Undifferentiated Pleomorphic Sarcoma Arising in a Fibrous Dysplasia Confirmed by GNAS Mutation Analysis: A Case Report.

CASE: We report the case of a 78-year-old woman with undifferentiated pleomorphic sarcoma (UPS) of the proximal femur arising in a previous site of fibrous dysplasia (i.e., a liposclerosing myxofibrous tumor) that had been treated 4 years earlier with curetted surgery. GNAS mutation was detected in both the previous benign lesion and the UPS.

CONCLUSION

Genetic analysis is useful in demonstrating the origin of the tumor especially in fibrous dysplasia with severe degeneration that have undergone malignant transformation.