Mahajan, Nandita N

There are limited data regarding the surgical management of primary pulmonary artery sarcomas (PPAS) because of their rarity and complicated diagnostic history. The objective of this study was to analyze our institution’s long-term surgical management outcomes for PPAS in the absence of a care pathway. From May 1997 to June 2013, 8 patients (mean age 60.6 ± 11.8 years; range, 40-73 years; 5 women and 3 men) underwent surgical intervention for PPAS at our institution. The most common computed tomography finding was a luminal filling defect obstructing the pulmonary artery (PA), without evidence of extraluminal extension. Three patients underwent debulking/pulmonary endarterectomy alone and 5 patients underwent a more radical resection with PA patch angioplasty, PA resection and reconstruction, pulmonary valve replacement, and unilateral pneumonectomy. The mean postoperative survival in this series was 3.8 ± 3.6 years (range, 1-11.9 years), with 2 radical surgical resection patients alive at 4.9 and 11.9 years, respectively. For those patients with incomplete resection, 3-dimensional (3D) models were created to demonstrate the advantage of a preoperative guide for a more complete resection and what it would entail. Six patients had local recurrences with mean disease-free interval of 14 ± 10.9 months (range, 2 months-2.5 years), and 2 patients with re-resections had an overall postoperative survival of 2.8 and 11.9 years, respectively. In our small cohort of PPAS, patients treated with radical surgical resection had better survival. The small number of PPAS cases in this series makes proving this association unlikely but warrants consideration.

This case report describes an 83-year-old woman with multiple comorbidities who presented with melena and coffee-ground emesis with diagnostic studies evident for a large prepyloric gastrointestinal stromal tumor. She underwent combined laparoendoscopic transgastric resection surgery for the tumor, performed by a team of gastroenterologist and thoracic surgeon with a successful outcome. The case and videos in this report provide a descriptive demonstration of the steps leading up to the surgical intervention followed by a step-by-step illustration of the combined surgical technique, thus highlighting the importance of multidisciplinary approach for optimal treatment of prepyloric gastrointestinal stromal tumor.