Rich D C, Corpron C A, Smith M B, Black C T, Lally K P, Andrassy R J
Division of Pediatric Surgery, University of Texas Houston Health Science Center, USA.
J Pediatr Surg. 1997 Feb;32(2):369-72. doi: 10.1016/s0022-3468(97)90213-x.
Currently, approximately 67% of children diagnosed with cancer can be expected to survive more than 5 years. Among the most significant late effects of cancer therapy is the development of second malignant neoplasm (SMN). This study was performed to identify the factors associated with the development of second malignant neoplasms after treatment for soft tissue sarcomas in childhood. Retrospectively the charts of 20 children who developed second malignant neoplasms after treatment for primary childhood soft tissue sarcoma were reviewed. Presentation, age at diagnosis, tumor histology, extent of tumor, treatment, family histories (when available), and outcome were recorded. The mean age of the patients (10 boys, 10 girls) was 8.5 years of age (range, 1 to 20 years). Most primary tumors were rhabdomyosarcoma (14/20) and occurred in an extremity (10/20). Ninety percent of the patients (18/20) had a complete response to treatment of the primary cancer. Eleven out of 20 received combined chemotherapy and radiation therapy. The most common secondary malignancy was a bone sarcoma (6/20), followed by brain tumors (n = 3), leukemia (n = 2), and other sarcomas (n = 2). Four of the bone sarcomas developed in the field of radiation treatment. Median follow-up was 16 years (range, 1 to 26 years). The median time to development of a SMN was 11.4 years (range, 1.5 to 21 years). Survival after a second malignancy was only 30%. Two patients developed a third malignant neoplasm. The occurrence of a secondary malignancy represents a serious complication of childhood cancer. Certain tumors are related directly to treatment such as osteosarcoma within irradiated fields and secondary leukemias or lymphomas after certain chemotherapy regimens. Combined radiotherapy and chemotherapy may play an additive role in the development of second malignant neoplasms. Genetic factors may predispose affected patients to the development of both primary and secondary malignancies. Close surveillance of children previously treated for childhood cancers is warranted.
目前，预计约67%被诊断患有癌症的儿童能够存活超过5年。癌症治疗最显著的晚期效应之一是第二原发性恶性肿瘤（SMN）的发生。本研究旨在确定儿童软组织肉瘤治疗后发生第二原发性恶性肿瘤的相关因素。回顾性地查阅了20例儿童原发性软组织肉瘤治疗后发生第二原发性恶性肿瘤的病历。记录了临床表现、诊断时的年龄、肿瘤组织学、肿瘤范围、治疗情况、家族史（如有）及转归。患者平均年龄为8.5岁（10名男孩，10名女孩；年龄范围1至20岁）。大多数原发性肿瘤为横纹肌肉瘤（14/20），且发生于四肢（10/20）。90%的患者（18/20）对原发性癌症治疗有完全缓解。20例中有11例接受了联合化疗和放疗。最常见的继发性恶性肿瘤是骨肉瘤（6/20），其次是脑肿瘤（n = 3）、白血病（n = 2）和其他肉瘤（n = 2）。4例骨肉瘤发生在放疗区域。中位随访时间为16年（范围1至26年）。发生SMN的中位时间为11.4年（范围1.5至21年）。发生第二原发性恶性肿瘤后的生存率仅为30%。2例患者发生了第三种恶性肿瘤。继发性恶性肿瘤的发生是儿童癌症的严重并发症。某些肿瘤与治疗直接相关，如放疗区域内的骨肉瘤以及某些化疗方案后的继发性白血病或淋巴瘤。联合放疗和化疗可能在第二原发性恶性肿瘤的发生中起累加作用。遗传因素可能使受影响患者易患原发性和继发性恶性肿瘤。对曾接受儿童癌症治疗的儿童进行密切监测是必要的。
