Case of urachal cancer treated by neoadjuvant chemotherapy with FOLFOX4 (oxaliplatin, 5-FU and leukovolin)

A 52-year-old woman was referred to our hospital for treatment of urachal cancer. She complained of supurapubic dull pain and gross hematuria. Computed tomography and magnetic resonance imaging showed a non-papillary sessile tumor, which was located on the dome of the bladder and invaded the small intestine. The tumor was diagnosed as Sheldon's stage IIIC urachal cancer. After three courses of neoadjuvant chemotherapy with FOLFOX4 (oxaliplatin, 5-FU and leukovolin), the tumor was reduced from 7 x 6 cm to 5.5 x 5 cm in size. Consequently, the patient underwent an en-bloc resection of the urachal tumor with the dome of the bladder and the parts of the ileum invaded by the tumor. One course of adjuvant chemotherapy (FOLFOX4) was performed. Surgical specimen revealed histologically well differentiated squamous carcinoma and invasion to the propria of the ileum. The surgical margins were negative for the cancer. For 1.5 years after the surgery, no local recurrence or distant metastasis has been observed.     

Laparoscopic partial cystectomy with en bloc resection of the urachus for urachal adenocarcinoma

Abstract:   Urachal carcinoma accounts for less than 1% of bladder cancers. The preferred treatment is wide local excision with partial or radical cystectomy. Two patients who presented with urachal carcinoma underwent laparoscopic partial cystectomy with en bloc resection of the urachus and bilateral pelvic lymphadenectomy as a less morbid, minimally invasive surgical alternative. Laparoscopic excision was successfully carried out in two cases with an uneventful convalescence.     

腹腔镜扩大膀胱部分切除术治疗成人脐尿管肿瘤五例报告并文献复习

目的探讨腹腔镜扩大膀胱部分切除术治疗成人脐尿管肿瘤的可行性和临床疗效。 方法回顾性分析我院采用普通腹腔镜和达芬奇机器人辅助腹腔镜下扩大膀胱部分切除术治疗脐尿管肿瘤5例临床资料,手术切除范围包括脐尿管、脐孔、脐尿管肿物、脐正中韧带、部分腹膜以及与脐尿管肿物相连的部分膀胱顶部,对手术时间、术后住院天数、有无术后并发症及随访有无肿瘤复发等指标进行分析,评价手术疗效。检索国内外相关文献,讨论脐尿管肿瘤的发病特点、影像学特征以及治疗方法。 结果5例手术均成功,无中转开放,平均手术时间140 min,术中出血少,术中术后无严重并发症出现,术后平均住院天数7.4 d,其中3例行术后辅助化疗3个疗程,化疗方案为顺铂+紫杉醇,术后随访1~4年无复发。术后病理诊断1例为绒毛状腺瘤,3例为脐尿管黏液腺癌,1例为浸润性高级尿路上皮癌,所有病例切缘及淋巴结均未见癌累及。 结论腹腔镜扩大膀胱部分切除术治疗成人脐尿管肿瘤,是一种安全、有效的方法。     

Transitional cell carcinoma of urachus: a case report

We report a case of transitional cell carcinoma of urachus in a 72-year-old man. At follow up cystoscopy for past history of bladder cancer, we found a papillary tumor in the right orifice that came out to the bladder cavity intermittently. Although there was no cancerous lesion on the surface of the bladder mucosa, a submucosal eminence at the dome of bladder was observed. Sagittal magnetic resonance imaging (MRI) revealed an extravesical tumor (2 cm) at the position of urachus. Under the diagnosis of right ureteral cancer and urachal cancer, we performed right distal ureterectomy, ureteral reimplantation and total resection of urachus. Pathological examination revealed transitional cell carcinoma in the urachus and right ureter. The urachal cavity was isolated completely from the bladder cavity. Tumor infiltrated to the muscularis of the bladder dome from the urachal cavity, but there was no cancerous lesion on the surface of the bladder mucosa. Therefore, our diagnosis was urachal transitional cell carcinoma and right ureteral carcinoma.     

