Urachal mixed adenocarcinoma and small cell neuroendocrine carcinoma with widespread metastasis and resistance to chemotherapy: a case report.

Neuroendocrine carcinoma arising from the urachus is extremely rare. We describe a case of a 33-year-old gentleman who presented with hematuria and diagnosed to have a composite adenocarcinoma and small cell neuroendocrine carcinoma arising from the urachus. The patient also had widespread metastasis at the time of presentation, therefore, he was referred for chemotherapy. However, the disease showed progression despite treatment. Recognition of neuroendocrine carcinoma component in urachal tumors, although rare, is very essential as this histologic type carries poor prognosis with aggressive clinical outcome.

Primary urachal adenocarcinoma: Case report.

INTRODUCTION AND IMPORTANCE: Primary urachal adenocarcinoma (PUA) is a rare form of cancer that arises from the urachus, a vestigial remnant of the allantois and cloaca during embryonic development. The exact pathogenesis of PUA is not well understood, but it is believed to arise from glandular epithelium remnants within the urachus. The rarity of this type of cancer makes it difficult to comprehensively study its epidemiology. CASE PRESENTATION: This case report describes a 47-year-old male patient who presented with intermittent painless hematuria and fatigue for two months. Cystoscopy showed a single growth at the dome of the urinary bladder, and abdominopelvic CT scan with contrast revealed a 3*2 cm enhancing growth at the dome of the bladder suspicious of urachal origin tumor. The patient was diagnosed with urachal adenocarcinoma (PT2) after pathological examination. The patient underwent partial cystectomy and umbilicectomy. CLINICAL DISCUSSION: Patients with PUA often present with nonspecific symptoms that can delay the diagnosis. The most common symptom is hematuria, which is present in approximately two-thirds of the patients. The diagnosis of PUA is challenging and relies on a combination of clinical presentation, imaging, and histopathological examination. CONCLUSION: The mainstay of treatment for PUA is surgical resection, which may include partial cystectomy or radical cystectomy with en bloc resection of the urachus and umbilicus. It is esential to report all cases of primary urachal adenocrcinoma.

The anatomy and pathology of the space of Retzius.

The space of Retzius is an important anatomic location for pathology. Pathology in the space of Retzius is more common than previously believed, especially as more cases are discovered with increased use of cross-sectional imaging. Knowledge of the anatomy of the Space of Retzius is crucial for identifying and distinguishing between benign and pathological findings. This paper uses several case examples to discuss benign etiologies, including normal tissue or a foreign body. The paper also demonstrates a case series with pathologic findings in the Space of Retzius under the broad categories of infection, neoplasm, hemorrhage, or urine extravasation. Understanding of the anatomy and these example cases can improve the diagnostic accuracy of radiologists.

Case report: Urachal perivascular epithelioid cell tumor.

Background: Urachal tumors are rare in clinical practice, among which urachal adenocarcinoma is the most common. In this study, we report a rare case of urachal perivascular epithelioid cell tumor to improve our understanding of the disease. Case presentation: A 26-year-old male patient was hospitalized for lower abdominal pain. The US showed a hypoechoic mass measuring 26mm × 18mm in the superior aspect of the bladder. MRI showed an irregular mass located anterior to the bladder roof, near the midline. The tumor exhibited hypointense on T1WI and heterogeneous hyperintense on T2WI. Additionally, contrast-enhanced T1-weighted imaging revealed obvious ring enhancement of the tumor. The patient underwent surgical resection of the urachal tumor, with subsequent pathological examination revealing a diagnosis of urachal PEComa. Following surgery, the patient underwent regular follow-up assessments, with no evidence of recurrence or metastasis observed after three and a half years. Conclusions: Urachal PEComa is a rare mesenchymal tumor that presents challenges in diagnosis through imaging and clinical symptoms. Definitive diagnosis relies on pathological and immunohistochemical analysis. Due to the rarity of urachal PEComa, prognosis assessment necessitates long-term follow-up and evaluation of more cases.

