Abstract
BACKGROUND: Low-grade myofibroblastic sarcoma (LGMS) is a rare spindle cell sarcoma especially in the pancreas, with myofibroblastic differentiation. Hitherto, only a few cases have been reported. CASE SUMMARY: Herein, we report a case involving the discovery of a pancreatic mass detected during a routine physical examination. Subsequent imaging and pathological tests of the patient led to the diagnosis of LGMS of the pancreas. Following surgical intervention, the patient experienced recurrence and metastasis. Conventional treatment is not effective for postoperative recurrent pancreatic LGMS with multiple metastases. After communicating with the patients and their families, informed consent was obtained for the treatment of anlotinib combined with pembrolizumab. Evaluation of imaging and clinical symptoms post-treatment revealed a relatively favorable response to the combination of anlotinib and pembrolizumab. CONCLUSION: Based on the comprehensive literature review, our report aimed to provide evidence for a better understanding of the disease characteristics, diagnostic criteria, imaging findings, and identification of LGMS. And explore novel treatment strategies for this disease.