Synchronous and multiple transitional cell carcinoma of the bladder and urachal cyst

Abstract:   Incomplete involution of the allantoic duct can result in different pathological forms of urachus which can give rise to inflammation or late malignant changes. Among urachal tumors, adenocarcinoma is most frequent, although other histological types can also be found. The synchronous presentation of a urachal transitional cell tumor, along with recurrent superficial bladder tumors has not been reported previously. We are reporting a 49-year-old male patient in whom transitional cell carcinoma of a urachal cyst was found with recurrent, multiple bladder tumors. The diagnosis of urachal cyst tumor was established according to ultrasonography and computed tomography. Most of the bladder tumors were resected transurethrally while open surgical excision of the urachal cyst with en bloc resection of the bladder dome was performed. Recurrent bladder tumors were afterwards treated with Bacillus Calmette Guerin (BCG) instillations. A year after surgery the patient has no signs of local recurrence or distant metastases of transitional cell carcinoma.     

脐尿管疾病的诊治研究及文献复习(附14例报告)

目的：探讨脐尿管疾病的诊断与治疗方法。方法：根据病史并结合经脐孔或膀胱美蓝灌注试验、膀胱镜检查、瘘管造影、B超、CT等辅助检查方法诊断为脐尿管异常疾病14例，并进行相应手术治疗。结果：手术及病理检查证实为脐尿管瘘并感染2例。脐尿管囊肿并感染5例。脐尿管窦道并感染5例。原发性脐尿管鳞状细胞癌1例。患者术后排尿正常。8～18d痊愈出院。1例脐尿管瘘并感染者拒绝手术出院。结论：经脐孔或膀胱美蓝灌注试验、膀胱镜检查、瘘管造影、B超、CT等辅助检查为脐尿管疾病有效的诊断措施；手术范围应以尽可能彻底切除脐尿管及其异常组织为宜。     

Treatment methods as a prognostic factor in eight patients with urachal carcinoma

Between 1960 and 1982, 8 patients with urachal carcinoma underwent segmental resection of the bladder or en bloc resection, and their five-year survival rate was 50%. One patient each with well, moderate and poorly differentiated adenocarcinoma and one patient with transitional cell carcinoma, died of cancer from 6 months to 2 years and 2 months after operation (mean duration: 1 year and 3 months). The patient with well differentiated adenocarcinoma underwent en bloc resection and was recognized to have peritoneal involvement of the tumor at the operation. The remaining three patients were diagnosed to have tumors confined to their bladder and urachal remnant and were treated with segmental resection of the bladder. Two patients each, with well and moderately differentiated carcinoma confined to their bladder and urachal remnant, were treated with en bloc resection and have been surviving from 8 years and 5 months to 24 years and 10 months (mean duration: 13 years and 7 months) postoperatively as of Dec. 1987. Therefore, patients with well and moderately differentiated adenocarcinomas confined to the bladder and the urachal remnant could be expected to survive longer by en bloc resection.     

Signet-ring cell carcinoma involving the urinary bladder. Report of a case and review of 21 cases

A case of signet-ring cell carcinoma involving the urinary bladder, which is a rare variant of adenocarcinoma, is presented. 21 cases have so far been reported in the occidental and Japanese journals. In 13 patients (62%) the carcinomas were of urachal origin and 8 (38%) of bladder origin. The prognosis of signet-ring cell carcinoma originating in bladder is poorer compared to that in urachus; 2-year survival of 40% for the bladder cancer group and 70% for the urachus. A radical cystectomy with excision of adjacent tissues, which might improve the prognosis in this fatal malignancy, should be considered.     

Combined endoscopic and laparoscopic en bloc resection of the urachus and the bladder dome in a rare case of urachal carcinoma

Urachal carcinoma is a rare neoplasm treated with surgical resection. We report a case of adenocarcinoma of the urachus treated with a new surgical technique. In a 44-year-old man affected by urachal carcinoma we performed a combined endoscopic-laparoscopic surgical en bloc resection of the urachus and bladder dome. The procedure lasted 240 min, and no postoperative complications were recorded. The patient was discharged on fourth day and the catheter was removed on eighth day. Bladder capability resulted normal with no evident physical change. Multiple bladder biopsy and computed tomography scans at 6, 12 and 18 months proved negative.     

