Problems and countermeasures for surgical resection of primary pulmonary artery intimal sarcoma

BACKGROUND: Pulmonary artery intimal sarcoma (PAIS) is a rare but aggressive malignancy. This study clarified the problems and countermeasures of surgical treatment by examining surgical cases of PAIS. METHODS: Between January 2007 and October 2020, 10 patients with PAIS who underwent surgery at our hospital were retrospectively examined. RESULTS: The surgical procedure that aimed at complete resection was pulmonary resection only (three cases), along with pulmonary artery vascular replacement (six cases) and pulmonary endarterectomy (PE) (one case). The positive rate of vascular stumps was 7/10. In all cases, chest computed tomography scan showed positive margins of ≤20 mm between the tumor and surgical dissection (6/6). In addition, the distance between the location of the tumor on computed tomography and the dissection line during surgery needed to be at least 20 mm (2/3). However, even at a distance of 25 mm, one case with a positive margin was observed. Postoperative recurrence was 8/9 cases, and the median recurrence period was as short as 10 months (range, 3–19 months). Postoperative treatment was required in 7/9 cases (operation/chemotherapy/radiotherapy/chemoradiotherapy/heavy ion radiotherapy =1/2/2/1/1). The median survival was 15 months (range, 0.5–36 months). CONCLUSIONS: Extended surgery should be performed as much as possible, with a distance of at least 20 mm between the location of the tumor on computed tomography scan and the incision line during surgery. The median postoperative recurrence period was as short as 10 months. Therefore, intensive care for intrathoracic recurrence follow-up is required for 1 year after surgery.