Rao B N, Hayes F A, Thompson E I, Kumar A P, Fleming I D, Green A A, Austin B A, Pate J W, Hustu H O
Division of Surgery, Hematology-Oncology, and Radiation Oncology, St. Jude Children's Research Hospital, Memphis, TN 38101.
Ann Thorac Surg. 1988 Jul;46(1):40-4. doi: 10.1016/s0003-4975(10)65849-3.
Approximately 10% of all cases of Ewing's sarcoma arise from a rib. Conventional management has included chest wall resection (3 or more ribs) and radiation therapy. These forms of therapy have led to complications such as scoliosis and local deformity. The addition of radiation therapy can result in damage to the lung and adjacent viscera and also potentiate pulmonary restrictive disease. Between 1971 and 1978, 9 patients were treated with surgery, radiation therapy, and combination chemotherapy (three- or four-drug regimen). Only 2 patients (22%) survive. Since 1979, 14 patients were entered into a new protocol consisting of sequential induction chemotherapy, followed by delayed surgical resection whenever feasible. Three patients had complete resection of their primary lesion at onset. Initially, 7 patients had either biopsy (N = 4) or incomplete chest wall resection N = 3). All 4 patients with biopsy only at diagnosis had excellent responses to induction chemotherapy, allowing delayed resection of the involved rib without chest wall resection. Overall, 12 of 14 patients (86%) treated since 1979 survive, with only 2 receiving radiation therapy for residual disease in the primary rib site.
约10%的尤因肉瘤病例起源于肋骨。传统治疗方法包括胸壁切除（3根或更多肋骨）和放射治疗。这些治疗方式导致了诸如脊柱侧弯和局部畸形等并发症。放射治疗的加入会导致肺部及邻近脏器受损，还会加重肺限制性疾病。1971年至1978年间，9例患者接受了手术、放射治疗及联合化疗（三药或四药方案）。仅2例患者（22%）存活。自1979年起，14例患者进入了一个新方案，该方案包括序贯诱导化疗，然后在可行时进行延迟手术切除。3例患者在发病时对原发灶进行了完整切除。最初，7例患者进行了活检（4例）或胸壁不完全切除（3例）。所有4例仅在诊断时进行活检的患者对诱导化疗反应良好，从而能够在不进行胸壁切除的情况下延迟切除受累肋骨。总体而言，自1979年以来接受治疗的14例患者中有12例（86%）存活，仅2例因原发肋骨部位的残留病灶接受了放射治疗。
