Liposarcoma masquerading as an inflammatory pseudotumor: a case report

BACKGROUND: Distinguishing an atypical lipomatous tumor/well-differentiated liposarcoma from a benign lipomatous tumor on morphology alone can be difficult and there is an established role for MDM2 fluorescent in situ hybridization studies in making this differential diagnosis. There is no literature on the role for MDM2 fluorescent in situ hybridization studies in distinguishing between a well-differentiated liposarcoma with extreme fibrosis and a fibrosing inflammatory pseudotumor. CASE PRESENTATION: We report the case of a 76-year-old Australian woman initially diagnosed by an excision biopsy with a retroperitoneal fibrosing inflammatory pseudotumor. She was then diagnosed 5 years later with a pleomorphic undifferentiated sarcoma. Upon review of the original resection specimen, we were able to show that the tumor demonstrated MDM2 amplification. MDM2 amplification was also present in some adjacent bland adipose tissue, and also in the tumor recurrence as a pleomorphic undifferentiated sarcoma. CONCLUSION: Taken together, our findings provide strong evidence that the original tumor was a misdiagnosed well-differentiated liposarcoma with extreme fibrosis, and the pleomorphic undifferentiated sarcoma represented a recurrence of the same tumor with dedifferentiation.