Adams G A, Shochat S J, Smith E I, Shuster J J, Joshi V V, Altshuler G, Hayes F A, Nitschke R, McWilliams N, Castleberry R P
Stanford University Medical Center, CA.
J Pediatr Surg. 1993 Mar;28(3):372-7; discussion 377-8. doi: 10.1016/0022-3468(93)90234-c.
Ninety-six patients with thoracic neuroblastoma were studied in a prospective fashion. Median age at presentation was 0.9 years. Forty-eight percent of the patients presented with stage A disease, 20% stage B, 13% stage C, 17% stage D, and 2% stage DS. Seventy-five patients have been followed for greater than 4 years. A posterior mediastinal mass was diagnosed on incidental chest roentgenograms performed for nontumor-related symptoms in 49% of the cases. Sixteen percent of the cases presented with neurological symptoms and 14% of the patients presented with acute respiratory distress. Urinary catecholamines were elevated in 76% of the cases. Complete surgical resection was carried out in 47% of the cases, while incomplete resection or biopsy was performed in 45%. No operation was performed in 3 patients. Minor surgical complications occurred in 20% of the patients, and 3% of the patients had significant perioperative complications. One patient died as a complication of therapy. Overall actuarial survival was 88% at 4 years. This study confirms the favorable outcome in children with mediastinal neuroblastoma. The basic biology of thoracic neuroblastomas seems to differ from that of other sites in that the majority of patients present at a younger age with localized disease or regional lymph node metastases, and have an improved survival even after correcting for age and stage. While complete excision is recommended, if possible, radical surgical procedures are not indicated since an excellent prognosis is associated with combined modality therapy.
对96例胸段神经母细胞瘤患者进行了前瞻性研究。就诊时的中位年龄为0.9岁。48%的患者表现为A期疾病，20%为B期，13%为C期，17%为D期，2%为DS期。75例患者随访时间超过4年。49%的病例因非肿瘤相关症状行胸部X线检查时偶然发现后纵隔肿块。16%的病例出现神经症状，14%的患者出现急性呼吸窘迫。76%的病例尿儿茶酚胺升高。47%的病例进行了完整的手术切除，45%进行了不完全切除或活检。3例患者未进行手术。20%的患者发生了轻微手术并发症，3%的患者出现了严重的围手术期并发症。1例患者死于治疗并发症。4年总精算生存率为88%。本研究证实了纵隔神经母细胞瘤患儿的良好预后。胸段神经母细胞瘤的基本生物学特性似乎与其他部位不同，因为大多数患者就诊时年龄较小，表现为局限性疾病或区域淋巴结转移，即使校正年龄和分期后生存率仍有所提高。虽然建议尽可能进行完整切除，但由于联合治疗预后良好，不建议进行根治性手术。
