Abstract
Giant cell tumour (GCT) of bone is a locally aggressive benign tumour. It can, however, undergo dedifferentiation, either de novo or secondarily after local recurrence or radiation. Whether spontaneously occurring or induced by previous irradiation, this malignant transformation is typically defined as a high-grade anaplastic sarcoma devoid of giant cells. Dedifferentiation of GCT into low-grade-appearing sarcoma has not been reported yet. Here, we describe the first case of dedifferentiated GCT in the appearance of low-grade fibroblastic osteogenic sarcoma with distant bone metastases. This disease progression occurred without previous irradiation. We confirm the aggressive behaviour of this tumour despite the deceptively bland appearance of the malignant component. We also alert others to the importance of recognizing this rare histology to avoid underdiagnosis and subsequent undertreatment.