Extra-Pleural Pneumonectomy (EPP) in Children and Adults with Locally Advanced Sarcoma: A CanSaRCC Study

SIMPLE SUMMARY: Extra-pleural pneumonectomy (EPP) involves the removal of the parietal and visceral pleura, ipsilateral lungs, pericardium, and hemi diaphragm. In patients with advanced sarcoma in the pleura, EPP is often the only option for local control. The aim of our study was to review our institutional experience with EPP. Of ten patients in our study, five were alive without disease at last follow-up after multi-modality therapy including EPP. Two patients had local recurrence and died of progressive disease. One patient died of brain metastasis, one patient died of radiation induced sarcoma, and one patient died of surgical complications. Our results suggest that EPP is a feasible option for patients when used in combination with chemotherapy and radiation. Moreover, high-volume cancer centers should discuss the use of EPP during tumor board discussion. ABSTRACT: Sarcoma can present as locally advanced disease involving pleura for which extra-pleural pneumonectomy (EPP) may be the only surgical option to ensure adequate local control. Data were collected on patients who underwent EPP between January 2009 and August 2021 at Princess Margret Hospital and SickKids (Toronto) using the CanSaRCC (Canadian Sarcoma Research and Clinical Collaboration). Ten patients with locally advanced sarcoma involving the pleura, aged 4 to 59 years (median 19.5 years) underwent EPP. Nine (90%) received pre-operative chemotherapy and eight (80%) achieved an R0 resection. Hemithoracic radiation was administered preoperatively (n = 6, 60%) or postoperatively (n = 4, 40%). Five (50%) patients were alive without disease at last follow-up (median 34.2 months) and time from EPP to last FU was median 29.2 months (range 2.2–87.5). Two patients (20%) had local recurrence, 4.3 and 5.8 months from EPP, and both died from progressive disease, 13.1 and 8.2 months from EPP, respectively. One patient died from brain metastasis (17 months), one died from radiation associated osteosarcoma (66 months), and one died from surgical complications (heart failure from constrictive pericarditis). EPP offers a feasible and life-prolonging surgical consideration for patients with locally advanced sarcoma involving the pleura in combination with chemotherapy and radiation. Consequently, EPP should be considered during multi-disciplinary tumor board discussions at high-volume centers.