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

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.

Extrapleural pneumonectomy for sarcoma: Outcomes of adult patients at a specialized center

“…Only one patient (12.5%) remained alive without disease after follow‐up of 37 months, five patients (62.5%) died of direct disease‐related causes, one (12.5%) died of radiation complications, and one (12.5%) was receiving palliative chemotherapy for metastatic disease at last follow up. Our survival outcomes appear inferior to two case series published in 2022 and 2021 from Rodrigues et al 7 and Hameury et al, 8 respectively; however, there are striking differences between our patient population and those described in the earlier case series.…”

“…Only one patient (12.5%) remained alive without disease after follow-up of 37 months, five patients (62.5%) died of direct disease-related causes, one (12.5%) died of radiation complications, and one (12.5%) was receiving palliative chemotherapy for metastatic disease at last follow up. Our survival outcomes appear inferior to two case series published in 2022 and 2021 from Rodrigues et al7 and Hameury et al,8 respectively; however, there are striking differences between our patient population and those described in the earlier case series.Rodrigues et al7 reported the outcomes of 10 patients aged between 4 and 59 years (median 19.5 years) who underwent EPP, including eight with soft tissue sarcoma and one with bone sarcoma.After a median follow-up of 29.2 months, five patients had no evidence of disease, three died of disease, and two died of treatmentrelated complications, including heart failure due to constrictive pericarditis and radiation-induced secondary sarcoma.Hameury et al8 described a case series of nine children aged between 9 and 17 years (median 15 years) with osteosarcoma (five cases), Ewing sarcoma (three cases), and undifferentiated sarcoma (one case). After a median follow-up of 6.8 years, four patients were in complete remission, one patient was alive with local recurrence, and four died of metastatic disease.F I G U R E 3 Overall survival of patients with pleural-based sarcoma after extrapleural pneumonectomy.…”

“…First described in 1949 for the management of pulmonary tuberculosis, experience with EPP has been chiefly gained in the management of pleural mesothelioma and more recently, for select patients with thymomas, advanced non‐small cell lung cancers and sarcomas 3,5,6 . Irrespective of the tumor type, EPP should always be performed by an experienced cardiothoracic team in a high‐volume thoracic surgery center due to the potential for high morbidity 7 . Given the rarity of pleural‐based sarcoma, literature on outcomes following EPP is sparse and no prospective studies exist.…”

“…et al and Hameury et al Rodrigues et al described one of 10 patients developed an early complication of empyema that necessitatedwound washout and one patient of ten patients who developed a late complication of fatal pericarditis and died 7 months post-surgery 7. In Hameury et al, three of nine patients had early postoperative complications including one case of pulmonary infection, one case of postoperative bleeding requiring surgery and one case of cardiac tamponade managed with an emergency pericardial window.…”

“…These results are similar to our perioperative complication rate of 37.5%, perioperative mortality rate of zero, median surgical operative time of 4 hours and median hospital length of stay of 14 days.The variable survival outcomes of patients in our study confirms the importance of patient selection for EPP. Previous publications have proposed the following criteria regarding patient selection for EPP3,7,8 : (1) recommendation by consensus opinions of multidisciplinary tumor board discussion; (2) pleural lesions limited to one hemithorax; (3) cases where there is no other alternative apart from palliative treatment; (4) good performance status; and (5) "sufficient" response to preoperative chemotherapy. Several authors have discussed the importance of EPP being offered as part of multimodal treatment in conjunction with chemotherapy and/or radiation and have cautioned that surgery alone may be insufficient to provide long-term disease control.…”