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OBJECTIVES: To identify the clinicopathological features of malignant urachal tumor in Japan, and analyze the 5-year overall survival of malignant urachal tumor patients. METHODS: We used the hospital-based cancer registry data to extract malignant urachal tumor cases that were diagnosed in 2008-2009 and 2012-2015, histologically confirmed, and received the first course of treatment. We analyzed the 5-year overall survival using the 2008-2009 cohort's data. RESULTS: We identified 456 patients, and malignant urachal tumor accounted for 0.4% of all malignant bladder cancers. The median age was 61 years (range 2-97), and 66% were men. The most common histology was adenocarcinoma (80%), followed by urothelial carcinoma (11%) and squamous cell carcinoma (3%). The proportions of patients were: 19% Ta/Tis/T1N0M0, 55% T2-3N0M0, 13% T4/N+ and 13% M+. Regarding the initial treatment, the proportions of surgery alone were 79% and 33% in cases of T3 or less N0M0 and T4/N+, respectively. The proportion of combination therapy including surgery and chemotherapy were 13% and 44% in T2-3N0M0 and T4/N+, respectively. Radiation therapy was not common at any stage. In the 2008-2009 cohort, the 5-year overall survival rate in Ta/Tis/T1N0M0, T2-3N0M0, T4/N+ and M+ were 60%, 64%, 63% and 12%, respectively. CONCLUSIONS: Malignant urachal tumors are quite rare in Japan, and most of those without metastasis are likely to be treated by surgery alone, even at advanced stages. A standard of care must be established for malignant urachal tumor patients at advanced stages or with metastasis, as the prognosis of these patients can be poor.
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Carcinoma de Células de Transição , Úraco , Neoplasias da Bexiga Urinária , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Sistema de Registros , Neoplasias da Bexiga Urinária/epidemiologia , Neoplasias da Bexiga Urinária/terapia , Adulto JovemRESUMO
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.
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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.
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Primary urachal leiomyosarcoma is rare entity with unclear prognosis and treatment strategy. We report a case of urachal leiomyosarcoma presenting, and treated with open urachal tumor resection and partial cystectomy. Pathological diagnosis showed low-grade leiomyosarcoma. At 53-months follow-up, no recurrence or metastasis was found. The purpose of this case is to raise awareness of the disease.
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Malakoplakia is a rare chronic neoplasm malady with advanced and variable etiologies and a high rate of clinical misdiagnosis. We reported a case of urachal malakoplakia involving the bladder. The patient was found a space-occupying lesion between the anterior superior of the bladder. The increased CT showed that the urachal lesion concerned the highest wall of the bladder, and therefore the high wall of the bladder was on an irregular basis thickened and increased. Urachal growth radical surgical operation combined with partial cystectomy was performed with the operative antibiotic medical aid. Pathological results showed urachal malakoplakia of the bladder. There was no local recurrence or metastasis during 18 months of follow-up.
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Primary actinomycosis occurring in urachal remnants is rarely documented in literature and may mislead the clinicians to diagnose urachal carcinoma. A 50-year-old man came with complaints of lower abdominal pain, dysuria, and dribbling of urine for 2 months. A vague mass of 7 cm was palpable in the suprapubic region. Imaging of the abdomen revealed an irregular mass seen superior to fundus of the urinary bladder appearing adherent to mesentery and bowel loops, suggesting an urachal remnant associated lesion with infiltration. Provisional diagnosis of urachal carcinoma was considered. Excision of the mass with partial cystectomy and resection of involved ileal segments were done. Microscopic examination revealed actinomycotic colonies surrounded by microabscesses and dense inflammatory fibrotic lesion. Small intestinal segments showed a similar lesion in the serosa.
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Urachal adenocarcinoma is a rare and aggressive form of bladder cancer that arises from the urachus. An en bloc resection of the urachus and umbilicus is recommended with either a total or partial cystectomy. However, there is no standard laparoscopic or robotic surgical technique for the operative management of these tumors. In the present report, we describe our robotic-assisted laparoscopic technique for the treatment of a primary malignant urachal tumor.
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Adenocarcinoma/cirurgia , Laparoscopia/métodos , Tratamentos com Preservação do Órgão/métodos , Procedimentos Cirúrgicos Robóticos/métodos , Neoplasias da Bexiga Urinária/cirurgia , Feminino , Humanos , Pessoa de Meia-IdadeRESUMO
We experienced a case of an inflammatory pseudotumor resembling an urachal tumor. The case involved a 46 year-old female patient, who complained lower abdominal pain and voiding symptoms with fever. An adult fist sized, hard ovoid fixed mass, was palpable in the suprapubic area. The pelvic ultrasonographic, CT and the MRI findings indicated an urachal tumor. The mass lesion was diagnosed from a percutaneous needle biopsy and cystoscopic biopsy, as an inflammatory pseudotumor. We administered antibiotics and performed conservative treatment only. The mass had disappeared on a bimanual physical examination and cystoscopy, 2 months later. There was no evidence of recurrence on follow-up CT scan 4 months later.