Cecchetto G, Carli M, Sotti G, Bisogno G, Dall'Igna P, Boglino C, Granata C, Antoniello L, Guglielmi M
Department of Pediatrics, Division of Pediatric Surgery, University of Padua, Padua, Italy.
Med Pediatr Oncol. 2000 Feb;34(2):97-101. doi: 10.1002/(sici)1096-911x(200002)34:2<97::aid-mpo4>3.0.co;2-8.
The goal of primary excision in soft tissue sarcomas is the complete removal of the tumor by a nonmutilating procedure. However, microscopic residuals may be left after a conservative procedure because of inadequate preoperative assessment or difficulties during the operation. The purpose of this report is to describe the treatment and the outcome in patients, enrolled in the Italian Cooperative Study RMS-88, with microscopic residuals after primary excision (IRS Group IIa).
Microscopic residuals were evident at histology in 52 of 90 patients who had a macroscopic complete primary excision: 25 rhabdomyosarcomas (RMS) and 27 nonrhabdo-soft tissue sarcomas (NRSTS). Eighteen patients were treated with primary reexcision (PRE) and chemotherapy (CT) using VA or IVA regimens; 27 patients received radiation therapy (RT; 40 Gy) and IVA; 7 children in whom PRE was not feasible and RT could not be administered for age <3 years were treated with CT (IVA) alone.
Of the 18 patients who underwent a successful PRE + CT, the local relapses were 3 (16.6%); of 27 cases who had RT + CT there were 4 local relapses (14.8%); 3 local relapses occurred in those 7 patients in whom CT alone was administered (43%).
Microscopic residuals after primary surgery were difficult to manage because of the absence of a measurable target. PRE represented the treatment of choice for children <3 years of age who cannot receive RT and for paratesticular sites. PRE and RT showed similar results in achieving local control in extremity and trunk sites, but they could not always avoid local recurrence. In particular PRE was not effective in tumors larger than 5 cm. If microscopic residuals could not be avoided and PRE was not possible, adequate RT was effective both for RMS and for NRSTS.
软组织肉瘤初次切除的目标是通过非致残性手术完整切除肿瘤。然而，由于术前评估不足或手术过程中的困难，保守手术后可能会残留微小病灶。本报告的目的是描述参与意大利合作研究RMS - 88的患者在初次切除后出现微小病灶残留（IRS IIa组）的治疗方法及结果。
90例肉眼下初次切除完整的患者中，52例在组织学检查时发现微小病灶残留：25例横纹肌肉瘤（RMS）和27例非横纹肌软组织肉瘤（NRSTS）。18例患者接受了初次再次切除（PRE）并采用VA或IVA方案进行化疗（CT）；27例患者接受了放射治疗（RT；40 Gy）并采用IVA方案；7例因年龄小于3岁无法进行PRE且不能接受RT的儿童仅接受了CT（IVA）治疗。
18例成功接受PRE + CT的患者中，局部复发3例（16.6%）；27例接受RT + CT的患者中有4例局部复发（14.8%）；7例仅接受CT治疗的患者中有3例局部复发（占43%）。
由于缺乏可测量的靶点，初次手术后的微小病灶残留难以处理。PRE是无法接受RT的3岁以下儿童以及睾丸旁部位的首选治疗方法。PRE和RT在实现肢体和躯干部位的局部控制方面显示出相似的结果，但它们并不能总是避免局部复发。特别是PRE对大于5 cm的肿瘤无效。如果无法避免微小病灶残留且无法进行PRE，适当的RT对RMS和NRSTS均有效。
