Department of Urology, Wake Forest University, Winston Salem, North Carolina, USA.
J Surg Oncol. 2012 Mar;105(3):258-60. doi: 10.1002/jso.22081. Epub 2011 Aug 30.
Urachal adenocarcinoma with peritoneal dissemination is an unusual presentation of a rare disease. It is associated with patients experiencing significant pain, poor outcomes, and historical median survival times between 12 and 24 months. We describe our 18-year experience in managing these patients with cytoreductive surgery (CRS) followed by hyperthermic intraperitoneal chemotherapy (HIPEC).
Five patients who underwent six CRS with HIPEC for disseminated urachal cancer were identified. Demographics, perioperative data, and oncologic results were reviewed.
All patients successfully completed CRS followed by HIPEC with Mitomycin C. Three patients had prior urachal mass excision and one had previous cystoprostatectomy with ileal conduit. At time of surgery, complete resection of all visible disease was only achieved in two patients. All patients developed local or distant disease recurrence at a median of 13 months postoperatively (range 7-31). The majority of patients (3/5) underwent postoperative intravenous chemotherapy for recurrence (2) or residual disease (1). All patients died of their disease, with median survival following date of surgery of 27 months (range 21-87). Symptomatic control of peritoneal disease was achieved in 2/5 (40%) of the cases.
Urachal adenocarcinoma with peritoneal dissemination is an aggressive, rare disease, which is uniformly fatal. In our experience, CRS followed by HIPEC with Mitomycin C may increase patient survival and palliation, although further treatment improvements are clearly required.
具有腹膜播散的脐尿管腺癌是一种罕见疾病的不常见表现。它与经历严重疼痛、不良预后的患者相关,历史中位生存时间在 12 至 24 个月之间。我们描述了我们在管理这些接受细胞减灭术(CRS)联合腹腔内热灌注化疗(HIPEC)治疗的患者方面的 18 年经验。
确定了 5 例接受 6 例腹膜播散性脐尿管癌的 CRS 联合 HIPEC 的患者。回顾了人口统计学、围手术期数据和肿瘤学结果。
所有患者均成功完成 CRS 联合 HIPEC 治疗,使用丝裂霉素 C。3 例患者先前有脐尿管肿块切除术,1 例有先前的膀胱前列腺切除术和回肠造口术。在手术时,仅 2 例患者完全切除了所有可见的疾病。所有患者均在术后中位时间 13 个月(范围 7-31)出现局部或远处疾病复发。大多数患者(3/5)因复发(2 例)或残留疾病(1 例)接受了术后静脉化疗。所有患者均死于疾病,术后中位生存时间为 27 个月(范围 21-87)。5 例患者中有 2 例(40%)获得了腹膜疾病的症状控制。
具有腹膜播散的脐尿管腺癌是一种侵袭性的罕见疾病,普遍致命。根据我们的经验,CRS 联合 HIPEC 联合丝裂霉素 C 可能会增加患者的生存时间和缓解程度,尽管显然需要进一步的治疗改善。