Pathology of pleomorphic/undifferentiated and dedifferentiated bone neoplasms

Primary malignant bone tumors are uncommon and their accurate classification requires careful correlation of clinical, radiological, and pathologic findings. It is a heterogeneous group of tumors with a wide spectrum of morphology and their biological potential can be of low- or high-grade, depending on their risk for developing metastases. Over the past several decades, the classification of bone sarcomas has remained largely constant. However, some of the tumors have been reclassified and several new entities have emerged. In this review, we will focus on pleomorphic fibrosarcoma/UPS and dedifferentiated bone tumors, discuss their key diagnostic features, differential diagnosis, and their relation to prognosis.