Purpose: Primary leiomyosarcoma of the pancreas (PLMSP) is rare. The clinical features and outcomes are still unclear. The present study aims to identify the clinical features, outcomes, and racial differences of PLMSP.
Methods: PLMSP cases reported in Chinese, English, French, and Japanese journals were collected and reviewed. Data from these reports were summarized and analyzed statistically.
Results: In addition to a female patient presented to our department with PLMS, a total of 87 cases reported in the literature were included in the present study. An equal incidence in gender was observed. The mean age was 53.8 years. Common symptoms were abdominal pain and abdominal mass. At the time of diagnosis or after a period of follow-up, 37.5% of patients had distant metastasis and 31.8% of patients had regional organs/vessels invasion. The 5-year mortality rate was 77.8%. The presence of distant metastasis and the absence of radical surgery are significantly associated with poor outcomes. Regional invasion was significantly more common in East Asians.
Conclusion: PLMSP is an aggressive tumor with a poor prognosis. Radical resection can decrease the mortality of the patients. Early detection of such tumor is recommended.
Keywords: Distal pancreatectomy; Mesenchymal tumors; Pancreaticoduodenectomy; Radical surgery; Smooth muscle actin.