Department of Paediatric Surgery, Birmingham Children's Hospital NHS Trust, Birmingham, UK.
Eur J Cardiothorac Surg. 2012 Mar;41(3):630-4. doi: 10.1093/ejcts/ezr005. Epub 2011 Oct 18.
To identify factors determining the surgical outcome of primary cervical and thoracic neuroblastoma.
Twenty-six children with primary thoracic neuroblastoma presented over the last 14 years were analysed for age, mode of presentation, tumour histopathology, biology and outcome.
Primary thoracic neuroblastoma was presented in 16 boys and 10 girls at a median age of 2 years (range 6 weeks-15 years). The International Neuroblastoma Staging System (INSS) classified these as Stage 1 (8), Stage 2 (5), Stage 3 (6) and Stage 4 (7). Computed tomography defined the tumour location at the thoracic inlet (11), cervical (2), cervico-thoracic (3), mid-thorax (9) and thoraco-abdominal (1). Twenty-two children underwent surgery that allowed an adequate exposure and resection. Surgical resection was achieved after initial biopsy and preoperative chemotherapy in 15 children, whereas primary resection was performed in 7 children. Four patients with Stage 4 disease underwent chemotherapy alone after initial biopsy; of which, two died despite chemotherapy. Favourable outcome after surgical resection and long-term survival was seen in 19 (86.4%) of the 22 children. Three had local recurrence (14 to 21 months postoperatively), all with unfavourable histology on initial biopsy. The prognostic factors that determined the outcome were age and INSS stage at presentation. In this series, all patients under 2 years of age are still alive, while mortality was seen in five older children.
Thoracic neuroblastoma in children under 2 years of age irrespective of stage and histology of the tumour results in long-term survival.
确定原发性颈胸神经母细胞瘤手术结果的决定因素。
对过去 14 年来的 26 例原发性胸神经母细胞瘤患儿进行了年龄、表现方式、肿瘤组织病理学、生物学和结果分析。
16 名男孩和 10 名女孩中位年龄为 2 岁（范围 6 周-15 岁）出现原发性胸神经母细胞瘤。国际神经母细胞瘤分期系统（INSS）将这些病例分为Ⅰ期（8 例）、Ⅱ期（5 例）、Ⅲ期（6 例）和Ⅳ期（7 例）。计算机断层扫描定义了肿瘤位于胸入口（11 例）、颈部（2 例）、颈胸（3 例）、中胸（9 例）和胸腹（1 例）。22 名儿童接受了手术，这些手术允许充分暴露和切除肿瘤。在 15 名儿童中，初始活检和术前化疗后实现了手术切除，而在 7 名儿童中则进行了原发性切除。4 名Ⅳ期疾病患儿在初始活检后仅接受化疗；其中 2 人尽管接受了化疗仍死亡。22 例儿童中 19 例（86.4%）手术切除后预后良好且长期存活。3 例患儿术后 14 至 21 个月局部复发，所有患儿初始活检均为不良组织学。决定预后的预测因素是年龄和初始时的 INSS 分期。在本系列中，所有 2 岁以下的患儿均存活，而 5 名年龄较大的患儿死亡。
无论肿瘤的分期和组织病理学如何，2 岁以下儿童的胸神经母细胞瘤均导致长期存活。
