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Low-grade central osteosarcoma with extraosseous dedifferentiation: a rare case

  • Kengo Kawaguchi,
  • Kenichi Kohashi,
  • Koji Sagiyama,
  • Mikiko Hashisako,
  • Akira Nabeshima,
  • Nokitaka Setsu,
  • Makoto Endo,
  • Takeshi Iwasaki,
  • Yasuharu Nakashima,
  • Yoshinao Oda

摘要

Low-grade central osteosarcoma (LGCOS), which arises from the intramedullary cavity of the metaphysis of long bones, occasionally exhibits extraosseous spread. Approximately 10–30% of patients with LGCOS exhibit dedifferentiation, but it is rare to experience a primary tumor with a dedifferentiated component. A 38-year-old female patient presented with right knee pain for two months. Imaging studies revealed a bone mass with extraosseous involvement. Wide resection was performed, and pathologic examination led to the diagnosis of LGCOS with a dedifferentiated extraosseous lesion. A single defect in the bone cortex constituted the boundary between the low- and high-grade components. The extraosseous high-grade component included more tumor cells with p53 overexpression and more murine double minute 2 (MDM2) copies compared with the low-grade component. These genetic mutations and copy number alterations can be associated with malignant transformation of LGCOS.