Raney R B, Ragab A H, Ruymann F B, Lindberg R D, Hays D M, Gehan E A, Soule E H
Cancer. 1982 Jun 15;49(12):2612-6. doi: 10.1002/1097-0142(19820615)49:12<2612::aid-cncr2820491234>3.0.co;2-e.
Disease-free survival data were obtained on 30 children with soft-tissue sarcoma of the trunk. The children were clinically grouped and treated in accord with the IRS protocol (Cancer 1977; 40:2015) from November 1972 through December 1976. Histologically, 40% of the tumors were alveolar rhabdomyosarcoma (RMS), 20% were embryonal RMS, 20% were extraosseous Ewing's sarcoma, 17% were undifferentiated sarcoma, and 3% were pleomorphic RMS. In 13 patients with primary tumors of the chest wall, the male-to-female (M:F) ratio was 3:11, and the median age was 12.5 yr. Five of the ten patients (50%) with localized tumor (Groups I-III) were disease-free at a median of 4.7 yrs. after diagnosis; two died of locally recurrent tumor, two died of metastases to lungs or bones, and 1 died of intracranial hemorrhage. None of the four with chest wall tumors and distant metastases (Group IV) survived. In ten patients with localized paraspinal primary tumors, the M:F ratio was 8:2, and the median age was 3.5 yrs. None had metastases. Seven of ten (70%) were disease-free at a median of 4.7 yrs. after diagnosis. Two died after regional recurrence developed (one retroperitoneal, one meningeal), and one died of lung metastases. In six patients with primary tumors of the abdominal wall, the M:F ratio was 3:3 and the median age was 9.5 yrs. Three of five with localized tumor (Groups I-III) were disease-free at a median of 5 yr. after diagnosis; one died after regional recurrence, and one died from accidental trauma. The one Group IV patient died of tumor. Overall, 15 of 30 patients (50%) are alive and free of recurrent disease at a median of 5+ yr. after initiation of treatment. The authors conclude that prognosis is most favorable for patients with paraspinal tumors, least favorable for those with chest wall tumors, and intermediate in patients with abdominal wall tumors. Prognostic differences appeared to be influenced by extent of disease at diagnosis and histologic subtype of the sarcoma.
我们获取了30例躯干软组织肉瘤患儿的无病生存数据。这些患儿于1972年11月至1976年12月期间按照IRS方案（《癌症》1977年；40:2015）进行临床分组和治疗。组织学上，40%的肿瘤为肺泡状横纹肌肉瘤（RMS），20%为胚胎型RMS，20%为骨外尤文肉瘤，17%为未分化肉瘤，3%为多形性RMS。在13例胸壁原发性肿瘤患者中，男女比例（M:F）为3:11，中位年龄为12.5岁。10例局限性肿瘤（I - III组）患者中有5例（50%）在诊断后中位4.7年时无病生存；2例死于局部复发肿瘤，2例死于肺或骨转移，1例死于颅内出血。4例胸壁肿瘤并有远处转移（IV组）患者均无存活。10例局限性脊柱旁原发性肿瘤患者中，男女比例为8:2，中位年龄为3.5岁。均无转移。10例中有7例（70%）在诊断后中位4.7年时无病生存。2例在出现局部复发后死亡（1例腹膜后复发，1例脑膜复发），1例死于肺转移。6例腹壁原发性肿瘤患者中，男女比例为3:3，中位年龄为9.5岁。5例局限性肿瘤（I - III组）中有3例在诊断后中位5年时无病生存；1例死于局部复发，1例死于意外创伤。IV组的1例患者死于肿瘤。总体而言，30例患者中有15例（50%）在开始治疗后中位5 +年时存活且无复发性疾病。作者得出结论，脊柱旁肿瘤患者的预后最为良好，胸壁肿瘤患者的预后最差，腹壁肿瘤患者的预后居中。预后差异似乎受诊断时疾病范围和肉瘤组织学亚型的影响。
