Primary pancreatic leiomyosarcoma: a retrospective analysis of clinical characteristics and prognosis of this rare disease.

Background/aims: Primary pancreatic leiomyoarcoma (PLMS) is a rare disease; its clinical characters and prognosis are poorly demonstrated. This study aimed to investigate the tumor's clinical characters and to reveal the true outcome.
Methodology: A retrospective review of both Chinese and worldwide PLMS patients were performed. Data was collected and analyzed and overall survival was described by Kaplan-Meier curve.
Results: In total, there were 9 cases identified in China and 55 cases identified in the worldwide literature. Incidence rate was general equal in gender and had an increased trend with age(mean +SD: 55+14 years). Abdominal pain, mass and weight loss were the most common presentations, mean tumor size was 10cm (SD 7.2cm) and most of them presented as a solid mass. Forty six cases accepted operation, 33 of which obtained a radical resection. Median overall survival time was 27 months.The patients who accepted radical resection had an obviously improved prognosis over non-resectable patients.
Conclusions: PLMS commonly occurred in the mid-age people and there was lack of specific non-invasive methods for precise preoperative diagnosis.PLMS has a much better prognosis compared with pancreatic ductal adenocarcinoma. Radical resection is the only hope for improving the outcome of this malignancy.