Estimation of the incidence of urachal cancer: A systematic review and meta-analysis of registry-based studies.

BACKGROUND: Urachal cancer (UrC) is a rare disease with limited availability of representative incidence and clinical data. Although, the prevalence is accounting for less than 1% of bladder tumors, the 5-year survival rate is around only 50% for patients with resectable tumors, and even worse for patients with metastatic disease. Due to the lack of comprehensive prospective studies, our current knowledge of UrC is still limited. OBJECTIVE: The present study aimed to summarize the available registry-based studies with unselected UrC patients to evaluate its incidence and clinicopathological characteristics. MATERIAL AND METHODS: We conducted a systematic literature search of registry-based UrC publications on the 15th of May 2023 in 5 databases, which identified 4,748 publications. After duplicate removal and selection by 2 independent investigators, 6 publications proved to be appropriate for the final meta-analysis. Estimated incidence and clinicopathological parameters were extracted. RESULTS: Estimated incidence ranged between 0.022 and 0.060/ 100.000 person-years, with the highest occurrence in Japan and the lowest in Canada, while the random effect model calculated an overall incidence rate of 0.04 (95%CI: 0.03-0.05) 100.000 person-years. The median age at first diagnosis was 60 years (range: 58-64). The female to male ratio was 2:3. Lymph node or distant metastases were present in 9% and 14% of patients. The predominant tumour type was adenocarcinoma (86%) followed by urothelial carcinoma (12%) and squamous cell carcinoma (2%). The 5-year survival rate was 51.0% with 95%CI: 45.2-57.4. CONCLUSIONS: Our study provides an up-to-date comparison of estimated incidence rates between 6 countries of 3 continents based on rigorously selected registry-based studies. The results suggest low incidence rates for UrC with considerable geographic differences. The present meta-analysis provides unbiased registry-based data on the incidence, clinicopathological parameters and survival of UrC.

Diffuse peritonitis secondary to urachal cyst abscess in a postpartum patient.

INTRODUCTION AND IMPORTANCE: Urachal cyst infections during pregnancy are exceptionally rare, posing diagnostic challenges. This case report contributes to the limited literature, emphasizing the rarity, diagnostic difficulties, and the need for heightened healthcare provider awareness for timely intervention. PRESENTATION OF CASE: A 32-year-old pregnant woman with persistent pelvic pain, fever, and urinary symptoms sought care with inconclusive initial diagnoses despite multiple ER visits. Labor revealed a palpable mass, and postpartum, a CT scan identified a urachal cyst abscess. Urgent laparoscopy confirmed peritonitis, leading to cyst removal, antibiotics, and a subsequent laparotomy. Histology confirmed an abscessed urachal cyst. DISCUSSION: Urachal cyst infections in pregnancy, exceptionally rare and diagnostically challenging, highlight the importance of considering them in abdominal pain differentials. Diagnostic tools, such as ultrasound and CT scans, can be misleading, emphasizing the necessity for a multidisciplinary approach. CONCLUSION: This case report underscores the challenges in diagnosing and managing an infected urachal cyst during pregnancy, stressing the need for awareness and a comprehensive diagnostic approach for optimal outcomes. The rarity of such cases warrants increased attention within the medical community.

A remnant never forgotten: the utility of circulating tumor DNA in treatment guidance of urachal cancer.

Urachal cancer is a rare malignancy of the urachus that is treated with surgical resection if localized and systemic chemotherapy for metastatic disease. Circulating tumor DNA (ctDNA) is a single-stranded or double-stranded DNA released by tumor cells into the blood and harbored the mutations of the original tumor, shedding new light on molecular diagnosis and monitoring of cancer. We report a case of resected localized urachal cancer with clear surgical margins and negative lymph node dissection but elevated ctDNA that progressed to metastatic disease. We also highlight the possibility of using ctDNA levels to assist in adjuvant therapy.

Management of urachal anomalies in pediatric patients: A comparison of treatment strategies between pediatric urology and general surgery.

