Walther M M, Choyke P L, Glenn G, Lyne J C, Rayford W, Venzon D, Linehan W M
Urologic Oncology Branch, Department of Radiology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.
J Urol. 1999 May;161(5):1475-9. doi: 10.1016/s0022-5347(05)68930-6.
Patients with hereditary forms of renal cancer are at risk for new tumors and metastases. Renal parenchymal sparing surgery has been performed to preserve renal function and quality of life, and prevent metastases. We evaluated a 3 cm. threshold for performing renal parenchymal sparing surgery in patients with von Hippel-Lindau disease and hereditary papillary renal cancer.
Patients with von Hippel-Lindau disease or hereditary papillary renal cancer and renal cancer were identified by screening affected kindred and by kindred history. Patients with small tumors were followed with serial imaging studies until the largest renal tumor was 3 cm., when renal parenchymal sparing surgery was performed. Renal tumors greater than 3 cm. were resected without delay. Parenchymal sparing techniques were used when possible in each group.
The 3 cm. surgical threshold was evaluated in 52 patients with von Hippel-Lindau disease (group 1) at a median followup of 60 months (range 6 to 205). None of these patients had metastatic disease and none has required renal transplantation or dialysis. In 44 patients with von Hippel-Lindau disease (group 2) renal tumors larger than 3 cm. developed. Median followup from the initial radiological diagnosis of renal cancer in this group was 66.5 months (range 0 to 321). Patients in group 1 underwent parenchymal sparing surgery instead of nephrectomy more frequently than those in group 2 (46 of 48 operations or 96% versus 45 of 72 or 63%, Fisher's exact test p <0.0001). In contrast to patients in group 1, metastatic renal cancer developed in 11 of the 44 in group 2 (25%) (Fisher's exact test p <0.0001). A total of 23 patients with hereditary papillary renal cancer were also identified. Median followup in these cases was 44 months (range 0 to 237). Ten patients had tumors less than 3 cm. No patient with tumors less than 3 cm. and 2 of the 13 (15%) with larger tumors had metastases.
Using a 3 cm. renal tumor diameter as an indication for renal surgery no patient with renal cancer and von Hippel-Lindau disease or hereditary papillary renal cancer had metastatic disease regardless of the number of tumors. Using a lesion size of 3 cm. as a threshold for performing renal parenchymal sparing surgery may help to prevent metastatic disease, unnecessary renal damage due to frequent surgery and renal dialysis or transplantation.
遗传性肾癌患者有发生新肿瘤和转移的风险。已开展保留肾实质手术以保留肾功能和生活质量，并预防转移。我们评估了对患有冯·希佩尔-林道病和遗传性乳头状肾癌的患者进行保留肾实质手术的3厘米阈值。
通过筛查受影响的亲属和家族病史来确定患有冯·希佩尔-林道病或遗传性乳头状肾癌及肾癌的患者。对小肿瘤患者进行系列影像学检查随访，直至最大肾肿瘤达到3厘米，此时进行保留肾实质手术。直径大于3厘米的肾肿瘤立即切除。每组尽可能采用保留实质技术。
对52例冯·希佩尔-林道病患者（第1组）评估了3厘米的手术阈值（中位随访时间为个月6月，范围6至205个月）。这些患者均无转移性疾病，也无患者需要肾移植或透析。在44例冯·希佩尔-林道病患者（第2组）中出现了大于3厘米的肾肿瘤。该组从肾癌初始影像学诊断开始的中位随访时间为66.5个月（范围0至321个月）。第1组患者比第2组患者更频繁地接受保留实质手术而非肾切除术（48例手术中的46例，即96%，对比72例中的45例，即63%，Fisher精确检验p<0.0001）。与第1组患者不同，第2组44例中的11例（25%）发生了转移性肾癌（Fisher精确检验p<0.0001）。还共确定了23例遗传性乳头状肾癌患者。这些病例的中位随访时间为44个月（范围0至237个月）。10例患者肿瘤小于3厘米。肿瘤小于3厘米的患者均无转移，13例肿瘤较大的患者中有2例（15%）发生转移。
以3厘米肾肿瘤直径作为肾手术指征，患有肾癌和冯·希佩尔-林道病或遗传性乳头状肾癌的患者无论肿瘤数量多少均无转移性疾病。以3厘米的病变大小作为进行保留肾实质手术的阈值可能有助于预防转移性疾病、因频繁手术导致的不必要肾损伤以及肾透析或移植。
