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


METHODS
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.
Department of Gastrointestinal Surgery, Sichuan University, Chengdu, China.
Xiao
X
Du
Zhong
Z
Cheng
Zong-Guang
ZG
Zhou
Ming-Ming
MM
Zhang
Zhi-Xin
ZX
Chen
D015746:Abdominal Pain / Q000209:etiology; D000328:Adult; D000367:Age Factors; D000368:Aged; D002681:China; D005260:Female; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D007890:Leiomyosarcoma / Q000150:complications / Q000401:mortality / Q000473:pathology / Q000601:surgery; D008297:Male; D008875:Middle Aged; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000150:complications / Q000401:mortality / Q000473:pathology / Q000601:surgery; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D047368:Tumor Burden; D015431:Weight Loss