INTRODUCTION: Persistence of embryonic urachal structures due to a failure of the urachus to involute into the median umbilical ligament is known as a urachal anomaly (UA). UAs may remain asymptomatic or lead to abdominal pain and recurrent infections. Management of UAs in pediatric patients has historically lacked a clear consensus between conservative and surgical management. While both urologists and general surgeons manage this pathology, a comparison of management style and outcomes between these specialties has not been published to our knowledge. OBJECTIVE: To (1) evaluate trends in management of UAs among pediatric urologists and general surgeons across three tertiary care children's hospitals and (2) identify factors that place patients at higher risk for requiring surgery. STUDY DESIGN: All patients diagnosed with a UA from 2016 to 2020 at our multi-site institution were identified by ICD-10 code Q64.4 "malformation of the urachus" and retrospectively reviewed. Patient demographics, treatment specialty, remnant subtype, and management strategy were recorded. Data was dichotomized between both urology and general surgery as well as between surgical and nonsurgical intervention to identify and compare management strategies. RESULTS: Overall, 143 patients diagnosed with UAs were identified. Of these patients, 74 were treated by urology and 69 were treated by general surgery. Patients who were treated by urology were significantly more likely to receive conservative treatment (66.2% treated conservatively vs. 33.8% treated surgically), while patients treated by general surgery were significantly more likely to undergo surgery (84.1% treated surgically vs. 15.9% treated conservatively, p < .0001). Though, urology was more likely to treat patients who presented incidentally (p < .01), and general surgery was more likely to treat patients who presented with an infected remnant (p < .01). Patients of male sex were more likely overall to receive surgery compared to female patients (p < .01). DISCUSSION: Management of UAs by urologists was more conservative than general surgeons. However, both specialties treat distinctly different patient presentations, with urology managing more incidental remnants and general surgery operating on more emergent, infected urachi. Limitations of the study included its retrospective nature and the insufficient reporting of urachal remnant subtypes and presence of infection among patients. CONCLUSIONS: Management strategies of UAs differ among urology and general surgery, but surgical and conservative treatments are necessary to appropriately treat their distinct patient populations. This study provides valuable insight into current practices of UA management and may help to inform future treatment.

Non­syndromic first case of pediatric rhabdomyosarcoma originating from the umbilical left medial ligament: A case report.

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children. The present study reports the case of a 2-year-old female who presented with abdominal pain and a palpable abdominal mass. Radiological investigations failed to reveal the tissue origin of the mass and a tru-cut biopsy confirmed the diagnosis of embryonal RMS. Surgical excision was performed after neo-adjuvant chemotherapy. The pelvic end of the mass was observed to continue with the left medial umbilical ligament. The patient's postoperative course was uneventful, and follow-up imaging showed no evidence of recurrence. Τhe present case report is the first non-syndromic case with left umbilical medial ligament-originated RMS.

Rare urachal mucinous cystic tumor of low malignant potential with peritoneal pseudomyxoma: A case report.

Mucinous cystic tumors of low malignant potential (MCTLMP) are rare urachal neoplasms. The morphological characteristics and clinical prognosis of MCTLMP is similar to that of mucinous cystic tumors occurring in the ovary and appendix. After complete resection, almost no cases of recurrence or metastasis have been reported. Because MCTLMP is rare, it may be missed in the clinic. MCTLMP can lead to the formation of pseudomyxoma peritonei (PMP), which manifests as the widespread production of mucus in the abdominal cavity and makes the disease complex or difficult to diagnose. At present, only 3 cases of MCTLMP with PMP have been reported in the literature. In the present study a fourth case of urachal MCTLMP in a 74-year-old male that resulted in widespread PMP is presented. Initially, a multilocular cystic lesion was revealed in the urachal duct area at the anterior upper margin of the bladder after a patient, experiencing lower abdominal pain, was imaged. As revealed using light microscopy, the cyst was lined with a mucous columnar epithelium, and part of the epithelium indicated pseudolamellar hyperplasia and papillary structures. The cells indicated mild atypia and low mitotic activity. There was no stromal infiltration of tumor cells, and a large amount of mucous exudate was observed. As preoperative computed tomography examination suggested the presence of a large amount of ascites and there were increased levels of blood tumor markers, carcinoembryonic antigen and carbohydrate antigen 125, clinicians considered that the diagnosis maybe a malignant tumor of the urachal gland with peripheral dissemination. However, the diagnosis of MCTLMP with PMP was confirmed by histopathological examination. The mass was completely removed, along with part of the peritoneum and bladder wall as these were within the tumor margin. The appendix appeared normal during surgery. A one off dose of intraperitoneal infusion chemotherapy with 1,000 mg 5-fluorouracil was performed after surgery. No recurrence was observed during the 8-month follow-up period.

