Fontanesi J, Pappo A S, Parham D M, Hurwitz C A, Rao B N, Luo X, Kun L E, Pratt C B
Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
Med Pediatr Oncol. 1996 Apr;26(4):264-7. doi: 10.1002/(SICI)1096-911X(199604)26:4<264::AID-MPO9>3.0.CO;2-H.
The role of irradiation in the management of synovial sarcoma (SS) in pediatric patients is evaluated. The review covers all children seen at St. Jude Children's Research Hospital between May 1969 and December 1992 with the diagnosis of soft tissue sarcoma, of the 37 patients with the subtype SS, 16 received irradiation for the management of primary site disease. There were four IRS Group I, six Group II, four Group III, and two Group IV patients receiving irradiation. Tumor grade included seven Grade II, and nine Grade III lesions. TMN staging identified eight T1 and eight T2 lesions. Follow-up has ranged from 14 to 117 months (med = 33 months). All IRS Group I patients had documented local control. Five of six IRS Group II and 4/4 Group III patients have had documented local control at last follow-up. IRS Group IV patients had either local control tumor stabilization (n=1) or evidence of tumor regression (n=1) at autopsy. Complications following irradiation include wound dehiscence (n=1), surgery to revise a painful scar (n=1) extremity length discrepancy (n=2), and femoral head avascular necrosis (n=1). At last follow-up, 10 of 14 patients receiving curative intent irradiation remain alive. This review indicates questionable benefit to the addition of irradiation for patients with adequate surgical resection and having "good" tumor characteristics (Grade I, II; IRS Group I, TMN T1A,T1B. For lesions that have had incomplete resection or partial response to chemotherapy, there is evidence that irradiation may provide durable local control. The role of irradiation in those patients with IRS Group IV disease is at present confined to palliative roles until the time when more effective chemotherapy will mandate the decision to treat primary disease for curative measures.
评估了放疗在小儿滑膜肉瘤（SS）治疗中的作用。该综述涵盖了1969年5月至1992年12月在圣裘德儿童研究医院就诊且诊断为软组织肉瘤的所有儿童，在37例SS亚型患者中，16例接受了放疗以治疗原发部位疾病。接受放疗的患者中有4例IRS I组、6例II组、4例III组和2例IV组患者。肿瘤分级包括7例II级和9例III级病变。TMN分期确定了8例T1和8例T2病变。随访时间为14至117个月（中位时间 = 33个月）。所有IRS I组患者均有局部控制的记录。6例IRS II组患者中有5例以及4/4例III组患者在最后一次随访时有局部控制的记录。IRS IV组患者在尸检时要么有局部控制肿瘤稳定（n = 1），要么有肿瘤消退的证据（n = 1）。放疗后的并发症包括伤口裂开（n = 1）、手术修复疼痛性瘢痕（n = 1）、肢体长度差异（n = 2）和股骨头缺血性坏死（n = 1）。在最后一次随访时，接受根治性放疗的14例患者中有10例仍存活。该综述表明，对于手术切除充分且具有“良好”肿瘤特征（I级、II级；IRS I组、TMN T1A、T1B）的患者，增加放疗的益处存疑。对于切除不完全或对化疗部分反应的病变，有证据表明放疗可能提供持久的局部控制。目前，放疗在IRS IV组疾病患者中的作用仅限于姑息治疗，直到更有效的化疗促使决定采取根治性措施治疗原发疾病。
