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Humeral metastasis from a sacrococcygeal chordoma: a case report
© Azarpira et al.; licensee BioMed Central Ltd. 2011
Received: 18 September 2009
Accepted: 1 August 2011
Published: 1 August 2011
Chordomas 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.
We 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.
Chordoma 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.
Chordomas are rare, low-grade, primary malignant bone tumors arising from primitive notochord remnants of the axial skeleton. The sacrum is the most common anatomical site of origin, accounting for 50% to 60% of all cases, followed by the skull base region (spheno-occipital and/or nasal), accounting for 25% to 35% of cases; the cervical vertebrae, accounting for approximately 10% of cases; and the thoracolumbar vertebrae, accounting for approximately 5% of cases [1–4].
These tumors are mainly locally aggressive, with a high incidence of local recurrence and a poor long-term prognosis. Metastatic lesions have been reported in the liver, lungs, lymph nodes, peritoneum, skin, heart, humeri, brain, and distant regions of the spine [1–7].
Chordomas arise from the remnant of the fetal notochord and grow slowly. The most common location is the sacrococcygeal region. This tumor is generally reported in adults; most patients are in the fifth to seventh decades of life [10–12]. Classic radiological findings for chordoma are a bony lytic lesion with an accompanying soft tissue mass. The sacrococcygeal lesions are known to metastasize more frequently than the other types, and radiation therapy raises the risk of metastasis. Despite all the available modalities of treatment, the prognosis for this condition remains poor because of the difficulty in gaining surgical clearance and the tumor's propensity to metastasize . Histologically, mitoses and anaplasia can be present in chordomas without adversely affecting the duration of a patient's survival. Previous studies have found that many of the metastatic lesions were asymptomatic or were found during post-mortem examination, which suggests that metastases from chordomas could be considered indolent in these cases [4, 5]. Our patient had an asymptomatic rib lesion as well as a large humeral metastasis with pathologic fracture. A pathological analysis of the metastatic tumor revealed features identical to the primary tumor. Metastasis to the long bones is a rare phenomenon.
The biological behavior of chordomas differs from patient to patient. Some patients harbor tumors capable of aggressive behavior both locally and distantly, while others have tumors that behave more indolently. There is no good method for predicting the behavior of individual chordomas [4, 11]. Therefore, it seems that aggressive surgical resection with local adjuvant treatment, such as carbon ion therapy, must be undertaken to prevent the development of recurrent lesions and metastatic neoplasms.
Chordomas are rare tumors. 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. Although these tumors rarely metastasize, they need to be considered in the differential diagnosis of metastatic epithelioid bone tumors.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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