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 Adenocarcinoma: a Case Report and Review of the Literature.

Urachal adenocarcinoma is one of the rare and aggressive neoplasms that often presents at an advanced stage and has a poor prognosis. Urachal adenocarcinoma makes up 0.17 to 0.34% of all bladder carcinomas. Patients commonly present with hematuria. Wide local excision of urachal mass with umbilicus and surrounding soft tissue en bloc combined with partial or radical cystectomy and bilateral pelvic lymphadenectomy is considered to be the primary surgical management. However, many publications in literature report that en bloc removal of tumor with umbilicus, entire urachal ligament, and bladder dome alone has long-term survival and disease-free period. Here, we present a case of a 50-year-old post-menopausal female patient with a fungating mass in the umbilical region of size approximately 10 cm in maximum diameter with mucopurulent discharge from the mass. The patient had no history of any hematuria, mucinuria, burning micturition, or any particles in urine. A laparotomy was performed, and tumor mass along with the cuff of the bladder dome was removed as en bloc along with umbilectomy. This case report highlights a rare case of urachal adenocarcinoma with a fungating mass of large size in a female patient which is otherwise more commonly seen in males. A review of published literature is also presented.

Urachal Adenocarcinoma: A Rare Primary Cancer Managed With FOLFOX Chemotherapy.

Urachal adenocarcinoma (UA) represents a rare subset of bladder tumors involving a urachal remnant. Incidental gross hematuria is often the only presenting symptom, with patients often presenting late in their course, thereby imparting an overall poor prognosis. Although there are prior case reports, there is little literature reported and no standardized treatment guidelines. We report a case of a middle-aged male who presented with incidental gross hematuria after a fall. Workup indicated the presence of a calcified bladder dome mass and pathology reported a primary urachal adenocarcinoma with pelvic nodal involvement. Patient underwent surgical resection and subsequent adjuvant, systemic chemotherapy regimen with leucovorin, fluorouracil, and oxaliplatin (FOLFOX). We hope to bring greater awareness to this rare cause of bladder malignancy.

Primary urachal leiomyosarcoma: a case report and literature review of clinical, pathological, and medical imaging features.

Background: Urachal tumors are exceedingly rare, and adenocarcinoma is the most common malignant urachal neoplasm. Here, an especially rare patient of primary urachal leiomyosarcoma from our hospital was reported, and only five patients have been reported thus far since 1981. Case description: A 24-year-old man was admitted due to urinary tract symptoms. Both urogenital ultrasonography and contrast-enhanced computed tomography showed a mass at the dome of the urinary bladder. Laparoscopic surgical resection was performed, and histopathologic examination of the mass confirmed the diagnosis of urachal leiomyosarcoma. No recurrence was noted after one and a half years. Conclusions: Because the leiomyosarcoma located in the extraperitoneal space of Retzius and may manifest with nonspecific abdominal or urinary symptoms, early and definitive preoperative diagnosis is challenging. Partial cystectomy with complete excision of the urachus is recommended. Because only a few patients have been recorded, clinical outcomes and recurrence risks are difficult to assess.

