogranuloma. While operative intervention was both diagnostic and therapeutic, we highlight the challenge in differentiating between benign and malignant processes for urachal masses. Herein, we show the importance of including urachal xanthogranuloma in the differential diagnosis of a urachal mass to prevent further morbidity associated with the treatment of this disease., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Relatively little is known about allantois and urachal development in early humans., Serial sagittal histological sections from eight human embryos and fetuses were examined to determine allantois development., At gestational age 6-7 weeks, the primitive allantois consists of an enlarged tube located between the umbilical cord and abdominal cavity, whereas the urachus is not yet developed. At 8 weeks, the allantois gradually withdraws from the distal to the proximal end of the umbilical cord, and both the proximal allantois and the rectum (hindgut) start to develop into the cloaca. At 10 weeks, the allantois was located mostly in the abdominal cavity., The urachus forms from the distal end of the allantois and develops into a closed fibrous cord between the base of the urinary bladder and the umbilicus. The urogenital sinus forms from the proximal end of the allantois.

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.

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., (© 2021 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

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., Competing Interests: Die Autoren bestätigen, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

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., (© 2020 Japan Pediatric Society.)

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 KRAS G12V 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 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.

Background: Bladder adenocarcinoma (AC) is a scarce histological variant and there are few studies on its proper management. No previous case reports present the management of a urachal tumor and the incidental finding of bladder adenocarcinoma. Clinical case: We present the case of a young woman with nonspecific symptoms, who presented with a prior history of dysuria, bladder tenesmus, suprapubic pain and urinary urgency for one year, which had been treated as recurrent urinary tract infection. A partial cystectomy plus extended lymphadenectomy was scheduled. We found a bladder tumor with characteristics of a urachal tumor and the pathological report indicated a primary bladder AC. The patient had a complete recovery at one year of follow-up. Conclusions: A patient can present with a tumor with urachal characteristics; however, the pathology report can show primary AC. The decision to perform partial cystectomy was an appropriate option for the location of this tumor, with optimal surgical results. Still, a long-term follow-up is necessary. More specific management guidelines are required for the treatment of AC., Competing Interests: No competing interests were disclosed., (Copyright: © 2019 Tejeda-Mariaca JE et al.)

Urachal sinus usually presents with umbilical discharge and the opening can rarely be located between the umbilicus and the symphysis pubis and the so called suprapubic sinus (SPS). There is another different entity of cases reported in literature with a similar presentation but with an opening anywhere between the umbilicus and symphysis pubis but differs from SPS in the pathway of the tract and the epithelial lining. We report a case of a 2-year-old boy presenting with a prepubic sinus that was managed with surgical excision. After a thorough literature review, we compare our case to other prepubic and SPS., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Urachal cancer is a rare but aggressive disease. In addition to the non-glandular tumors, non-cystic urachal adenocarcinomas are nowadays distinguished from the primary cystic variant. (Immunohistochemical) markers are only of minor differential diagnostic value and, therefore, the diagnosis is primarily established in a multidisciplinary approach. The non-cystic variant accounts for the majority of cases (83%), is more common in men (63%), shows a median age at diagnosis of 51 years and has a 5-year survival rate of about 50%. In organ-confined disease, usually a partial cystectomy of the tumor in the bladder dome, including the median umbilical ligament and umbilicus, is performed. In advanced stages, systemic therapy is needed while 5‑fuorouracil (5-FU) containing regimes have been shown to be more effective. Due to the rarity of the tumor, targeted therapy approaches based on a biological rationale are becoming increasingly relevant. As molecular data are still sparse, we compiled and analyzed the largest urachal cancer cohort to date. In 31% of the cases, MAPK-/PI3K signaling pathway alterations were detected (especially in K-/NRAS) with implications for anti-EGFR therapy approaches. Further potentially therapeutic alterations were detected in FGFR1, MET, PDGFRA, and erbB2/HER2. Additionally, PD-L1 tumor cell expression (clone: 22C3) was demonstrated in 16% of cases, therefore making anti-PD-1/PD-L1 immuno-oncological approaches worth considering despite the absence of mismatch repair deficiency (MMR-d) and/or high microsatellite instability (MSI-h). Finally, urachal adenocarcinomas seem to be a distinct entity on the molecular level with closer resemblance to colorectal adenocarcinomas than to urothelial carcinomas.

