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Giant Cell Tumor of Bone (GCT) is a benign yet locally aggressive neoplasm that primarily affects the metaphysis of long bones, particularly around the knee joint, such as the distal femur and proximal tibia, though it can occur in other locations such as the pelvis and spine. Characteristically, it presents as a well-defined lytic bone lesion that can cause significant bone destruction, often leading to the expansion of the bone cortex. GCT is composed of multinucleated giant cells, mononuclear stromal cells, and the presence of a reactive or inflammatory background, which contributes to the tumor's distinctive histological appearance. The multinucleated giant cells are thought to derive from osteoclast precursors, indicating a possible role in bone resorption. This tumor typically manifests in young adults, particularly in individuals between the ages of 20 and 40, and exhibits a slight female predominance. The exact cause of GCT remains uncertain, though it is hypothesized to be related to dysregulation of local osteoclast activity, which may be influenced by various genetic factors and hormonal components. Some studies suggest that alterations in the RANK/RANKL/OPG pathway, which is crucial in bone remodeling, play a vital role in its pathogenesis. Patients often present with localized pain and swelling, which may be mistaken for other conditions such as fractures or joint injuries. As the tumor progresses, it can lead to complications such as joint instability and further bone destruction, making timely diagnosis and treatment essential. Imaging studies, such as X-rays, CT scans, and MRI, are critical for evaluation, revealing a lytic lesion with possible cortical thinning or breakthrough. The treatment of choice for GCT is surgical intervention, typically involving curettage and bone grafting, although more extensive procedures such as resection may be necessary in aggressive cases or those with complications. Adjuvant therapies, such as the use of phenol, liquid nitrogen, or radiotherapy, may be applied to reduce recurrence rates. While GCT is considered benign, it has a propensity for local recurrence, with rates varying depending on the treatment method and the completeness of the resection. A small percentage of cases may undergo malignant transformation, especially after aggressive surgical intervention or if they recur multiple times. Given these potential complications, follow-up is critical to monitor for signs of recurrence or complications, underscoring the importance of a comprehensive management approach for those diagnosed with this tumor. Overall, Giant Cell Tumor of Bone presents unique challenges due to its aggressive nature and the necessity for careful management to prevent recurrence and preserve function.