Cricoid chondrosarcoma presenting as arytenoid hypertelorism.

BACKGROUND

Arytenoid hypertelorism (arytenoid cartilages spaced too widely apart) appears to be the most common initial recognizable physical finding of cricoid chondrosarcoma. Nine cases from the Center for Voice Disorders are presented. With arytenoid hypertelorism caused by cricoid chondrosarcoma, usually the posterior larynx is open. In fact, patients with arytenoid hypertelorism caused by cricoid chondrosarcoma may be aphonic even though the anterior membranous vocal folds make contact during attempted phonation.

METHODS

Between 1991 and 2002, nine patients were diagnosed with cricoid chondrosarcoma. Patients' charts and video examinations were retrospectively evaluated for symptoms and the presence of arytenoid hypertelorism on endoscopic evaluation. Two were women, and seven were men, with a mean age of 70 (range 53-72) years at diagnosis.

RESULTS

Eight (88%) patients had aphonia or dysphonia caused by arytenoid hypertelorism as their presenting symptom. Six were diagnosed after failed medialization laryngoplasty or other laryngeal rehabilitation surgery. All nine patients had a low-grade tumor. The initial treatment in eight patients was hemicricoidectomy, and one patient had a total laryngectomy. In four cases, a second procedure was needed 1 to 6 years later because of recurrence. Seven patients are alive without clinically significant disease; one is alive with moderate disease, and one has died from an unrelated cause.

CONCLUSIONS

Severely dysphonic, elderly patients presenting with arytenoid hypertelorism of unknown cause should be evaluated by fiberoptic laryngoscopy and by computed tomography scan to rule out cricoid chondrosarcoma. Because these tumors behave so benignly, the authors recommend conservative surgery (unilateral hemicricoidectomy) as the diagnostic/treatment modality of choice.