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.

A 57-year-old man presented with a 6-month history of pelvic fullness. He had no lower urinary tract symptoms or altered bowel habits. On examination, there was a non-tender pelvic mass which extended from the pubic symphysis to the level of the umbilicus. CT scan of the abdomen demonstrated a 22×11×11 cm cystic mass arising from the pelvis extending into the midline and superiorly to the umbilicus. Other than raised carcinoembryonic antigen of 7.6 ng/mL (<5.0), the remainder of his blood test were unremarkable. Flexible cystoscopy demonstrated a convex deformity of the bladder wall in keeping with the compression and displacement as seen on the CT. The patient underwent an open excision of the cystic structure (urachal remnant), partial cystectomy, partial excision of anterior abdominal wall and pelvic lymphadenectomy. A check cystogram performed 12 days following the initial operation was unremarkable., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: To determine the ideal management of uncomplicated adult urachal remnants and to confirm if conservative management with repeated imaging is acceptable., Patients and Methods: 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., Results: 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., Conclusions: 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.

Neoplasms arising from the urachus are extremely rare. We present a case of a young woman with mild abdominal pain and a palpable swelling in the mid abdomen with incidental detection of a subumbilical mass. Repeated fine-needle aspiration cytology failed to reveal the true nature of the lesion. F-FDG PET/CT showed tracer uptake in the cranial end of urachal remnant lesion. Core biopsy from the site with maximal FDG uptake turned out to be lymphomatous involvement in urachus, which is extremely rare and previously unreported.

Purpose: Peritoneal carcinomatosis or pseudomyxoma peritonei from urachus is a rare form of presentation, often diagnosed at an advanced state of tumor burden. Because of its rarity, little is known about its natural history, prognosis, or optimal treatment. We searched a large international multicenter database of peritoneal surface disease to identify cases of peritoneal carcinomatosis of urachus that were treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) at expert centers. The aim is to improve knowledge and understanding of the disease and standardize its treatment., Methods: A prospective multicenter international database was retrospectively searched to identify all patients with urachus tumor and peritoneal metastases who underwent CRS and HIPEC through the Peritoneal Surface Oncology Group International (PSOGI). Postoperative complications, long-term results, and principal prognostic factors were analyzed., Results: The analysis included 36 patients. After median follow-up of 48 months, median overall survival (OS) was 58.5 months. Three- and 5-year OS was 55.4 and 46.2%, respectively. Patients who underwent complete macroscopic CRS had significantly better survival than those treated with incomplete CRS, with median OS not achieved and of 20.1 months, respectively [95% confidence interval (CI) 4.4-30.5, p < 0.001]. There were no postoperative deaths, and 37.9% of patients had major complications., Conclusion: CRS and HIPEC may increase long-term survival in selected patients with peritoneal metastases of urachus origin, especially when complete CRS is achieved.