Multimodality management of urachal carcinoma: the M. D. Anderson Cancer Center experience

PURPOSE: Enteric type adenocarcinomas arising in the dome of the bladder or along the urachal ligament are uncommon. To improve our understanding of urachal carcinoma and define outcome with current management, we performed a retrospective review of cases seen at the M. D. Anderson Cancer Center. MATERIALS AND METHODS: We reviewed the records of 42 patients with urachal carcinoma evaluated at our institution from 1985 to 2001. Specifically, we sought to evaluate the importance of extent of disease, surgical characteristics and systemic therapy on clinical outcome. RESULTS: Of the 42 patients 7 had clinically evident metastases at diagnosis and 35 had resectable disease that was managed initially with surgery. Overall survival from diagnosis for all 42 patients was 46 months with 40% surviving at 5 years. Of the resected cases 16 (46%) remain disease-free (median followup 31 months). Covariates associated with long-term survival were negative surgical margins (p = 0.004) and absence of nodal involvement (p = 0.01). Median survival from recognition of metastatic disease was 24 months in 26 patients in whom metastases ultimately developed. Chemotherapy for metastatic disease produced only 4 significant responses, including 3 of 9 patients treated with 5-fluorouracil and cisplatin containing regimens. CONCLUSIONS: Urachal carcinomas are usually locally advanced at presentation with a high risk of distant metastases. However, long-term survival following radical resection occurs in a significant fraction of patients (16 of 35 in our series), supporting an attempt at margin-negative, en bloc resection if at all possible. Chemotherapy appropriate for enteric type adenocarcinoma can induce objective responses but meaningful improvement in survival is not yet demonstrated.     

PARTIAL CYSTECTOMY DURING RADICAL SURGERY FOR NONUROLOGICAL MALIGNANCY

PURPOSE: It is not uncommon for a locally advanced, nonurological malignancy to invade the bladder. Partial cystectomy may be required to ensure complete tumor eradication. To our knowledge the true benefit of this procedure is unknown. MATERIALS AND METHODS: A total of 45 patients underwent partial cystectomy as part of radical surgery for a nonurological malignancy. We retrospectively reviewed these cases to determine which malignancies are prone to invade the bladder, the incidence of malignant invasion, the complication rate and the prognosis after wide en bloc resection. RESULTS: Colorectal adenocarcinoma accounted for the majority of cases. Tumor invaded only 11 bladder specimens (21.5%). Radical surgery was performed with curative intent in 30 patients, of whom 23 had negative surgical margins. At a mean followup of 30.7 months 16 of these 23 patients (69.6%) were free of disease or died of other disease processes. Disease progression and/or cancer related death occurred in 14 of the 15 patients (93.3%) who underwent surgery for palliation and in 16 of the 17 (94.1%) with positive margins at a mean of 21.7 months. Overall disease specific survival in those with malignant invasion was 27.3% compared to 41.2% when the bladder was fixed by a dense fibrous reaction only. No reported complications were related to partial cystectomy at followup. CONCLUSIONS: Advanced primary and recurrent nonurological malignancies often involve the bladder. Partial cystectomy may be necessary due to a dense fibrous reaction or direct tumor extension. While this distinction is made only after formal pathological results are reviewed, wide en bloc resection is necessary to ensure complete excision. When radical surgery is performed with curative intent and negative surgical margins are achieved, patients are likely to experience prolonged disease-free survival.     

先天性脐尿管异常疾病的诊治体会（附9例报告）

目的：探讨先天性脐尿管异常疾病的诊断与治疗方法。方法：根据病史并结合脐孔或膀胱美蓝注射试验、膀胱镜检查、瘘管造影、B超、CT等辅助检查方法诊断脐尿管异常疾病9例,并进行相应手术治疗。结果：手术及病理检查证实脐尿管瘘并感染3例,脐尿管囊肿并感染3例,脐尿管窦道并感染1例,膀胱顶部憩室1例,确诊后自动出院1例。患者术后排尿正常,8－18天痊愈出院,随访至今无复发及癌变。结论：脐孔或膀胱美蓝注射试验、膀胱镜检查、瘘管造影、B超、CT等辅助检查为有效的诊断措施;手术范围应以尽可能彻底切除脐尿管及其异常组织为宜。     

