Huge primary dedifferentiated pancreatic liposarcoma mimicking carcinosarcoma in a young female: A case report

BACKGROUND Pancreatic liposarcoma is a rare tumor. According to a literature review, the patient described in this study is the seventh case of pancreatic liposarcoma reported in the English literature and the third case of dedifferentiated liposarcoma. Furthermore, this case had the largest primary tumor volume, and a primary pancreatic liposarcoma was diagnosed based on sufficient evidence. CASE SUMMARY We here report a rare case of a 28-year-old female with a huge dedifferentiated liposarcoma in the pancreatic tail. In June 2015, the patient underwent distal pancreatectomy with splenectomy. During the operation, a huge liposarcoma of approximately 28.0 cm × 19.0 cm × 8.0 cm was found, which had a yellow and white fish-like incisal surface. Based on both pathology and MDM2 gene amplification, the tumor was diagnosed as a dedifferentiated liposarcoma. The patient was treated with surgery but declined postoperative chemotherapy. She was well at the 26-mo follow-up, and no relapse was observed. CONCLUSION Pancreatic liposarcoma has a low incidence. Chemotherapy should be included in the treatment regimens. Complete resection is the only effective treatment.