Rationale: Urachal borderline mucinous cystadenoma is very rare and has only 9 cases in the current literature with the biological behavior between adenoma and adenocarcinoma., Patient Concerns: We reported a 41-year-old man with moderate lower abdominal pain, and the imaging examination found an irregular cystic lesion extending from umbilicus to the dome of urinary bladder with significant separations and calcifications., Diagnoses: The diagnosis was confirmed according to the specific anatomical location and pathological examination which was proved as mucinous cystadenoma with low malignant potential., Interventions: The patient undertook radical excision and partial cystectomy., Outcomes: His postoperative condition was good., Lessons: Urachal borderline mucinous cystadenoma can be located by image examination, which may also offer several diagnostic tips according to separation, calcification, and enhancement in computed tomography scan. When combined with pathological findings, qualitative diagnosis can be determined. Surgical resection should be chosen as an optimal treatment. Our present study reviewed the clinical and biological information of all previous cases which were diagnosed as urachal borderline mucinous cystadenoma and we supplemented more data for further study., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Rationale: Pseudomyxoma peritonei is an unusual clinical condition, and the appendix and ovaries are reported as the primary sites., Patient Concerns: A 44-year-old man who was reported a 3-month history of lower abdominal pain and distention, along with increased abdominal girth, was admitted with a palpable tender mass in the central lower abdomen., Diagnosis: Ultrasonography showed a large well-circumscribed cystic-solid mass with lobulated margin, extending from the anterosuperior dome of the urinary bladder to the anterior abdominal wall. A computed tomography (CT) scan revealed a midline heterogeneous, hypodense, irregular polycystic-solid mass adjacent to the anterior wall of the abdomen and anterior to the dome of the urinary bladder. fluorodeoxyglucose positron-emission tomography/CT showed intense fluorodeoxyglucose uptake in the thickened wall of the mass. Intraperitoneal laparoscopic exploration also revealed a midline abdominal mass adjacent to the dome of the urinary bladder. Laparotomy showed that the mass originated from the dome of the urinary bladder and was disconnected with the urinary bladder lumen. The final histopathological diagnosis was urachal mucinous adenocarcinoma associated with high-grade pseudomyxoma peritonei., Interventions: The patient underwent surgical cytoreductive procedure and the perioperative intraperitoneal chemotherapy., Outcomes: The patient made an uneventful recovery, and 7 months later had no recurrence., Lessons: The urachus is a tubular structure, which extends medially from the apex of the bladder to the allantoid during fetal development, and it usually obliterates after birth. Urachal remnants can cause urachal carcinoma or bladder cancers. Pseudomyxoma peritonei originating from mucinous neoplasm of the urachus is extremely rare.

