Extranotochordal extralaryngeal chordoma : a case report

Abstract Chordomas are rare, malignant, slowly growing neoplasms which develop from vestigial remnants of the fetal notochord. Most chordomas arise in the sacrococcygeal and spheno-occipital region. Extranotochordal chordomas are extremely unusual. A case of extranotochordal chordoma with extralaryngeal localization is described. A 73-year-old male presented with swallowing difficulties and hoarseness. Contrast-enhanced magnetic resonance imaging of the neck revealed a well-encapsulated tumor mass that was well enhanced and located in the left retrolaryngeal space at the level of C4, dislocating the larynx to the right. Left radical neck dissection and tumor extirpation were performed. The tumor had not invaded cervical vertebra and the surrounding soft tissue but superficial erosions of the ossificated thyroid and cricoid cartilage were found. High-power pathologic examination and immunohistochemistry defined the lesion as a dedifferentiated type of chordoma. The patient received adjuvant radiotherapy. Four years after the surgery, the patient has been free from tumor recurrence.