| 1 | Ⅳ期神经母细胞瘤患者的远期预后显示文摘Aim of Study: Long-term outcome studies in survivors with stage IV neuroblastoma (NB) are sparse. This review evaluates late complications and long-term outcomes in stage IV NB survivors. Methods: A retrospective review of stage IV NB survivors was performed to analyze outcomes,including longterm morbidity,recurrence,and survival. Main Results: Of 153 patients with stage IV NB,52 (34% ) survived (male-female,26:26). Age at diagnosis was 29.1 ± 31.7 months in survivors. Eighteen were 1 year or younger and 34 were older than 1 year compared with 10 nonsurvivors 1 year or younger and 91 older than 1 year (P = 0.0003,Fisher’ s Exact test). Primary tumor sites were adrenal (35),retroperitoneal (11),mediastinal (3),pelvic (2),and no primary with tumor metastases identified (1). Ten survivors had favorable and 16 had unfavorable histology compared with 1 favorable and 18 unfavorable in nonsurvivors (P = 0.01). Four survivors had MYCN amplification (≥ 10 copies) and 2 deletions of 1p and 11q. Sites of metastasis in survivors and nonsurvivors were similar. Treatment in survivors included surgery in 51 (75% [39/51] complete tumor resection [CTR]); chemotherapy,50; radiation,17; stem cell transplantation,20; and bone marrow transplant,1. In nonsurvivors,13 (25% ) of 53 (P< 0.0001) had CTR,18 stem cell transplantation,and 12 bone marrow transplant. Six patients had tumor recurrence but survived (mean,9.3 ± 8.3 years; range,6 months-24 years). Recurrence was local (1),distant (2),and both (3) and was treated by resection,chemotherapy,and radiation. The mean age of survivors was 12.4 ± 8.3 years (range,2-34 years). In all stage IV cases,event-free survival was 30% and overall survival was 34% . Long-term complications occurred in 23 (44% ) survivors,including endocrine disturbances (7),orthopedic (5),cataracts (2),adhesive bowel obstruction (2),hypertension (1),bronchiolitis (1),blindness (1),peripheral neuropathy (1),nonfunctioning kidney (1),cholelithiasis (1),and thyroid nodule (1). Conclusion: Only 34% of patients with stage IV NB survived despite aggressive multimodal therapy. Age of younger than 1 year,favorable pathology,CTR,and no recurrence were the only statistically significant factors that favored survival. Forty-four percent of survivors experienced late morbidity,and tumor recurred in 6 (11.5% ) of 52. Patients should be monitored for tumor recurrence and long-term sequelae. New methods of treatment are required to achieve better outcomes. | Escobar M.A. Grosfeld J.L. Powell R.L. 刘凯 | 2006 | 世界核心医学期刊文摘(儿科学分册)2006，0，9： | 0 |
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