Méjean Arnaud, Rouprêt Morgan, Larousserie Frédérique, Hopirtean Vincent, Thiounn Nicolas, Dufour Bertrand
Department of Urology, Hospital Necker, Paris, France.
J Urol. 2003 Apr;169(4):1287-90. doi: 10.1097/01.ju.0000050221.51509.f5.
We describe diagnostic and therapeutic characteristics, and long-term followup of collecting (Bellini) duct carcinoma.
Ten patients underwent surgery, including radical nephrectomy in 9 and partial nephrectomy in 1, for collecting duct carcinoma diagnosed by histological and immunohistochemical criteria.
Collecting duct carcinoma, which represented 1.3% of renal cell carcinomas diagnosed between 1986 and 1999, developed at a mean patient age of 66.2 years (range: 50.7 to 81). It was detected clinically but never because of macroscopic hematuria. Mean tumor size was 94 mm. (range 30 to 150). Stage was pT3, pT2 and pT1 in 7, 1 and 2 cases, respectively. Seven patients had lymph node or extranodal metastases, and 8 and 2 had Fuhrman grades 3 and 4 disease, respectively. Eight patients died, including 3 early during the perioperative or postoperative period, which was attributable to major surgical difficulties associated with these invasive tumors. Two of the 3 patients who did not have metastases were alive 99 and 100 months after surgery, respectively. Median overall survival +/- SD was 9 +/- 6 and 6 +/- 8 months for patients with metastases. The all survival rate was 20% at 2 years.
Most collecting duct carcinomas are already metastatic at presentation. Because the prognosis is dismal despite radical nephrectomy, biopsy should be performed first when radiological findings are suggestive of collecting duct carcinoma. For metastatic collecting duct carcinoma radical nephrectomy alone does not seem to be useful except for palliative reasons or in the framework of new multicentric chemotherapy protocols in progress.
我们描述集合管(贝氏管)癌的诊断和治疗特点以及长期随访情况。
10例患者接受了手术,其中9例行根治性肾切除术,1例行部分肾切除术,均为经组织学和免疫组化标准诊断为集合管癌的患者。
集合管癌占1986年至1999年间诊断的肾细胞癌的1.3%,患者平均年龄为66.2岁(范围:50.7至81岁)。临床发现该疾病,但从未因肉眼血尿发现。肿瘤平均大小为94毫米(范围30至150毫米)。分期方面,7例为pT3,1例为pT2,2例为pT1。7例患者有淋巴结或结外转移,8例和2例分别为Fuhrman 3级和4级疾病。8例患者死亡,其中3例在围手术期或术后早期死亡,这归因于与这些侵袭性肿瘤相关的重大手术困难。3例无转移的患者中,2例分别在术后99个月和100个月存活。有转移患者的中位总生存期±标准差为9±6个月和6±8个月。2年总生存率为20%。
大多数集合管癌在初诊时已发生转移。尽管行根治性肾切除术预后仍不佳,但当影像学检查结果提示集合管癌时,应首先进行活检。对于转移性集合管癌,除姑息性原因或在正在进行的新的多中心化疗方案框架内,单纯根治性肾切除术似乎并无益处。