Sarcoma

Ask the Doc - Peter Galvin, MD

An 11-year-old girl presented to the orthopedic clinic in Taipei Veterans General Hospital in Taiwan with a one-month history of right thigh pain that worsened at night. She had no systemic (generalized) symptoms. Physical examination was notable for soft-tissue swelling over the distal (lower) right thigh and limited range of motion of the right knee. Radiographs (X rays) of the knee showed a metaphyseal (end part of a long bone where the growth plate is) osteoblastic (destructive) lesion with ill-defined margins and cortical (inner part of a bone) destruction in the distal femur (Panel A, anteroposterior [front to back] view; Panel B, lateral view). The mass had a sunburst appearance, a finding that is seen in aggressive bone lesions that results from the formation of divergent (or chaotic) bone spicules (bone growth) as the periosteal (outer bone) reaction to the rapid growth of the tumor.

On the basis of subsequent magnetic resonance imaging (MRI) and a bone biopsy, a diagnosis of high-grade osteosarcoma was made. Osteosarcoma is the most common primary malignant bone tumor in children. It typically manifests in the second decade of life owing to the rapid bone growth during that stage of development. Further imaging in this case did not reveal any distant spread of the tumor. The patient was treated with neoadjuvant (treatment before the main therapy or surgery) chemotherapy, wide excision of the tumor, reconstruction, and adjuvant (treatment given after the main treatment or surgery in order to destroy any remaining cancer cells) chemotherapy.

Thankfully, after more than 10 years of follow-up, the patient has remained cancer-free with independent ambulation, an outcome that was certainly unexpected given the aggressive nature of the tumor.

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