Primary Bone Tumors
A 55-year-old man presents with progressive low back pain and constipation over 6 months. Rectal examination reveals a presacral mass. MRI shows a large lobulated mass centered in the sacrum, hyperintense on T2, with destruction of S3-S5. CT shows characteristic bone destruction with preserved cortical margins. Biopsy reveals epithelioid cells with abundant bubbly cytoplasm (physaliphorous cells) positive for brachyury and cytokeratin. Regarding chordoma:
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Chordoma is a rare primary malignant bone tumor arising from notochordal remnants; it occurs exclusi...
Histologically, chordomas contain characteristic physaliphorous cells (cells with abundant bubbly/va...
Chordoma arises from mesenchymal cells; it occurs in appendicular skeleton; the most common location...
Imaging shows: CT reveals osteolytic bone destruction with soft tissue mass, often with calcificatio...
Chordomas are relatively radioresistant to conventional radiotherapy; high-dose proton beam or carbo...
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