Clinical characteristics and prognosis of primary leiomyosarcoma of the pancreas: a systematic review

BACKGROUND: Primary pancreatic leiomyosarcoma (PLMS) is rare. The clinical characteristics and prognosis is still not completely understood. The aim of the present study is to identify the clinical characteristics and long-term outcomes of PLMS from the existing reported cases in different scientific literature. METHODS: PLMS cases reported in Chinese and English journals were collected and reviewed. Clinical features and long-term outcomes of these cases were summarized and analyzed statistically. RESULTS: A total of 69 cases reported from both Chinese and English journals were included in the present study. An equal incidence in gender was observed. The mean age was 53.9 ± 14.7 years. The most common symptoms were abdominal mass, abdominal pain, and weight loss. The mean size of the tumor was 11.4 ± 7.1 cm. The incidence of PLMS between the head and body-tail of the pancreas had a similar pattern. Twenty-five percent of patients had distant metastasis and 19% of patients had adjacent organs/vessels invasion at the time of diagnosis. But lymph node metastasis was documented in only one (1.5%) patient. The median survival time was 48 months. The overall 1-, 3-, 5-, and 10-year survival rates were 66.6%, 51.2%, 43.9%, and 29.3%, respectively. Results from the multivariate analysis showed that non-radical resection (P = 0.000; hazard ratio (HR) 5.128; 95% confidence interval (CI) 2.041-12.987) was the independent adverse prognostic factor. Adjacent organs/vessels invasion (yes) may be considered as an another potential independent adverse prognostic factor (P = 0.071; HR 2.708; 95% CI 0.981-7.474). CONCLUSIONS: PLMS is rare without specific clinical features. PLMS is an aggressive tumor and has a poor prognosis. Radical resection can prolong survival time of the patients.