Stea B, Kinsella T J, Triche T J, Horvath K, Glatstein E, Miser J S
Radiation Oncology, Branch, National Cancer Institute, Bethesda, MD 20892.
Int J Radiat Oncol Biol Phys. 1987 Dec;13(12):1797-805. doi: 10.1016/0360-3016(87)90344-0.
Adolescent and young adult patients with pelvic sarcomas continue to have a poor prognosis with standard combination chemotherapy and local irradiation. In addition to a significant risk of local failure, these patients are at high risk for systemic relapse. Twenty-three consecutive patients with Ewing's sarcoma, alveolar rhabdomyosarcoma, undifferentiated sarcoma, or malignant peripheral neuroepithelioma originating in the pelvis were treated with short, intensive combined modality therapy. This approach integrates 5 cycles of VADRIAC chemotherapy (Vincristine, Adriamycin, Cyclophosphamide) with high dose irradiation to the primary lesion (55-60 Gy) and sites of gross metastatic disease (45-50 Gy). Following achievement of a complete response, intensification therapy consisting of total body irradiation (TBI) (8.0 Gy), high dose VADRIAC chemotherapy, and autologous bone marow transplantation is given. All therapy is completed within 6-7 months. No maintenance chemotherapy is given; no surgery is intended. Of the twenty-three patients with pelvic sarcomas treated on this combined modality protocol, 22 achieved a complete remission. Local control was achieved and maintained in all twenty-three patients. With a median follow-up of 21 months since initiation of treatment, there have been nine relapses (all systemic). Seven relapses occurred among the thirteen patients who presented with overt metastatic disease and the other two relapses were among the ten patients with localized disease at presentation. All seven metastatic patients who relapsed have died, whereas both of the relapsed localized patients remain alive. Acute and late toxicities have been acceptable using this aggressive combined modality approach. Induction chemotherapy had a significant impact on reduction of the typically large (greater than 10 cm diameter) soft tissue mass associated with these pelvic tumors, thus facilitating achievement of local control by high dose irradiation. Of 18 patients with measureable soft tissue tumor, all experienced a partial response (greater than 50% reduction in size) following the initial two cycles of chemotherapy given prior to local irradiation. In conclusion, this short, intensive chemoradiotherapeutic regimen is highly effective in controlling the primary lesion (100% local control) and inducing a complete response in a high proportion (96%) of these high risk pediatric and young adult patients with pelvic sarcomas. The role of TBI as "systemic" adjuvant therapy to control micrometastatic disease is discussed as still under investigation.
患有盆腔肉瘤的青少年和年轻成年患者采用标准联合化疗和局部放疗的预后仍然很差。除了局部复发风险很高外，这些患者还存在全身复发的高风险。连续23例起源于盆腔的尤因肉瘤、肺泡横纹肌肉瘤、未分化肉瘤或恶性外周神经上皮瘤患者接受了短期强化综合治疗。这种方法将5个周期的VADRIAC化疗（长春新碱、阿霉素、环磷酰胺）与对原发灶（55 - 60 Gy）和肉眼可见转移病灶部位（45 - 50 Gy）的高剂量放疗相结合。在达到完全缓解后，给予包括全身照射（TBI）（8.0 Gy）、高剂量VADRIAC化疗和自体骨髓移植的强化治疗。所有治疗在6 - 7个月内完成。不进行维持化疗；也不打算进行手术。在按照这种综合治疗方案治疗的23例盆腔肉瘤患者中，22例实现了完全缓解。所有23例患者均实现并维持了局部控制。自治疗开始以来，中位随访时间为21个月，出现了9次复发（均为全身复发）。13例出现明显转移病灶的患者中有7例复发，另外2例复发发生在初诊时为局限性疾病的10例患者中。所有7例复发的转移患者均已死亡，而2例复发的局限性患者仍然存活。采用这种积极的综合治疗方法，急性和晚期毒性反应均可接受。诱导化疗对减少这些盆腔肿瘤通常较大（直径大于10 cm）的软组织肿块有显著影响，从而有助于通过高剂量放疗实现局部控制。在18例可测量软组织肿瘤的患者中，所有患者在局部放疗前给予的最初两个周期化疗后均出现部分缓解（大小缩小超过50%）。总之，这种短期强化放化疗方案在控制原发灶（100%局部控制）以及使这些高风险的儿童和年轻成年盆腔肉瘤患者中高比例（96%）实现完全缓解方面非常有效。TBI作为控制微转移疾病的“全身”辅助治疗的作用仍在研究中。
