部分原因是他们的罕见,国家综合癌症网络(NCCN)对儿科癌症的准则落后于成人癌症的创造。然而,该组织正在追赶,其最近公布的儿科血液癌症指南,包括急性淋巴细胞白血病,霍奇金淋巴瘤和侵略性成熟的B细胞淋巴瘤。1-3此外,NCCN最近发布了Wilms肿瘤(肾细胞瘤)的第一个准则,罕见的实体肿瘤,患有约5%的儿童癌症和超过20年患者的肾癌的90%以上。4.

The panel of 25 experts from numerous US cancer centers synthesized the evidence on screening, diagnosis, risk stratification, and treatment, largely based on a number of trials dating back to 1969 led by the National Wilms Tumor Study Group, which later became the Children’s Oncology Group (COG). While making impressive inroads in treating Wilms tumors — 5-year survival is now greater than 90% — these trials have created a markedly complex treatment landscape.

“很明显,这是威尔斯肿瘤的一个实体代表了一种显着的生物学谱,其中治疗儿童可以只取出肿瘤和肾脏,或者可能需要非常密集的[治疗],具有长期影响的重大风险,”said Elizabeth Mullen, MD, program leader for renal tumors at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center and a member of the NCCN panel. “That is in part what makes treating this disease daunting and is exactly why it’s helpful to have these guidelines.”


继续阅读

值得注意的是,NCCN指南仅涉及具有有利组织学的肿瘤,并且不包括弥漫性包塑肿瘤,该肿瘤仅代表5%的胰肿瘤,并且Mullen博士统计学治疗差异较小。

从筛选开始,指南建议患有已知的易感条件的儿童每3个月经历体检和肾超声,直到至少8岁。高达20%的威尔姆斯肿瘤病例发生在具有易受令人遗症条件的儿童中,包括生长障碍Beckwith-Wiedemann综合征;Denys-Drash综合征,其特征在于雄性伪脉络系统和肾小球病;和WAGR综合征,其促使个体患有威尔姆斯肿瘤,Aniridia,泌尿病问题和发育延误。

这种筛选方法在早期检测肿瘤时非常成功,这使得更有可能是可能的。。。you’ll be able to successfully remove the tumor from their kidney without having to take the kidney out,” noted Frank M. Balis, MD, professor of pediatrics and Louis and Amelia Canuso family endowed chair for clinical research in Oncology at the Children’s Hospital of Philadelphia and the University of Pennsylvania’s Abramson Cancer Center, who chairs the NCCN guidelines panel for Wilms Tumor.