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[脐尿管癌。一种罕见的膀胱肿瘤类型]

[Cancer of the urachus. A rare form of tumors of the bladder].

作者信息

Vergos M, Messina M H, Lhomme Desages B, Chapuis O

机构信息

Service de Chirurgie viscérale, Hôpital National de Nouakchott, République Islamique de Mauritanie.

出版信息

J Urol (Paris). 1992;98(1):56-9.

PMID:1527402
Abstract

Cancers of the urachus are very infrequent, making up 0.1% to 0.7% of all bladder tumors. This type of tumor affects male subjects in 60% to 75% of cases, in the 5th or 6th decade of live. In more than 90% of all cases, the lesion is a mucosecretory adenocarcinoma developing from embryonic remains within the wall of the bladder and respecting the superficial urothelium of the bladder, which is affected only secondarily, contrary to what occurs in the other adenocarcinomas of the bladder. The pathogenesis remains controversial, especially in the role of urachus patency and of bladder carcinogens. The diagnosis is most often established late, because of a long clinical latency. It is mainly based on ultrasonography and CT scan. Explorations of the bladder are useful only at an advanced stage. The prognosis is very poor, survival at 5 years ranging from 6.5% to 25% according to the authors. The treatment is mainly surgical. Only extensive resection with partial cystectomy extending to the peri- and supravesical environment, including the peritoneum and the umbilicus, and associated with pelvic lymphadenectomy may give some hope. Chemotherapy and radiation therapy, either alone or adjuvant, are disappointing. The prognosis may be improved only by an early diagnosis.

摘要

脐尿管癌非常罕见,占所有膀胱肿瘤的0.1%至0.7%。这种类型的肿瘤在60%至75%的病例中影响男性患者,发病年龄在生命的第五或第六个十年。在所有病例中,超过90%的病变是黏液分泌性腺癌,由膀胱壁内的胚胎残余物发展而来,不累及膀胱浅表尿路上皮,膀胱浅表尿路上皮仅继发受累,这与膀胱其他腺癌的情况相反。其发病机制仍存在争议,尤其是脐尿管通畅和膀胱致癌物的作用。由于临床潜伏期长,诊断往往较晚。诊断主要基于超声检查和CT扫描。膀胱探查仅在晚期有用。预后非常差,根据作者的研究,5年生存率在6.5%至25%之间。治疗主要是手术治疗。只有进行广泛切除,包括部分膀胱切除术并延伸至膀胱周围和膀胱上环境,包括腹膜和脐部,并联合盆腔淋巴结清扫术,才可能带来一些希望。单独或辅助的化疗和放疗效果都不尽人意。只有早期诊断才能改善预后。

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