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

Our plain-English summary of Extra-Pleural Pneumonectomy (EPP) in Children and Adults with Locally Advanced Sarcoma: A CanSaRCC Study, by C. Rodrigues, H. Peretz Soroka, A. Pierro, et al (Current Oncology, 2022).

The study

• Authors: C. Rodrigues, H. Peretz Soroka, A. Pierro, R.M. Baertschiger, M. Cypel, L. Donahoe, D.S. Tsang, J. Cho, M. De Perrot, T.K. Waddell, A.A. Gupta
• Journal: Current Oncology
• Published: 2022;29(6):4260-4266
• Cited by: 4 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

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.

In plain English

This was a retrospective review of ten patients, aged 4 to 59 years, with sarcoma (a cancer of soft tissue or bone) that had invaded the pleura, the lining around the lung. All had extra-pleural pneumonectomy (EPP), an operation removing the lung, pleura, pericardium and half of the diaphragm, at hospitals in Toronto between January 2009 and August 2021. Surgery was combined with chemotherapy and/or radiation. The study did not examine asbestos exposure. Mesothelioma appears only in the authors' description of other studies. Eight of the ten patients had negative (R0) surgical margins. All ten received radiation, and nine received chemotherapy before surgery. At last follow-up, five patients were alive without disease. Two had pleural recurrence at 4.3 and 5.8 months and died of progressive disease. One died of brain metastasis, one of radiation-associated sarcoma, and one of heart failure caused by a surgical complication. No patient died within 30 or 90 days. The authors concluded that EPP with chemotherapy and radiation is feasible and appears safe, but that the series is too small to confirm its efficacy.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: retrospective case series (review of one institutional group's experience)
• Who was studied: 10 children and adults with locally advanced sarcoma involving the pleura, treated with EPP at University Health Network and the Hospital for Sick Children, Toronto, January 2009 to August 2021; ages 4 to 59 years, median 19.5
• Exposure: No asbestos or other environmental exposure was studied. The article reports a treatment: EPP as part of multimodal therapy with chemotherapy (always doxorubicin-based) and/or hemithoracic radiation.
• Main results:
  • Eight of 10 patients had negative (R0) margins; two had positive margins
  • Nine of 10 patients received pre-operative chemotherapy
  • All 10 patients received radiation therapy
  • Two patients (one with undifferentiated pleomorphic sarcoma, R0; one with synovial sarcoma, R1) had local pleural recurrence at 4.3 and 5.8 months after EPP
  • Both patients with local recurrence died of progressive disease, at 13.1 and 8.2 months after EPP
  • Five of 10 patients (50%) were alive without disease at last follow-up
  • One patient died of brain metastasis at 17 months and one of radiation-associated sarcoma at 66 months after EPP
  • One patient died of heart failure from constrictive pericarditis, a surgical complication, 7 months after EPP
  • No deaths within 30 or 90 days of surgery
• The authors' conclusion: The authors concluded that EPP combined with chemotherapy and pre- or postoperative radiation is feasible and appears to be safe in patients with primary or metastatic/recurrent sarcoma.
• Limitations the authors state:
  • Because the review was retrospective, a standardized scale for reporting complications could not be used
  • The series is too small to confirm the true efficacy of EPP
  • The comparison between chemo-sensitive and less chemo-sensitive subtypes rests on a small number of patients
  • Longer follow-up is needed to assess long-term effects

Topics

The National Library of Medicine indexes this study under subjects our site covers: Mesothelioma.

Medical terms in this study, explained in our Lexicon: Pneumonectomy, Sarcoma, Pleura, Chemotherapy, Metastasis, Tumor Board, High-Volume Center.

Related studies

• Extrapleural pneumonectomy for sarcoma: Outcomes of adult patients at a specialized center — B.Y. Zhang, A.R. Wilson-Smith, et al. (2024)
• Two cases of secondary soft tissue sarcomas after radiotherapy and radiochemotherapy — C. Saggia, G. Forti, et al. (2004)
• Radio frequency hyperthermia of advanced human sarcomas — F.K. Storm, R.S. Elliott, et al. (1981)
• Squamous cell carcinoma arising from the pleura after pneumonectomy for squamous cell carcinoma of the lung — M. Franke, H.D. Chung, et al. (2010)
• Extra-pleural pneumonectomy versus no extra-pleural pneumonectomy for patients with malignant pleural mesothelioma: clinical outcomes of the Mesothelioma and Radical Surgery (MARS) randomised feasibility study — T. Treasure, L. Lang-Lazdunski, et al. (2011)
• Extra-pleural pneumonectomy in the era of image-guided intensity-modulated radiotherapy — M. Trovo, D. Franceschini, et al. (2019)

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