Purpose: To investigate the prognostic significance of tumor location on survival outcomes in patients with urinary bladder adenocarcinoma (BAC)., Methods: We retrospectively analyzed cases of BAC with known tumor location from the Surveillance, Epidemiology, and End Results database from 1973 to 2012. Data regarding patient demographics, tumor characteristics, and oncological and survival outcomes were collected. Patients were subgrouped according to tumor location into urachal/dome (dome and urachus [UD]), lateral wall (anterior, posterior, and lateral bladder walls [LW]), and base (trigone, ureteral orifices, and bladder neck [BL])., Results: A total of 1,361 cases of BAC with known tumor location were identified. More UD tumors were low grade (grade I and II; 51%) than LW (33%) and BL (43%) tumors (P<0.0001). UD lesions were the most likely to have metastatic spread (23% vs. 17% for LW and 15% for BL) (P<0.0001). The 5-year overall survival (OS) and disease-specific survival (DSS) rates were 37.3% and 49.0%, respectively, for all BAC. Furthermore, the 5-year OS rates were 42.3%, 35.9%, and 28.4% for UD, LW, and BL lesions, respectively (P<0.0001), whereas the 5-year DSS rates were 50.2%, 51.7%, and 42.1% for UD, LW, and BL lesions, respectively (P = 0.0097). Multivariate Cox regression analysis controlling for tumor stage and grade demonstrated that both tumors of the LW (hazards ratio [HR] = 1.52 for OS and 1.30 for DSS) and BL (HR = 1.71 for OS and 1.57 for DSS) conferred a worse prognosis relative to those of the UD (P< 0.05)., Conclusions: Tumor location of BAC is an independent prognostic factor for disease outcome. Our results suggest that the urachal and dome locations are associated with relatively favorable survival and oncological outcomes, whereas basal location confers poorer outcomes., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background and Objectives: Urachal carcinoma (UrC) is a rare and poorly investigated disease. Our current knowledge is mainly based on single-institutional studies. Despite growing interest in UrC, the included case numbers in recently published studies are still low. Therefore, we aimed to provide a comprehensive meta-analysis on the clinical, prognostic, and therapeutic aspects of UrC., Methods: A systematic Medline/PubMed search was performed on UrC using the terms "urachal carcinoma," "urachal cancer," and "urachus." Original articles and reviews in English language with case numbers>10 were selected., Results: The vast majority (91%, 489/532) of UrCs are diagnosed at later stages (Sheldon≥III) when the tumor invades the urinary bladder. About 21% (136/646) of UrC patients have distant metastasis at first presentation. Although for patients with non-metastatic UrC surgical treatment provides an acceptable disease control, the systemic treatment of patients with progressed/metastatic UrC-in lack of prospective clinical trials-are less well established. Comparing cisplatin-based and 5-FU-based therapies in 74 published UrC cases, we found the latter to be superior in terms of radiographic response rates (9% vs. 44%, P = 0.043), but the combination of these 2 therapies provided the lowest progression rate (14%) with a similarly high response rate (43%)., Conclusions: Owing to the lack of evidence-based guidelines, the therapy of UrC remains challenging. Given the infrequency of UrC, large prospective studies comparing different systemic therapies can hardly be conducted. Our metadata indicates that 5-FU-containing chemotherapy regimens are more effective than cisplatin-based treatment modalities, whereas their combination seems to provide the strongest antitumor effect. Nevertheless, in the lack of evidences from prospective clinical trials, therapeutic decision-making necessarily remains on an individual basis. In this situation, targeted therapies may provide a reasonable alternative. Therefore, better understanding of the molecular background of UrC is needed to rationalize treatment decisions in UrC., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Urachal carcinoma is a rare form of cancer. It often is diagnosed incidentally, like in our case report, because its cardinal symptom also occurs in a number of other urological diseases. We report the case of a 26-year-old man with a mucinous adenocarcinoma of the urachus. The carcinoma was removed via partial cystectomy with umbilical tumour excision., (© Georg Thieme Verlag KG Stuttgart · New York.)

Inflammatory myofibroblastic tumors (IMTs) are uncommon benign neoplasms in the abdomen, and urachal involvement is very rare. IMT of the urachus is usually mistaken clinically and radiologically for a malignant tumor. The tumor has spindle cell proliferation with a prominent inflammatory infiltrate and myxoid degeneration favoring other designations such as inflammatory pseudotumor and fibromyxoid pseudotumor. Complete excision is the treatment of choice. The case documented here was in a 50-year-old male, with an abdominal mass diagnosed on computed tomography scan as adenocarcinoma of urachus infiltrating the bladder. Histopathology showed a benign spindle cell tumor invading bladder muscle. Immunohistochemistry aided in diagnosing an anaplastic lymphoma kinase (ALK)-1 positive IMT. Approximately, 50% of the IMTs show ALK positivity, and though when present, it indicates neoplastic nature of the spindle cells, the pathogenesis of IMT is still elusive. The urachal IMTs need documentation in literature for their rarity and diagnostic dilemma posed by them.

Urachal fistula is a neonatal condition. There are two reported forms: a congenital and an acquired form. We describe the case of a 6-hour-old female alpaca cria that was presented with a damp umbilicus and a meconium impaction. Conservative treatment of the urachal fistula with local and systemic medication was unsuccessful after 6 days; therefore, a resection of the umbilicus under general anaesthesia was performed. Reconvalescence was uneventful.