Inflammatory suppurated tumor of urachus mimicking a malignant urachus adenocarcinoma.

This is a case report about a patient presenting with a urachal mass mimicking a urachus adenocarcinoma. Cystoscopy showed a vesicourachal patent diverticulum. Histological findings after the removal of the umbilicus, urachus, urachal tumor, as well as a bladder cuff, consisted of a nonspecific polymorphous suppurative inflammatory infiltrate. Urachal adenocarcinoma is an aggressive tumor with poor prognosis if not treated while it is still localized. Surgical excision is the only recommended treatment that offers the best chances of survival. As no preoperative procedure has been proven accurate enough to rule out the diagnosis of adenocarcinoma, surgery appears to be inevitable.

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.

A Very Rare Disease of Patent Urachus Cyst With Vesico-Umbilical Urinary Fistula in Adults: A Case Report and Short Review.

Rare developmental anomalies known as urachal remnants are brought on by flaws in the foetal developmental process. However, depending on the location and degree of incomplete obliteration, the urachus can undergo a variety of urachal anomalies. An umbilical fistulogram and a voiding cystourethrogram both supported the existence of the adult urachal cyst in this case. To treat the sepsis, we provided the patient with antibiotics first, then a surgical procedure. The entire vesico-umbilical tract with the urachal cyst was removed using the open approach. The excised specimen's histology revealed a foreign body giant cell reaction without any indication of malignancy. The presentation and diagnosis of vesico-umbilical urinary fistula (VUUF) in adults can occasionally be difficult. They happen very rarely. So we began putting forward this case for the same reason.

Modern methods in managing urachal adenocarcinoma.

Objectives: We sought to evaluate modern diagnostic and treatment options for urachal adenocarcinoma (UAC) and to provide clarity regarding the available options and their outcomes for this poorly understood yet damaging disease. Material and methods: We conducted a systematic literature search in PubMed and Medline focusing on updated management of UAC. Results: Surgical intervention continues to be the mainstay of treatment for localized UAC. However, with the increased availability of molecular and genetic profiling, chemotherapy has consistently demonstrated promising response rates and survival outcomes, especially for a disease that commonly presents in a metastatic stage. The role of checkpoint inhibitors remains under investigation. Cross-sectional imaging is vital during postoperative surveillance. However, there may also be a role for the adoption of cystoscopy to detect bladder recurrence. Conclusions: Although the importance of surgical resection remains unchanged, improved survival outcomes with chemotherapy have been found in small retrospective studies. Randomized trial data are required to further assess the influence of systemic treatment as a primary or adjuvant therapy. Moreover, a stringent follow-up regimen incorporating evaluation for distant and local recurrence of UAC must be evaluated and adopted.

A Giant Urachal Accessory Spleen: Case Report.

Accessory spleen (AS) refers to single or multiple splenic tissues which appear outside the relative normal spleen position results from embryonic dysplasia similar in structure and function to the spleen. AS is frequently observed in the splenic hilus and or adjacent to the tail of pancreas, and only a few cases occurred in the pelvic cavity. We present an extremely rare AS case in urachus, which was initially considered as an urachal neoplasm revealed on CT images with big mass. However, the postoperative pathology confirmed it was an AS that had not been reported at urachal before. Urachal AS can be misdiagnosed as a tumor, so it is vital to make an accurate imaging preoperative diagnosis to avoid unnecessary biopsy and surgery.

Prenatally diagnosed giant umbilical cord resulted from patent urachus in a monochorionic diamniotic twin with selective intrauterine growth restriction.

A long diffuse giant umbilical cord (GUC), caused by umbilical cord edema associated with a patent urachus, is an extremely rare anomaly. While patients with diffuse GUC appear to experience no significant complications and a good prognosis, little is known about their etiology and prenatal course. Here, we report the first case of prenatally diagnosed diffuse GUC resulted from patent urachus in a monochorionic diamniotic twin with selective intrauterine growth restriction. This case indicates that GUC is epigenetic and unrelated to multiple births.