Abstract
IntroductionChordomas are rare tumors of the skeletal system that arise from an intra-osseous benign precursor of notochordal cells. They are mainly locally aggressive. However, metastases to other sites, including the humeri, resulting in pathological fractures have been reported. We report the case of a patient with a metastatic chordoma that produced a pathologic fracture of the humerus.Case presentationWe report the case of a 60-year-old Iranian woman who presented with a fracture of her right humerus following a minor trauma. She had a history of a sacrococcygeal chordoma. Histological and immunohistochemical studies of the fracture site suggested the diagnosis of a chordoma.ConclusionsChordoma is a rare tumor and rarely metastasizes, but it should be considered in the differential diagnosis of epithelioid bone tumors. The only current effective treatment for this type of tumor is carbon ion therapy. There is currently no effective medical therapy available for advanced chordoma, and this type of tumor is not very responsive to radiotherapy.
Highlights
Chordomas are rare tumors of the skeletal system that arise from an intra-osseous benign precursor of notochordal cells
Chordoma is a rare tumor and rarely metastasizes, but it should be considered in the differential diagnosis of epithelioid bone tumors
On the basis of these findings, we considered differential diagnoses such as myxoid chondrosarcoma, myxoid liposarcoma, primary cells stained positive for AE1/3 (Figure 4), ethidium monoazide (EMA), S100, and vimentin
Summary
They are relatively radioresistant, and currently there is no effective medical therapy for treating them. Complete local excision is the most effective treatment for achieving a long-term cure. These tumors rarely metastasize, they need to be considered in the differential diagnosis of metastatic epithelioid bone tumors.
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