Pappo A S, Fontanesi J, Luo X, Rao B N, Parham D M, Hurwitz C, Avery L, Pratt C B
Department of Hematology-Oncology, St Jude Children's Research Hospital, Memphis, TN 38101-0318.
J Clin Oncol. 1994 Nov;12(11):2360-6. doi: 10.1200/JCO.1994.12.11.2360.
We reviewed the clinical records and pathologic findings of 37 children and adolescents with synovial sarcoma treated at our institution over a 30-year period to evaluate the prognostic significance of tumor size, invasiveness, histology, and other features.
The 20 male and 17 female patients with synovial sarcoma had a median age of 13.7 years at diagnosis. Primary tumor sites were the extremities (n = 27), trunk (n = 8), and head and neck (n = 2). Disease stage (clinical group) was as follows: group I, n = 21; group II, n = 7; group III, n = 4; and group IV, n = 5. Nineteen patients had invasive (T2) lesions, 20 had tumors more than 5 cm in diameter, and 14 had histologic grade 3 lesions. The estimated 5-year survival rate (+/- SE) for patients with group I or II disease was 80% +/- 9%, compared with 17% +/- 15% for those with group III or IV tumors (P = .0003). An exact log-rank test, adjusted for clinical group, showed that tumor invasiveness and grade independently predicted overall and progression-free survival (P < .05); tumor size was significantly correlated with progression-free survival. A borderline significant relationship with overall survival was found for both tumor size and histologic subtype (P = .09).
A controlled trial of adjuvant chemotherapy is merited in children with resected synovial sarcoma (clinical group I or II) who present with unfavorable clinicopathologic features such as large, invasive, or grade 3 lesions. Children with unresected or metastatic disease fare poorly despite multimodality therapy and require novel treatment approaches.
我们回顾了30年间在本机构接受治疗的37例儿童和青少年滑膜肉瘤患者的临床记录及病理结果，以评估肿瘤大小、侵袭性、组织学及其他特征的预后意义。
20例男性和17例女性滑膜肉瘤患者诊断时的中位年龄为13.7岁。原发肿瘤部位为四肢（n = 27）、躯干（n = 8）和头颈部（n = 2）。疾病分期（临床分组）如下：Ⅰ组，n = 21；Ⅱ组，n = 7；Ⅲ组，n = 4；Ⅳ组，n = 5。19例患者有侵袭性（T2）病变，20例患者肿瘤直径超过5 cm，14例患者组织学分级为3级。Ⅰ组或Ⅱ组疾病患者的估计5年生存率（±标准误）为80%±9%，而Ⅲ组或Ⅳ组肿瘤患者为17%±15%（P = .0003）。经临床分组校正的精确对数秩检验显示，肿瘤侵袭性和分级可独立预测总生存率和无进展生存率（P < .05）；肿瘤大小与无进展生存率显著相关。肿瘤大小和组织学亚型与总生存率均存在临界显著关系（P = .09）。
对于具有大的、侵袭性或3级病变等不良临床病理特征的切除性滑膜肉瘤患儿（临床Ⅰ组或Ⅱ组），辅助化疗的对照试验是有必要的。未切除或转移性疾病的患儿尽管接受了多模式治疗，预后仍较差，需要新的治疗方法。
