Henly D R, Farrow G M, Zincke H
Department of Urology, Mayo Clinic, Rochester, Minnesota.
Urology. 1993 Dec;42(6):635-9. doi: 10.1016/0090-4295(93)90526-g.
Urachal carcinoma is a rare neoplasm (0.22% of all bladder cancers) associated with a dismal prognosis. The obscure anatomic position of the tumor often minimizes early symptoms and precludes a timely diagnosis. The reported survival for urachal neoplasms has been inauspicious, and radical surgery (en bloc cystoprostatectomy and wide excision of the urachus and umbilicus) has been recommended as the primary treatment. To provide a rationale for the surgical management of this cancer, 38 patients with urachal carcinoma were reviewed, including 28 men and 10 women (2.8:1) (median age at diagnosis, 56 years; range, 28 to 88 years). The majority of the tumors were of high grade and stage at the time of diagnosis; they were exclusively DNA aneuploid in the 10 patients studied. Most patients had partial (segmental) cystectomy/umbilectomy (n = 30) or en bloc radical cystoprostatectomy/umbilectomy (n = 4) as their initial treatment; five-year overall survival of 43 percent and 50 percent revealed essentially comparable outcomes for the two surgical approaches. Thus, in the initial management of urachal carcinoma, umbilectomy with partial cystectomy may be considered in selected cases; this can enhance quality of life without necessarily influencing survival adversely. Rather, disease outcome seems more dependent on the adverse biologic potential of this usually DNA aneuploid tumor, necessitating the development of more innovative systemic treatment modalities.
脐尿管癌是一种罕见的肿瘤(占所有膀胱癌的0.22%),预后较差。肿瘤隐匿的解剖位置常常使早期症状不明显,难以及时诊断。据报道,脐尿管肿瘤的生存率不佳,根治性手术(整块膀胱前列腺切除术以及广泛切除脐尿管和脐部)被推荐作为主要治疗方法。为了为这种癌症的手术治疗提供理论依据,对38例脐尿管癌患者进行了回顾性研究,其中包括28名男性和10名女性(男女比例为2.8:1)(诊断时的中位年龄为56岁;范围为28至88岁)。大多数肿瘤在诊断时为高级别和高分期;在研究的10例患者中均为DNA非整倍体。大多数患者最初接受了部分(节段性)膀胱切除术/脐切除术(n = 30)或整块根治性膀胱前列腺切除术/脐切除术(n = 4);两种手术方法的五年总生存率分别为43%和50%,结果基本相当。因此,在脐尿管癌的初始治疗中,对于某些病例可考虑行脐切除术加部分膀胱切除术;这可以提高生活质量,而不一定会对生存产生不利影响。相反,疾病的预后似乎更取决于这种通常为DNA非整倍体肿瘤的不良生物学潜能,因此需要开发更具创新性的全身治疗方法。