Extrapleural pneumonectomy for sarcoma: outcomes of adult patients at a specialized center and literature review.

Background: Extrapleural pneumonectomy (EPP) is a complex surgical procedure involving en-bloc resection of the parietal and visceral pleura, lung, pericardium, and ipsilateral diaphragm. Small case series of pleural-based sarcoma of predominantly paediatric patients suggest EPP may be a life-prolonging surgical option. We aimed to describe the characteristics and outcomes of adults who underwent EPP at a specialized sarcoma center. Methods: Clinicopathologic variables, surgical details and follow-up information were extracted for eight patients undergoing EPP for pleural-based sarcoma between 2017-2020. Primary outcomes were event-free survival (EFS) and overall survival (OS) from the date of EPP. Secondary outcomes were disease-free interval (DFI) prior to EPP, and early and late postoperative complications. Results: At median follow-up of 22.5 months, median EFS was 6.0 months and OS was 20.7 months. Six patients had disease recurrence; five patients died of progressive disease. Two patients had not recurred: one died of a radiation-related esophageal rupture, and one was alive with no evidence of disease at 37.0 months. Characteristics of those with the longest EFS included low-grade histology and achieving a metabolic response to preoperative chemotherapy. Early postoperative complications included one ventilator-associated pneumonia with new-onset atrial fibrillation and two cases of hydropneumothorax. Late surgical complications included a case of infective endocarditis and septic shock. Conclusions: In adult sarcoma patients, EPP is rarely curative but appears to be a feasible salvage procedure when performed at specialized centers. Patient selection is critical, with a strong consideration for multimodal treatment to optimize oncological outcomes.