Urachal anomalies and tumor: clinical investigation of 14 cases

Diseases associated with persistent urachus are relatively rare. During the past 9-year period, there have been 14 patients with urachal disease consisting of 10 with urachal abscess and 4 with urachal cancer. The 10 patients with urachal abscess consisted of 7 males and 3 females aged 19-77 years (mean, 46 years). The 4 patients with urachal cancer consisted of 2 males and 2 females aged 48-81 years (mean, 57 years). As symptoms, lower abdominal pain was frequently observed in the patients with persistent urachus with abscess and gross hematuria in those with urachal cancer. Echo and magnetic resonance imaging (MRI) were useful for visualizing the lesion. Computed tomogtaphic (CT) scanning could not visualize the lesion in 2 patients. Nine patients underwent MRI, which visualized the lesion in all of them. As urachal abscess, an umbilical fistula was observed in 3 patients, urachal cyst in 4, and urachal diverticulum in 1. The preoperative diagnosis was urachal cancer in 6 patients, and pathological examination showed 4 patients with adenocarcinoma, 1 with inflammatory granuloma, and 1 with pseudosarcoma. Urachal abscess was treated by resection of the abscess in 6 patients, transurethral resection in 1, and resection of the umbilicus and urachus and total cystectomy in the other. Of the patients with urachal cancer, 1 underwent total cystectomy and the other 3 underwent total urachal resection and partial cystectomy. In 2 patients with persistent urachus with abscess, the differentiation between abscess and malignant tumor was difficult.     

Clinical investigation of 9 cases of urachal carcinoma]

Recently, we encountered 9 cases of urachal tumor and conducted clinical studies on its diagnosis and treatment. The chief complaint was gross hematuria in all cases. Computed tomographic scan was performed in 7 cases, and in all cases it revealed a tumor between the dome of the bladder and the Retzius' cavity. This finding strongly suggested a urachal tumor before pathological study. Magnetic imaging resonance (MIR) was performed in one of the 9 cases, and it indicated the extent of the tumor more clearly than computed tomography. Partial cystectomy + en bloc removal of the umbilicus and urachus was performed in 5 cases, radical cystectomy + en bloc removal of the umbilicus and urachus was performed in 2 cases, and radical cystectomy was performed in 2 cases. As postoperative therapy, radiotherapy was administered to 4 cases, and chemotherapy was administered to 4 cases. One case died from cancer after 4 years and 10 months, while the other 8 cases are being treated on an outpatient basis without any local recurrence. All 5 patients who underwent partial cystectomy are alive (mean survival time: 97 months). Thus we believe that cure can be achieved by partial cystectomy and adjuvant therapy. In addition, serum carcinoembryonic antigen is thought to be useful as an indicator of residual tumor or local recurrence.     

Laparoscopic management of urachal cysts in adulthood

PURPOSE: Managing persistent and symptomatic urachal anomalies requires wide surgical excision. Such intervention is recommended to prevent symptom recurrence and complications, most notably malignant degeneration. However, traditional open excision is associated with significant morbidity and prolonged convalescence. We report our experience with the laparoscopic excision of urachal remnants as a less morbid, minimally invasive surgical alternative. MATERIALS AND METHODS: Between October 1993 and December 1999, 4 patients with a mean age of 43.3 years who had a symptomatic urachal cyst underwent laparoscopic radical excision of the urachal remnant. Using 2, 10 mm. and 1 or 2, 5 mm. ports the urachus and medial umbilical ligaments were divided at the umbilicus cephalad to the cyst. The specimen, which included the urachus, cyst and medial umbilical ligaments, was then separated from the bladder dome with or without the bladder cuff and removed intact. We reviewed the perioperative records to assess morbidity, recovery and outcome. RESULTS: All 4 procedures were completed successfully. No intraoperative or postoperative complications were reported at a mean followup of 15 months (range 2 to 24). Mean operative time was 180 minutes (range 150 to 210) and average hospital stay was 2.75 days (range 1 to 4). Pathological evaluation confirmed a benign urachal remnant in each case. All patients resumed normal activity within 2 weeks. CONCLUSIONS: To minimize the morbidity of radical excision the laparoscopic management of benign urachal remnants in adulthood is efficacious and our preferred method of management.     

Laparoscopic partial cystectomy for various bladder pathologies

OBJECTIVE: To present our initial experience with laparoscopic partial cystectomy (LPC) in selected patients with various bladder pathologies. PATIENTS AND METHODS: Between December 2004 and April 2006, four patients had LPC at our centre (mean age 52 years, range 35-70); the transperitoneal approach was used for three and a pre-peritoneal approach for one. The surgical procedures used sequentially included transurethral incision around the lesion, laparoscopic excision of the lesion (partial cystectomy) and intracorporeal suturing. Laparoscopic pelvic lymphadenectomy was also used for the two patients with malignancy. RESULTS: All operations proceeded smoothly; the bladder pathologies included one bladder endometriosis, one bladder leiomyoma, one urothelial carcinoma within the bladder diverticulum and one urachal adenocarcinoma. The mean (range) operative duration was 197.5 (120-300) min, the estimated blood loss 70 (50-100) mL, the hospital stay 6.75 (5-9) days, and duration of Foley catheterization 7.25 (6-9) days. No open conversion was required and no patient had peri-operative complications. The surgical margins were free of cancer and the dissected lymph nodes were negative in those two patients with bladder malignancy. CONCLUSIONS: LPC is safe and feasible in selected patients with various bladder pathologies.     

