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集合管(贝利尼管)肾细胞癌:日本全国性调查

Collecting duct (Bellini duct) renal cell carcinoma: a nationwide survey in Japan.

作者信息

Tokuda Noriaki, Naito Seiji, Matsuzaki Osamu, Nagashima Yoji, Ozono Seiichiro, Igarashi Tatsuo

机构信息

Department of Urology, Saga Prefectural Koseikan Hospital, 1-12-9 Mizugae, Saga 840-8571, Japan.

出版信息

J Urol. 2006 Jul;176(1):40-3; discussion 43. doi: 10.1016/S0022-5347(06)00502-7.

Abstract

PURPOSE

Collecting duct carcinoma, a rare type of renal cell carcinoma, remains poorly understood. To analyze the nature of collecting duct carcinoma a retrospective survey was performed in Japan.

MATERIALS AND METHODS

This survey was done from August 2001 to April 2003. A total of 281 institutions throughout Japan were requested to document all cases of collecting duct carcinoma. All pertinent clinical information was compiled, including patient age, sex, mode of presentation, evaluation modality, preoperative laboratory data, surgery type, macroscopic and microscopic findings, and survival data. Two urological pathologists reviewed microscopic slides of all tumor specimens to confirm collecting duct carcinoma.

RESULTS

Two pathologists confirmed collecting duct carcinoma in 81 of the 120 cases documented as collecting duct carcinoma. Mean patient age was 58.2 years and males comprised 71.6% of all patients. The mode of presentation was classified as symptomatic in 65.4% of cases, incidental in 24.7% and not available in 9.9%. Regional lymph node metastasis was histologically detected in 44.2% of patients who underwent lymph node dissection, while 32.1% of the population had distant metastasis at presentation. Although postoperative adjuvant therapy against metastasis or recurrence was performed in 25 patients, no obvious responses were identified except in 1 with lung metastases, who showed a partial response to combined gemcitabine and carboplatin therapy. At a median followup of 15 months 1, 3, 5 and 10-year disease specific survival was 69.0%, 45.3%, 34.3% and 13.7%, respectively.

CONCLUSIONS

We report what is to our knowledge the largest known series of collecting duct carcinoma. Since advanced or recurrent collecting duct carcinoma is resistant to standard treatment modalities, new treatment strategies are needed for advanced collecting duct carcinoma.

摘要

目的

集合管癌是一种罕见的肾细胞癌类型,目前人们对其了解甚少。为分析集合管癌的性质,在日本开展了一项回顾性调查。

材料与方法

该调查于2001年8月至2003年4月进行。要求日本全国共281家机构记录所有集合管癌病例。收集了所有相关临床信息,包括患者年龄、性别、临床表现方式、评估方式、术前实验室数据、手术类型、大体和显微镜下所见以及生存数据。两名泌尿外科病理学家复查了所有肿瘤标本的显微镜切片以确诊集合管癌。

结果

两名病理学家在记录为集合管癌的120例病例中确诊了81例。患者平均年龄为58.2岁,男性占所有患者的71.6%。65.4%的病例临床表现为有症状,24.7%为偶然发现,9.9%未提及。在接受淋巴结清扫的患者中,44.2%经组织学检查发现有区域淋巴结转移,而32.1%的患者在初诊时已有远处转移。尽管25例患者接受了针对转移或复发的术后辅助治疗,但除1例肺转移患者对吉西他滨联合卡铂治疗有部分反应外,未发现明显疗效。在中位随访15个月时,1年、3年、5年和10年的疾病特异性生存率分别为69.0%、45.3%、34.3%和13.7%。

结论

我们报告了据我们所知最大的已知集合管癌系列病例。由于晚期或复发性集合管癌对标准治疗方式耐药,因此需要针对晚期集合管癌的新治疗策略。

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