Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.
J Urol. 2010 Jul;184(1):59-63. doi: 10.1016/j.juro.2010.03.035. Epub 2010 May 15.
Despite aggressive screening patients with hereditary renal cancers can present with large multifocal tumors. We present oncological outcomes in patients with hereditary renal cell carcinoma treated with partial nephrectomy for multifocal solid tumors with the largest lesion greater than 4 cm.
Between 1995 and 2008 we identified 58 patients with hereditary renal cell carcinoma treated at our institution with partial nephrectomy for solid tumors greater than 4 cm. Data collected included demographic parameters, tumor size, pathological findings and laterality. Overall and metastasis-free survival was calculated based on information from the most recent followup evaluation and imaging.
The cohort included 41 patients (71%) with von Hippel-Lindau disease, 10 (17%) with Birt-Hogg-Dubé syndrome and 7 (11%) with hereditary papillary renal carcinoma. Mean age was 43.7 years (range 18 to 63) and mean largest tumor size was 5.3 cm (range 4 to 13). A mean of 6.4 kidney tumors (range 1 to 44) was resected. There was a predominance of nuclear grade 2 tumors (51 cases or 85%) and clear cell histology (44 or 73%), followed by papillary type I histology (7 or 11.7%). Overall and metastasis-free survival rates were 93% and 96.5%, respectively, at a median followup of 45 months (range 2 to 163).
Metastasis-free and overall survival of our patients is similar to that in the literature of those who undergo partial nephrectomy for T1B tumors in the sporadic population. Multifocality does not affect oncological outcomes at intermediate followup. Partial nephrectomy can be offered to patients with hereditary disease who present with multifocal tumors greater than 4 cm.
尽管对遗传性肾癌患者进行了积极的筛查,但他们仍可能表现为多发的大肿瘤。我们报告了对最大肿瘤直径大于 4cm 的多发性实体肿瘤患者行部分肾切除术的肿瘤学结果,这些患者患有遗传性肾细胞癌。
1995 年至 2008 年间,我们在本机构共识别出 58 例接受部分肾切除术治疗最大肿瘤直径大于 4cm 的实体肿瘤的遗传性肾细胞癌患者。收集的数据包括人口统计学参数、肿瘤大小、病理发现和肿瘤侧别。根据最近的随访评估和影像学检查结果,计算总生存率和无转移生存率。
该队列包括 41 例(71%)von Hippel-Lindau 病患者、10 例(17%)Birt-Hogg-Dubé 综合征患者和 7 例(11%)遗传性乳头状肾细胞癌患者。患者的平均年龄为 43.7 岁(18 岁至 63 岁),最大肿瘤的平均直径为 5.3cm(4cm 至 13cm)。平均切除 6.4 个肾脏肿瘤(1 个至 44 个)。肿瘤核分级为 2 级(51 例或 85%)和透明细胞组织学(44 例或 73%)最为常见,其次是乳头状 I 型组织学(7 例或 11.7%)。中位随访 45 个月(2 个月至 163 个月)时,总生存率和无转移生存率分别为 93%和 96.5%。
我们的患者的无转移生存率和总生存率与在散发性人群中接受部分肾切除术治疗 T1B 肿瘤的文献报道相似。在中期随访中,多发病灶并不影响肿瘤学结果。对于最大肿瘤直径大于 4cm 的多发性肿瘤患者,可以向遗传性疾病患者提供部分肾切除术。