Adenocarcinoma of the bladder (author's transl)]

During a 10 year follow-up, 18 patients with primary adenocarcinoma of the bladder or urachus were treated operatively. We report on the therapy and results. The criteria for differentiation between primary adenocarcinoma of the bladder and urachal carcinoma are stressed. Chronic irritation of the urothelium is most likely involved in the natural histroy of primary adenocarcinoma. Cystectomy should be the therapy of choice for the adenocarcinoma of the bladder. A partial resection is recommended for urachal carcinoma. Palliation is possible with cytotoxic agents. Irradiation seems to be of no beneficial effect on tumor growth.     

Extended resection for locally advanced colorectal carcinoma.

We reviewed the medical records of 101 patients who underwent extended resection for locally advanced colorectal carcinoma between 1965 and 1989. Preoperative symptoms related to the genitourinary system were present in 46 patients. Malignant invasion of genitourinary structures by colorectal carcinoma was found in 43 of these 46 patients (93%). In contrast, 51% of the patients without such symptoms had malignant invasion of contiguous structures. Preoperative intravenous pyelography, computerized tomographic scans, and cystoscopy correctly predicted the presence or absence of malignant invasion in 89%, 83%, and 87% of patients, respectively. Tumor-positive resection margins had a negative impact on survival (mean survival: 11.4 months). The 5-year actuarial survival rate for the patients who underwent a curative extended resection (margins tumor negative) was 54%. A thorough preoperative evaluation can identify a significant number of patients with colorectal cancer extending into adjacent organs and structures. Such evaluation is vital for operative planning and patient preparation, since an appropriate extended resection can produce long-term local control and patient survival.     

Manejo laparoscópico de las anomalías sintomáticas del uraco

IntroductionAcquired urachal anomalies are a rare pathology. Gold-standard treatment for this clinical situation remains the resection of the urachus in its entire tract with or without partial resection of the bladder. Our aim is to up-date authors ´s experience in the minimally invasive surgical treatment of acquired urachal disease.MethodsFrom 2001 to 2007, 14 patients were operated for acquired urachal disease at our institution. A three portal technique previously described by the authors was employed. The diagnosis of acquired uracal disease was confirmed in all cases and the resection of the urachus in its entire tract performed in cephalocaudal direction onto the bladder. Operative and demographic data was prospectively collected and analysis retrospectively performed.ResultsMean operative time was 63 minutes (45,110), minimal blood loss, and no conversions to open surgery or perioperative complications were verified. The majority of the patients were discharged in the first 24 hours. At a follow-up of 22 months no recurrences of urachal pathology recurrences have been verified.ConclusionsLaparoscopy plays a significant role in the management of symptomatic urachus anomalies. It allows objective confirmation of clinical diagnosis and adequate resection of the urachus in a safe and efficient fashion, while providing the well-known advantages of minimally acces surgery. Preoperative evaluation work-up has minimal impact of therapeutical decision.     

Surgical management and prognosis of renal cell carcinoma invading the vena cava

A total of 44 patients with renal cell carcinoma and vena caval tumor thrombus underwent surgical resection. Of these patients 27 had primary tumor confined within Gerota's fascia, negative lymph nodes and no distant metastases (stage T3cN0M0). Patients who underwent extraction of a mobile tumor thrombus from the vena cava had a 69% 5-year survival rate (median 9.9 years) but patients with tumor thrombus directly invading the vena cava had a 26% 5-year survival rate (median 1.2 years), which improved to 57% (median 5.3 years) if the involved vena caval side wall was resected successfully. Of these patients 17 had renal cell carcinoma with vena caval thrombus as well as extrafascial extension, regional lymphadenopathy or distant metastases, and the 5-year survival rate was less than 18% in all groups (median survival less than 0.9 years). Prognosis was determined by the pathological stage of the renal cell carcinoma and by the presence or absence of vena caval side wall invasion but not by the level of tumor thrombus extension. Patients with incomplete resection of localized renal cell carcinoma with tumor thrombus do not survive any longer than those with extensive cancer, positive lymph nodes or distant metastases. However, when partial venacavectomy establishes negative surgical margins then survival markedly improves.