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Neuroblastoma: clinical and biological approach to risk stratification and treatment

机译：神经母细胞瘤：临床和生物学方法风险分层和治疗方法

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Neuroblastoma is the most common extra-cranial solid tumor of childhood and the most common in the first year of life. It is a unique malignancy in that infants often present with either localized or metastatic disease that can spontaneously regress without intervention while older children can succumb to the disease after months to years of arduous therapy. Given this wide range of outcomes, the International Neuroblastoma Risk Group was created to stratify patients based on presenting characteristics and tumor biology in order to guide intensity of treatment strategies. The goal has been to decrease therapy for low-risk patients to avoid long-term complications while augmenting and targeting therapies for high-risk patients to improve overall survival. The international risk stratification depends on age, stage, histology, MYCN gene amplification status, tumor cell ploidy and segmental chromosomal abnormalities. Treatment for asymptomatic low-risk patients with an estimated survival of >?98% is often observation or surgical resection alone, whereas intermediate-risk patients with an estimated survival of >?90% require moderate doses of response-adjusted chemotherapy along with resection. High-risk patients undergo multiple cycles of combination chemotherapy before surgery, followed by consolidation with myeloablative autologous hematopoietic stem cell transplantation and local radiation and finally immunotherapy with differentiation therapy as maintenance phase. With this approach, outcome for patients with neuroblastoma has improved, as the field continues to expand efforts in more targeted therapies for high-risk patients.
机译：神经母细胞瘤是儿童最常见的颅脑肿瘤，是生命的第一年中最常见的。这是一种独特的恶性肿瘤，婴儿通常存在于局部或转移性疾病，这些疾病无需干预，而年龄较大的儿童可能在数月遭受艰苦的治疗之后遭受侵害。鉴于这种广泛的结果，创建了国际神经母细胞瘤风险组，以基于呈现特征和肿瘤生物学来分析患者，以指导治疗策略的强度。目标是减少低风险患者治疗，以避免长期并发症，同时增强和针对高风险患者的治疗，以改善整体生存。国际风险分层取决于年龄，阶段，组织学，MyCN基因扩增状态，肿瘤细胞倍性和节段性染色体异常。无症状低风险患者的治疗估计的估计生存> 98％往往是单独观察或手术切除，而估计生存的中间风险患者> 90％需要中等剂量的响应调整后的化疗以及切除术。高风险患者在手术前进行多次组合化疗，然后用髓鞘性自体造血干细胞移植和局部辐射和最终用分化治疗作为维护阶段的免疫治疗。通过这种方法，由于神经母细胞瘤的患者的结果提高，因为该领域继续扩大对高危患者的更具针对性疗法的努力。

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