The urachus is a remnant of the urogenital sinus and allantois, which persists after antenatal involution. Despite its rudimentary postnatal presence, it can undergo metaplasia and tumor formation. Malignant transformation of the urachus is rare. However, these tumors can recur and develop pseudomyxoma peritonei. Thus, they should remain on the differential for any female patient with a pelvic mass. A 28-year old G9P2062 presented at 8 weeks' gestation requesting pregnancy termination. On ultrasound, a single live intrauterine pregnancy was visualized. Also identified was a left-sided 8- × 7- × 6-cm complex echogenic mass with thickened septations, heterogeneous appearance, and fluid-fluid levels suspicious for a mature cystic teratoma. Surgical termination of pregnancy and diagnostic laparoscopy was planned. Intraoperatively a multiloculated midline mass was identified and excised. Final pathology identified a mucinous urachal neoplasm of low malignant potential. The patient followed up with urology and underwent surgical staging. Midline location is a key feature that helps distinguish ovarian masses from urachal or bladder masses. Many urachal and bladder neoplasms are managed with complete surgical excision and staging, illustrating the importance of preoperative identification. If laterality of a mass is unclear, further imaging is recommended to characterize the mass preoperatively. This case also reveals the feasibility of a minimally invasive abdominal wall mass resection., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Urachal anomalies are most often seen in children, seldom in adults, but are in general considered rare. The estimated incidence is one in 5000-7000 live births and appears twice as common in males. Despite their rarity, they need to be considered by clinicians, as diseases in the urachus can mimic many abdominal and pelvic conditions and constitute an important differential diagnosis to these. Diagnosis can be made by clinical examination and imaging modalities (computed tomography, ultrasonography, magnetic resonance imaging, voiding cystourethrogram), but some are discovered incidentally. Management of symptomatic urachal anomalies is surgery. Histological examination of the specimen should always be performed to rule out malignancy. We report on the first adolescent described in the literature diagnosed with a urachal sinus harboring a benign teratoma. A combination of the two pathologies is by inference an extremely rare condition, which we here report on and we review the relevant literature on this topic., (© 2014 APMIS. Published by John Wiley & Sons Ltd.)

Published experience remains limited for glandular neoplasms of the urachus, especially mucinous cystic tumors. We reviewed 55 glandular urachal neoplasms to evaluate their clinical features and histopathologic spectrum and to devise a classification system for the mucinous cystic forms. Within the 55 cases studied, we observed 2 groups with differing clinical, gross, and histopathologic features. The first group, invasive, noncystic adenocarcinomas (n=24), had clinicopathologic features in accord with the known spectrum of urachal adenocarcinoma (mean age 50 y, female:male ratio 1.7, with recurrence or death from disease in 9/16 cases over a 45 mo mean follow-up). The second group, mucinous cystic tumors (n=31), morphologically resembled mucinous cystic tumors of the ovary and appeared classifiable by the same approach (mean age 47 y, female:male ratio 1.4) and included mucinous cystadenoma (n=4), mucinous cystic tumor of low malignant potential (n=22, including 2 cases with intraepithelial carcinoma), and mucinous cystadenocarcinoma with microscopic (n=4) or frank invasion (n=1). Follow-up information was available for 13 patients with mucinous cystic tumors (mean 41 mo); we observed no local recurrence or distant metastasis. This experience suggests that there is a distinct group of glandular, cystic tumors of the urachus that is classifiable in a manner similar to ovarian neoplasms and that has a favorable prognosis after complete excision. As with cystic neoplasms of other organs, rigorous sampling is recommended to identify potentially small foci of carcinoma that could be missed by inadequate sampling. Accordingly, classification based on methods other than complete surgical excision may be hazardous.

Urachal carcinoma is an uncommon neoplasm. Benign urachal tumours are extremely rare. All urachal mucinous tumours, regardless of tumour type, have the potential to behave in an aggressive clinical manner that includes the development of pseudomyxoma peritonei. We report a 58-year-old man who presented with lower abdominal pain. Ultrasound and CT imaging defined a large lobulated cystic mass superior to the urinary bladder. At laparotomy, the multiloculated cystic mass, 10 x 8 x 6 cm, could be completely separated from the bladder and was excised. It was smooth-walled and filled with thick mucinous fluid. Histology revealed a complex mucinous cystadenoma of undetermined malignant potential of the urachus. The cystic spaces were lined by mucin-secreting columnar epithelium that showed focal areas of mild atypia. The epithelium lacked architectural features of villous adenoma. There was no stromal invasion to support an invasive neoplastic process. No pseudomyxoma peritonei was present. The patient was well, with no tumour recurrence, at 6 months follow-up.

