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

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.

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.

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).

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.