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.

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.

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.

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.

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.

Peritoneal Carcinomatosis of Urachus Origin Treated by Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC): An International Registry of 36 Patients.

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.

An unexpected mass of the urachus: a case report.

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.

Complex mucinous cystadenoma of undetermined malignant potential of the urachus: a rare case with review of the literature.

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 associated with a urachal remnant: report of a case.

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.

Valved shunt as a treatment for obstructive uropathy: does pressure make a difference?

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.

[Urachal adenocarcinoma treated with robotic assisted laparoscopy partial cystectomy]. / Cistectomóa parcial laparoscópica asistida por robot Da Vinci para el tratamiento del adenocarcinoma de uraco.

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.

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.

Cystic mucinous tumors of the urachus: carcinoma in situ or adenoma of unknown malignant potential?

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.

[Multislice spiral computer tomography imaging characteristics of urachus lesions].

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.

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.

Metastatic urachal carcinoma of the ovary.

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.

[Colo-urachal-cutaneous fistula in an adult]. / Fistule colo-cutanée d'origine allantoïde chez un adulte

BACKGROUND: An infected urachal cyst is an uncommon finding in adults. AIM: To report a case of a sigmoid- urachal- cutaneous fistula. CASE REPORT: A 51 years old adult male presented with fecaloid leaking from rupture of an infected urachal cyst. Definitive management consisted of resection of the urachus with a bladder cuff, along with removal of the affected bowel segments. CONCLUSION: Colo-urachal-cutaneous fistula is a rare condition; its management is rather complex.