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Telangiectatic Osteosarcoma (TOS) is a rare and aggressive variant of osteosarcoma, which typically affects the metaphysis of long bones, particularly around the knee. This subtype is characterized histologically by the presence of numerous dilated blood vessels, giving rise to its name, "telangiectatic." Clinically, TOS often presents in adolescents and young adults, frequently manifesting as localized pain and swelling in the affected area. The diagnosis can be challenging as the symptoms may mimic other conditions, leading to delays in appropriate management. Diagnostic imaging, including X-rays, MRI, and CT scans, often reveals a lytic lesion with permeative borders and soft tissue masses, which can further complicate the picture. On histopathological examination, TOS exhibits a mixture of malignant osteoid, spindle-shaped cells, and an abundance of vascular structures, leading to multiple differential diagnoses, including hemangioma and other vascular tumors. The presence of osteoid production is a distinguishing feature that confirms its osteosarcomatous nature. While the telangiectatic variant has high vascularity, it is also associated with a high risk of local recurrence and distant metastasis, often leading to poorer outcomes compared to other conventional osteosarcoma types. Treatment generally consists of a multi-modal approach, combining preoperative chemotherapy, surgical resection, and postoperative chemotherapy. The choice of chemotherapy agents may include high-dose methotrexate, doxorubicin, and cisplatin, among others, tailored to the individual patient's response and tolerance. Surgical management primarily focuses on achieving wide margins during resection to minimize the risk of recurrence. In cases where limb salvage is feasible, limb-sparing surgery is preferred; otherwise, amputation may be necessary. Despite aggressive treatment, the prognosis for telangiectatic osteosarcoma remains guarded, and careful surveillance for metastasis is essential. Clinical studies indicate that the overall survival rates for TOS are lower than those of other osteosarcoma variants, primarily due to its aggressive behavior and the potential for pulmonary metastasis. Combined with the complexity of its clinical presentation, TOS requires a high index of suspicion and a multidisciplinary approach for optimal management. Research continues to explore the underlying genetic and biological mechanisms of this variant, which may pave the way for targeted therapies in the future. Understanding the unique features of telangiectatic osteosarcoma is crucial for clinicians in order to provide timely diagnosis and appropriate treatment strategies, ultimately aiming to improve outcomes for affected patients.