Bansal Deepak, Totadri Sidharth, Chinnaswamy Girish, Agarwala Sandeep, Vora Tushar, Arora Brijesh, Prasad Maya, Kapoor Gauri, Radhakrishnan Venkatraman, Laskar Siddharth, Kaur Tanvir, Rath G K, Bakhshi Sameer
Pediatric Hematology Oncology Unit, Department of Pediatrics, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Department of Pediatric Oncology, Tata Memorial Hospital, Parel, Mumbai, India.
Indian J Pediatr. 2017 Jun;84(6):446-455. doi: 10.1007/s12098-017-2298-0. Epub 2017 Apr 3.
Neuroblastoma (NBL) is the most common extra-cranial solid tumor in childhood. High-risk NBL is considered challenging and has one of the least favourable outcomes amongst pediatric cancers. Primary tumor can arise anywhere along the sympathetic chain. Advanced disease at presentation is common. Diagnosis is established by tumor biopsy and elevated urinary catecholamines. Staging is performed using bone marrow and mIBG scan (FDG-PET/bone scan if mIBG unavailable or non-avid). Age, stage, histopathological grading, MYCN amplification and 11q aberration are important prognostic factors utilized in risk stratification. Low-risk disease including Stage 1 and asymptomatic Stage 2 disease has an excellent prognosis with non-mutilating surgery alone. Perinatal adrenal neuroblastoma may be managed with close observation alone. Intermediate-risk disease consisting largely of unresectable/symptomatic Stage 2/3 disease and infants with Stage 4 disease has good outcome with few cycles of chemotherapy followed by surgical resection. Paraspinal neuroblastomas with cord compression are treated emergently, typically with upfront chemotherapy. Asymptomatic Stage 4S disease may be followed closely without treatment. Organ dysfunction and age below 3 mo would warrant chemotherapy in 4S. High-risk disease includes older children with Stage 4 disease and MYCN amplified tumors. High-risk disease has a suboptimal outcome, though the survival is improving with multimodality therapy including autologous stem cell transplant and immunotherapy. Relapse after multimodality therapy is difficult to salvage. Late presentation, lack of transplant facility, malnutrition and treatment abandonment are additional hurdles for survival in India. The review provides a consensus document on management of NBL for developing countries, including India.
神经母细胞瘤(NBL)是儿童期最常见的颅外实体瘤。高危神经母细胞瘤被认为具有挑战性,是儿童癌症中预后最差的类型之一。原发性肿瘤可沿交感神经链的任何部位发生。就诊时出现晚期疾病很常见。通过肿瘤活检和尿儿茶酚胺升高来确诊。分期采用骨髓检查和间碘苄胍扫描(如果无法进行间碘苄胍扫描或间碘苄胍不摄取,则采用氟代脱氧葡萄糖正电子发射断层扫描/骨扫描)。年龄、分期、组织病理学分级、MYCN扩增和11q异常是风险分层中使用的重要预后因素。低危疾病包括1期和无症状的2期疾病,仅通过非致残性手术预后良好。围生期肾上腺神经母细胞瘤可仅通过密切观察进行处理。中危疾病主要包括不可切除/有症状的2/3期疾病以及4期疾病的婴儿,经过几个周期的化疗后进行手术切除,预后良好。伴有脊髓压迫的椎旁神经母细胞瘤需紧急治疗,通常先进行前期化疗。无症状的4S期疾病可密切观察而无需治疗。4S期出现器官功能障碍且年龄小于3个月者需要化疗。高危疾病包括患有4期疾病且肿瘤MYCN扩增的大龄儿童。高危疾病的预后不理想,不过通过包括自体干细胞移植和免疫治疗在内的多模式治疗,生存率正在提高。多模式治疗后复发难以挽救。就诊晚、缺乏移植设施、营养不良和放弃治疗是印度患者生存面临的额外障碍。本综述为包括印度在内的发展中国家提供了一份关于神经母细胞瘤管理的共识文件。