Inflammatory Pseudotumour Of The Urachus: Case Report And Literature Review.

A 52 year old woman presented to the emergency department of Affiliated Hospital of Zunyi Medical University, Zunyi, China in May 2022, complaining of a palpable lower abdominal mass since two days. She denied haematuria, umbilical drainage, or any other urinary symptoms. Previous health record indicated that the patient was diagnosed with urachal inflammatory pseudotumour. Inflammatory pseudotumourous masses of the urachal canal are rare chronic inflammatory disorders with only a few case reports. Ultrasonography is the preferred method for diagnosing urachal lesions. Contrast- enhanced ultrasonography (CEUS) allows real-time visualization of the microvascular blood flow within the solid lesion, reducing the probability of misdiagnosis of the disease. We have reported a case of urachal inflammatory pseudotumour and analyzed its ultrasonographic findings from two-dimensional conventional ultrasonography and CEUS to provide support for the diagnosis of urachal inflammatory pseudotumour in the clinic and to assist clinical selection of effective treatment modalities.

Urachal xanthogranuloma: a rare but important case presenting as a urachal mass.

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.

Surgical treatment of urachal remnants in an adult population-a single-centre experience.

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.

Urachal Actinomycosis Presenting with Recurrent Urinary Tract Infections in a Middle-aged Woman: A Rare Case Report.

The urachal cyst, a remnant of allantois sac during embryogenesis, is a rare condition in adulthood. Urachus is an embryologic remnant degenerating after birth. Abberrant obliteration of the urachus causes urachal abnormalities. The urachal cysts are almost always symptomatic when infected. The symptoms include fever, abdominal pain, tenderness, lower abdominal mass, nausea, vomiting, and dysuria. Ultrasonography, computerised tomography, and magnetic resonance imaging techniques may be insufficient for diagnosis. In most cases, staphylococcus species are isolated from cultures of urachal cysts. Other microorganisms such as Escherichia coli, Enterococcus faecium, Klebsiella pneumonia, and rarely actinomyces may be isolated. Actinomyces, an anaerobic gram-positive filamentous bacterium, is a rare cause of granulomatous disease. In this case report, a 56-year woman with urachal cyst infection with actinomyces is discussed according to current knowledge. Key Words: Urachal cyst, Urachus, Chronic cystitis, Actinomyces.

Urachal adenocarcinoma: A case report and review of the literature.

The urachus is a canal between the allantois and the early fetal bladder. Urachal carcinoma is a rare and aggressive type of bladder cancer. This cancer usually presents at an advanced stage. We report a 70-year-old patient with malignant transformation of urachal cyst several years later. The patient was treated with partial cystectomy and adjuvant radiotherapy. A review of the published literature is also presented.

Case of Infected Urachal Cyst in an Adult Presenting as an Incarcerated Umbilical Hernia.

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.

The Allantois and Urachus: Histological Study Using Human Embryo and Fetuses.

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.

Anterior wall adenocarcinoma of bladder with similar clinicopathological and prognostic characteristics as common bladder carcinomas should not be treated as or classified into urachal adenocarcinomas.

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.

[Uroperitoneum in neonatal foals - a review of the literature]. / Uroperitoneum beim Fohlen ­ eine Literaturübersicht.

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.

Ex vivo modelling of drug efficacy in a rare metastatic urachal carcinoma.

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.

Evaluation and management of urachal remnants in children.

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.

An infected urachal cyst presenting as acute abdominal pain in a child: A case report.

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.

Suprapubic versus prepubic sinus: a literature review.

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.

Urachal cancer-current concepts of a rare cancer.

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 5­fuorouracil (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.

Urachal Mucinous Cystadenocarcinoma.

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.

Case Report: Bladder adenocarcinoma: primary or urachal?

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.

Patterns of Urachal Remnant Involvement by Urothelial Carcinoma: Intraluminal Noninvasive Spread Can Mimic a Deep-seated Bladder Invasion.

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.

Carcinoma del uraco / Urachal Carcinoma

Objetivo Revisar la patología tumoral del uraco, haciendo énfasis en su clínica, los métodos diagnósticos empleados y el manejo terapéutico. Métodos Presentamos el caso de un varón de 39 años con carcinoma del uraco que simuló clínicamente un absceso umbilical. Resultados Los hallazgos en la TC fueron sospechosos de patología del uraco. El examen anatomopatológico reveló células atípicas y la PET-TC demostró extensión a pared abdominal y epiplón, requiriéndose la resección completa. Conclusiones El carcinoma del uraco es una neoplasia muy poco frecuente, que tiene su origen en el epitelio que recubre la luz del uraco, un vestigio de la alantoides que conecta la vejiga con el ombligo y normalmente involuciona en la etapa embrionaria. La clínica insidiosa hace que el diagnóstico sea tardío y el pronóstico pobre. Las pruebas de imagen tienen un papel fundamental en su sospecha, así como en la definición de la relación con la pared vesical y posible extensión local o a distancia. Una vez confirmado el diagnóstico histológicamente, está indicada la resección quirúrgica completa.

Objective To review tumoral pathology of urachus, emphasizing its clinical manifestations, diagnostic methods and therapeutic management. Method We present the case of a 39-year-old male with urachal carcinoma who clinically mimicked an umbilical abscess. Result CT findings were suspected of urachus pathology. Histologic analysis of the resected specimen demonstrated atypical cells, and PET-CT showed extension to the abdominal wall and omentum, requiring complete resection. Conclusions Urachal carcinoma is a very rare neoplasm that originates in the epithelium that covers the lumen of the urachus, a vestige of the allantois that connects the bladder with the navel and normally involves in the embryonic stage. The insidious clinic makes late diagnosis and poor prognosis. Imaging tests play a fundamental role in their suspicion, as well as in the definition of the relationship with the bladder wall and possible local or distant extension. Once the diagnosis has been confirmed histologically, complete surgical resection is indicated.