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Study Sees Limited Success for Extrapleural Pneumonectomy in Sarcoma Cases


In this retrospective analysis, outcomes data were evaluated on patients receiving care related to extrapleural pneumonectomy for soft tissue sarcoma or bone sarcoma.

Extrapleural pneumonectomy (EPP) deserves careful consideration among adult patients who have soft tissue sarcoma (STS) or bone sarcoma, due to its apparent usefulness as a salvage procedure but also because it rarely results in cure, according to new findings published in Cancer Reports.1

“Patient selection is critical with strong consideration given to multimodal therapy to optimize patient outcomes,” the study authors emphasized. “In the absence of a confirmed response to neoadjuvant treatment, long-term survival is poor and EPP should not be recommended.”

The procedure, which has potential to be life prolonging, is complicated. The en bloc resection—which involves the removal of an entire tumor mass and an additional layer of healthy surrounding tissue2—involves the parietal and visceral pleura, lung, pericardium, and ipsilateral diaphragm. In this retrospective analysis, the investigators analyzed outcomes data among 7 patients with STS and 1 patient with bone sarcoma, who had localized disease with a primary thoracic sarcoma, sarcoma recurrent to the thorax, or de novo metastatic disease. Their primary outcomes were event-free survival (EFS)—time elapsed between EPP and first event (recurrence or death)—and overall survival (OS), with secondary outcomes of disease-free interval (DFI) prior to EPP and early and late postoperative complications.

Patient median age was 41 (range 22-70) years on date of EPP, and all had been told the surgery was their only hope of a cure; any other treatment would have been considered palliative. Among the patients with STS, the most common type was synovial sarcoma in 3, and there was 1 case each of intimal sarcoma, malignant solitary fibrous tumor, rhabdomyosarcoma transformed from nonseminomatous germ cell tumor, and undifferentiated pleomorphic sarcoma. Tumors ranged in size from 90 to 250 mm. Six patients also received neoadjuvant systemic therapy.

There were no deaths in the 30 days after the EPP. However, 1 patient each developed an early and serious complications: ventilator-associated pneumonia with new-onset atrial fibrillation with rapid ventricular response requiring transesophageal cardioversion, ipsilateral hydropneumothorax requiring chest tube insertion, and ipsilateral hydropneumothorax without a chest tube. A fourth patient developed later-onset group A streptococcal bacteremia and eventual septic cardiomyopathic shock.

Operation | Image Credit: Gorodenkoff - stock.adobe.com

Extrapleural pneumonectomy is an en bloc resection; the entire tumor mass and additional health surrounding tissue are removed | Image Credit: Gorodenkoff - stock.adobe.com

The median time for EPP was 4 (range, 2-6) hours and hospital stay, 14 (9-45) days.

Over the median follow-up of 22.5 months, the investigators saw a median EFS of 6 months and a median OS of 20.7 months, both following EPP. In the 6 patients with disease recurrence, 4 cases were of distant recurrence and 2, local. By the final follow-up, 5 patients had died and a sixth was receiving palliative chemotherapy. The earliest recurrence, in a patient with primary pleural-based sarcoma, was 2 months. This patient had not received any perioperative treatment.

In the 2 patients not classified as having recurrence or whose EFS extended beyond 1 year, they had low-grade histology or a partial response to neoadjuvant chemotherapy. One of these patients remained disease free for almost 4 years, before dying from chronic sepsis.

Overall, just 1 patient remained disease free, and alive, after 3 years.

The authors highlighted that their survival outcomes are inferior to those seen in previous studies, but that only case reports on EPP for pleural-based sarcoma in adults and children are available. Additional limitations on their findings are the heterogeneity of their patients and their disease courses, the inability to conduct a formal statistical analysis because of the small case series they evaluated, and it being retrospective.


1. Zhang BY, Wilson AR, Connolly EA. Extrapleural pneumonectomy for sarcoma: outcomes of adult patients at a specialized center. Cancer Rep (Hoboken). 2024;7(4):e2065. doi:10.1002/cnr2.2065

2. Boriana S. En bloc resection in the spine: a procedure of surgical oncology. J Spine Surg. 2018;4(3):668-676. doi:10.21037/jss.2018.09.02

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