Artikel
Highly malignant sacral chordoma in an adolescent – case report
Hochmalignes sakrales Chordom bei einem jungen Erwachsenen – Fallbericht
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Veröffentlicht: | 11. April 2007 |
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Gliederung
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Objective: Sacral chordomas are of low-level malignancy but show a high level of recurrence. Metastases occur in 10% of sacral chordomas, usually late and after multiple resections. Wide en-bloc resection with postoperative radiation is the treatment of choice. Recent studies investigate the value of particle beam therapy versus conventional photon radiotherapy. This report details the authors experience with a malignant sacral chordoma.
Methods: The authors report about a 35-year-old man with a sacral chordoma who underwent 4 surgeries, conventional radiation therapy, and 1 resection of a cervical spine metastasis.
Results: Despite aggressive therapy avoiding progression of the tumor was not succesful. 4 years after initial diagnosis multiple metastases, affecting liver, lung, heart and neck, were diagnosed, and the patient got into a terminal state of his disease.
Conclusions: The treatment of sacral chordoma is an arduous clinical undertaking that requires a multidisciplinary approach and attention to detail from the outset. Despite aggressive well-planned surgical management and adherence to strict surveillance protocols, frequent recurrence and the late onset of metastatic disease are to be expected in a substantial proportion of patients, especially those with a very large chordoma. Adequate surgical treatment results in substantial functional impairment and numerous complications; however, it does offer the possibility of a disease-free survival. We advocate an attempt at complete resection, when there is still a possibility of cure, and aggressive treatment of local recurrences.