Abstract
Simple SummaryPediatric sarcoma patients with pleuropulmonary lesions have a dismal prognosis because the impossibility to achieve local control. Local therapy with radiotherapy, whether in combination with chemotherapy, appears to be insufficient to eliminate the tumor cells. The aim of this study was to determine if pleuropneumonectomy (PP) could be a therapeutic option. We retrospectively reviewed nine patients who underwent 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. Outcome is variable, four patients died within 14 months after PP, one patient suffered from local recurrence, and four patients are in long-lasting remission. This extended operation is quite well tolerated. Lung function seems preserved, and quality of life is generally good. Because it gives improvement of survival in patients with pleural lesions, PP can be considered as effective salvage therapy in selected patients.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 (n = 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.
Highlights
Pediatric sarcoma patients with pleuropulmonary lesions, either initial or found at a later stage, have a dismal prognosis due to the lack of local control [1,2]
Pleural lesions were present at diagnosis in three patients or at relapse in six patients
Polites et al reported no survivor at one year after pneumonectomy for six metastatic osteosarcoma patients, suggesting pneumonectomy was not a good indication for these patients [22]
Summary
Pediatric sarcoma patients with pleuropulmonary lesions, either initial or found at a later stage, have a dismal prognosis due to the lack of local control [1,2]. PP is a standardized procedure with en bloc resection of the parietal and visceral pleura of the lung, the lung, pericardium, and homolateral diaphragm [3] This procedure was first used in adult patients with tuberculosis infection [4] and subsequently applied in adult patients with malignant pleural mesothelioma [5,6,7] and thymomas [8,9], with mainly loco regional involvement of disease. This procedure is described in patients with lung metastases [10], and primary sarcomas of the hemithorax [11,12,13]. In this procedure about one-third of all adult patients experienced a surgical complication after
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