Congenital prepubic sinus is a rare congenital anomaly situated in the midline of the lower abdomen. We report a case of congenital prepubic sinus, closely associated with a urachal remnant. Preoperative magnetic resonance imaging showed clearly that the sinus tracked the urachus caudally. This finding supports the theory that the anomaly is caused by abnormal remnant tissue originating from the cloacal membrane, which tracks the allantois duct caudally along with fetal longitudinal growth.

We report an otherwise well developing 2-year-old girl who presented with a urachal sinus with its opening located at the midline between umbilicus and the pubic symphysis. Diagnosis was made by ultrasound preoperatively, and confirmed during surgery. Additionally, columnar epithelium was found in the portion of sinus tract traversing the abdominal wall. Metaplasia of the sinus tract is worrisome, as urachal carcinomas have been traditionally characterized to result from this process., (© 2013.)

Objective: To describe a case of urachal adenocarcinoma treated with robotic assisted laparoscopic partial cystectomy and en-bloc exeresis of urachus and umbilicus and bibliographic review., Methods: A 63 year-old man with hematuria and hypogastric pain. He was diagnosed of urachal adenocarcinoma by transurethral resection and axial tomography. We performed a robotic assisted laparoscopic partial cystectomy using a da Vinci® S HD (Intuitive Surgical System) device. We describe the surgical technique and examine total length of time for surgery and for console, pathology report, margin status, postoperative outcome and oncological status 7 months after surgery., Results: 4 ports were used for robotic arms and one additional for the assistant. Cystoscopy was performed during surgery to mark tumor margins. Bladder was closed using a running suture with Poliglactin 0. Total length time for surgery was 2hs 28 minutes, console time was 1h54'. Two days later patient was discharged and no complication was reported. After two weeks Foley cathether was removed and bladder volume was 300ml. Pathology report informed undifferentiated urachal adenocarcinoma with perivesical tissue infiltration with margins free from tumor, corresponding to Sheldon IIIB and Ontario III classification. Seven months later patient was fee from recurrence., Conclusion: Robotic assisted laparoscopy partial cystectomy with en-bloc exeresis of urachal and umbilicus is feasible.

Purpose: A valved ventriculo-peritoneal shunt (V-P shunt) as a vesico-amniotic shunt (V-A shunt) preserves the filling/emptying cycle and normal bladder development in fetal lambs with bladder outlet obstruction. The optimal pressure for such shunts is unknown., Materials and Methods: We created obstructive uropathy in 60-day gestation fetal lambs. A V-A shunt was placed 3 weeks later, using a low-pressure (Group L: 15-54 mmH2O) or a high-pressure (Group H: 95-150 mmH2O) V-P shunt. We included non-shunted (obstructive uropathy, Group O) and control lambs (Group C). All were delivered at 130 days. Bladder volumes, bladder thickness, renal and bladder histology were compared., Results: Seventeen lambs had an obstructive uropathy created. Five Group L (four survived), four Group H (three survived) and five Group O survived. Body weight and crown-to-rump lengths of the three groups were not significantly different. Group H lambs had a dilated urachus, urinary ascites and severe ureteral dilatation similar to Group O lambs. There were four Group C lambs. Bladder volume was 10, 15 and 1,150 ml in Group H, 115 ± 67.9 ml in Group L, 128 ± 99.8 ml in Group O and 24.5 ± 3.84 ml in Group C. Unlike Group O lambs, Group L did not have urinary ascites, urinomas or renal dysplasia., Conclusion: Low-pressure shunts preserved both bladder volume and renal development. High-pressure shunts did neither.

