Giant cell tumor of bone (GCTB), osteosarcoma, and Ewing sarcoma represent the most characteristic primary bone neoplasms, and optimization of their diagnostic and therapeutic strategies remains a research priority in the field of bone and soft tissue oncology. The National Comprehensive Cancer Network (NCCN) released the Clinical Practice Guidelines in Bone Cancer (Version 2.2026), introducing key evidence-based updates spanning equivalent biological therapy alternatives, perioperative chemotherapy conceptual restructuring, expanded management strategies for recurrent/progressive disease, refined molecular diagnostic protocols, fertility preservation, and standardization of rare subtype treatment. This article systematically interprets the revision highlights of this guideline, integrates recent high-level clinical research evidence, analyzes its evidence-based foundation and clinical translation pathway, and compares it with relevant domestic diagnostic and therapeutic standards to provide academic reference for multidisciplinary standardized management of bone tumors in China.