Geller James I, Ehrlich Peter F, Cost Nicholas G, Khanna Geetika, Mullen Elizabeth A, Gratias Eric J, Naranjo Arlene, Dome Jeffrey S, Perlman Elizabeth J
Division of Pediatric Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
Section of Pediatric Surgery, Department of Surgery, CS Mott Children's Hospital, University of Michigan School of Medicine, Ann Arbor, Michigan.
Cancer. 2015 Jul 15;121(14):2457-64. doi: 10.1002/cncr.29368. Epub 2015 Apr 6.
The current study was conducted to characterize the epidemiology, histology, and radiographic features of as well as the surgical approach to pediatric and adolescent renal cell carcinoma (pRCC).
pRCC cases prospectively enrolled on the Children's Oncology Group study AREN03B2 underwent central pathology, radiology, surgery, and oncology review.
As of June 2012, 120 of a total of 3250 patients enrolled on AREN03B2 (3.7%) were found to have unilateral RCC (median age, 12.9 years [range, 1.9-22.1 years]; 52.5% were female). Central review classified these as translocation morphology (56 patients), papillary (20 patients), renal medullary carcinoma (13 patients), chromophobe (4 patients), oncocytoma (1 patient), conventional clear cell (1 patient), and RCC not otherwise specified (25 patients). Lymph node (LN) involvement (N+) was found in 35 of 73 cases (47.9%) for which LNs were sampled, including 19 of 40 cases with primary tumors measuring <7 cm (47.5%). Using a size cutoff of 1 cm, imaging detection of LN involvement had a sensitivity of 57.14% (20 of 35 cases; 95% CI, 39.35%-73.68%) and a specificity of 94.59% (35 of 37 cases; 95% CI, 81.81%-99.34%). Distant metastases were present in 23 cases (19.2%). Initial surgery was radical nephrectomy in 88 patients (73.3%), nephron-sparing surgery in 18 patients (15.0%), and biopsy in 14 patients (11.7%). Compared with patients undergoing radical nephrectomy, those treated with nephron-sparing surgery were less likely to have LNs sampled (6 of 18 patients [33.3%] vs 65 of 88 patients [73.9%]; P = .002).
Translocation RCC is the most common form of pediatric and adolescent RCC. Lymph node disease is common and observed among patients with small primary tumors. Imaging has a high specificity but relatively low sensitivity for the detection of such lymph node disease. Failure to sample LNs results in incomplete staging and potentially inadequate disease control for younger patients with RCC.
本研究旨在描述儿童及青少年肾细胞癌（pRCC）的流行病学、组织学、影像学特征以及手术方式。
对前瞻性纳入儿童肿瘤协作组AREN03B2研究的pRCC病例进行中心病理学、放射学、手术及肿瘤学评估。
截至2012年6月，在AREN03B2研究纳入的3250例患者中，有120例（3.7%）被发现患有单侧肾细胞癌（中位年龄12.9岁[范围1.9 - 22.1岁]；52.5%为女性）。中心评估将这些病例分类为易位型（56例）、乳头状（20例）、肾髓质癌（13例）、嫌色细胞型（4例）、嗜酸细胞瘤（1例）、传统透明细胞型（1例）以及未另行指定的肾细胞癌（25例）。在73例进行了淋巴结采样的病例中，有35例（47.9%）发现有淋巴结转移（N+），其中在40例原发肿瘤直径<7 cm的病例中有19例（47.5%）。以1 cm为大小截断值，影像学检测淋巴结转移的敏感性为57.14%（35例中的20例；95%可信区间，39.35% - 73.68%），特异性为94.59%（37例中的35例；95%可信区间，81.81% - 99.34%）。远处转移见于23例（19.2%）。初始手术为根治性肾切除术的有88例（73.3%），保留肾单位手术的有18例（15.0%），活检的有14例（11.7%）。与接受根治性肾切除术的患者相比，接受保留肾单位手术的患者进行淋巴结采样的可能性较小（18例中的6例[33.3%]对88例中的65例[73.9%]；P = 0.002）。
易位型肾细胞癌是儿童及青少年肾细胞癌最常见的形式。淋巴结疾病常见，且在原发肿瘤较小的患者中也可观察到。影像学检测此类淋巴结疾病具有较高的特异性，但敏感性相对较低。未对淋巴结进行采样会导致分期不完整，并可能使年轻肾细胞癌患者的疾病控制不足。