Objective: To analyze the multislice spiral computer tomography (MSCT) manifestations and its value in the diagnosis of urachus lesions., Methods: A total of 26 patients with urachus disease diagnosed by pathological examination in our hospital between October 2007 and May 2011 were included in this study. We retrospectively analyzed MSCT images on the size, location, and shape character of the foci, and the image features of Retzius space., Results: In the group, 12 patients had simple urachal cyst with homogeneous fluid filling the cavity with thin wall in CT scanning; five had infected urachal cyst with thickened and enhanced wall, some patch and strip appearing in Retzius space surrounding the lesion; two had infected urachal sinus, and another 3 had urachus leakage. Four had urachal tumor showing irregular cysticsolid mass over the apex of the bladder with apparent enhancement in contrast imaging, and 2 had calcification. Invasion of the bladder wall was found in 4 patients, extensive invasion of the ileum, rectum, uterus and retroperitoneal lymph nodes was found in 1., Conclusion: MSCT scan can demonstrate the location, shape, extent of the lesion as well as the relation with adjacent structures, thereby providing valuable information for the diagnosis and differential diagnosis of urachus lesions.

Mucinous cystadenocarcinomas of the urachus are rare. Mucinous benign or premalignant tumors are even rarer, yet pose a challenge in diagnosis and management. We report a case of a 66-year-old man with lower abdominal pain who had a large cystic tumor at the dome of the bladder. En-bloc resection of the tumor with partial cystectomy revealed mucinous cystadenocarcinoma in situ. We reviewed the characteristics of all seven previously reported cases. These tumors are pre-malignant and can cause significant morbidity and mortality. They need to be treated similar to conventional mucinous cystadenocarcinoma by wide surgical resection and partial cystectomy.

The urachal mucinous adenocarcinoma is a rare malignant neoplasm located between the bladder and the umbilicus. It is usually found in an advanced stage at the moment of diagnosis. We have analyzed a clinical case in which the PET-CT study provided valuable morphological and metabolic information for diagnosis and staging., (Copyright © 2011 Elsevier España, S.L. y SEMNIM. All rights reserved.)

Urachal mucinous cystadenocarcinoma causing pseudomyxoma peritonei is very rare. We report a case of a 59-year-old man with urachal mucinous adenocarcinoma associated with pseudomyxoma peritonei, and our radiologic findings. Ultrasonography revealed a well delineated, large cystic tumor adjacent to the anterior wall of the abdomen. Computed tomography and magnetic resonance imaging revealed a tumor of which the left posterior wall was defective. A large amount of ascites was present in the peritoneal cavity. The ascites caused displacement of the intestinal structures toward the dorsal region. The tumor wall and septa in the ascites were well enhanced on contrast-enhanced images. Radiologically, pseudomyxoma peritonei due to rupture of urachal cystic tumor was considered. The pathologic diagnosis was mucinous adenocarcinoma and pseudomyxoma peritonei.

A 52-year-old woman had undergone a partial cystectomy for a cystic mass at the dome of the urinary bladder in 1997. The pathological diagnosis was a urachal mucinous cystadenoma with borderline malignancy. Twelve years later, multiple masses were noted on the uterus, ovaries and abdominal wall. She underwent debulking surgery, including a total hysterectomy, bilateral salpingo-oophorectomy, pelvic lymph node dissection, infracolic omentectomy, appendectomy, colon mass removal and abdominal wall mass removal. Remnants of the tumor were seeded throughout the abdominal cavity. The pathological evaluation confirmed metastatic urachal mucinous adenocarcinoma in both ovaries, the abdominal wall, the colon mass, the uterosacral ligament and the bladder. Adjuvant chemotherapy with paclitaxel and carboplatin was administered, which showed stable disease. She is now undergoing second-line chemotherapy., (© 2011 The Authors. Journal of Obstetrics and Gynaecology Research © 2011 Japan Society of Obstetrics and Gynecology.)