Hameury Frédéric, Marec-Berard Perrine, Eymery Mathilde, Wijnen Marc H W, van der Kaaij Niels, Mure Pierre-Yves, Tronc François, Chotel Franck, Libbrecht Clara, van Boven Wim Jan P, Haveman Lianne M
Department of Pediatric Surgery, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Claude Bernard University, 69677 Bron, France.
Institute of Hematology and Pediatric Oncology, 69008 Lyon, France.
Cancers (Basel). 2021 Jul 21;13(15):3655. doi: 10.3390/cancers13153655.
Pediatric sarcoma patients with pleuropulmonary lesions have a dismal prognosis because the impossibility to obtain local control. The aim of this study was to determine if pleuropneumonectomy (PP) could be a therapeutic option. We retrospectively reviewed nine patients who underwent salvage PP for pleuropulmonary localization of primary localized sarcoma or metastatic recurrence. Surgery and complications were analyzed, pulmonary function tests were conducted, and quality of life was determined with EORTC-QLQ-C30 questionnaire. At the time of PP age was between 9-17 years. Underlying disease included metastatic osteosarcoma ( = 5), Ewing sarcoma (two metastatic, one primary), and one primary undifferentiated sarcoma. Early complications occurred in three patients. Mean postoperative hospitalization stay was 14.5 days. Pulmonary function test showed 19-66% reduction of total lung capacity which led to mild exercise intolerance but did not affect daily life. Four patients died of multi-metastatic relapse <14 months after PP, one patient had a local recurrence, and four patients are in complete remission between 1.5 and 12 years after PP. In conclusion, in this small patient group treated with a pleuropneumonectomy for primary or metastatic lesions, outcome is variable; however, this extended surgical technique was generally quite well tolerated. Postoperative lung function seems well preserved, and it seems to lead to at least an extension of life with good quality and therefor can be considered as salvage therapy.
患有胸膜肺部病变的小儿肉瘤患者预后不佳，因为无法实现局部控制。本研究的目的是确定胸膜肺切除术（PP）是否可以作为一种治疗选择。我们回顾性分析了9例因原发性局限性肉瘤或转移性复发的胸膜肺部定位而接受挽救性PP的患者。分析了手术及并发症情况，进行了肺功能测试，并使用欧洲癌症研究与治疗组织生活质量核心问卷（EORTC-QLQ-C30）确定了生活质量。PP手术时患者年龄在9至17岁之间。基础疾病包括转移性骨肉瘤（n = 5）、尤因肉瘤（2例转移性、1例原发性）和1例原发性未分化肉瘤。3例患者出现早期并发症。术后平均住院天数为14.5天。肺功能测试显示肺总量降低了19%至66%，导致轻度运动不耐受，但不影响日常生活。4例患者在PP术后<14个月死于多部位转移复发，1例患者出现局部复发，4例患者在PP术后1.5至12年处于完全缓解状态。总之，在这一小群因原发性或转移性病变接受胸膜肺切除术治疗的患者中，结果各不相同；然而，这种扩大的手术技术总体耐受性良好。术后肺功能似乎保存良好，似乎至少能延长高质量的生命，因此可被视为挽救性治疗。
