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Osteosarcoma of the larynx is probably the rarest mesenchymal tumour of the larynx, with only 16 cases reported so far. The majority of them occur in males between the sixth and eighth decades of life. Patients usually present with non-specific symptoms such as dysphonia and upper airway compromise. The most common site of distant metastasis is the lung. Clinically, the tumour follows an aggressive course and is associated with high mortality. The case we present is unusual as it occurred at a young age (38 years) as compared with the norm and it did not arise from the endolarynx, unlike many of the other cases. This was the only known case where a Pearson near-total laryngectomy was performed whereby the patient's natural speech mechanism was preserved. This surgery was possible because the contralateral half of the larynx was clearly disease free and the interarytenoid region was uninvolved. The patient underwent postoperative adjuvant external beam radiotherapy beginning 4 weeks after surgery. The patient is doing well after 15 months of follow-up and shows no signs of recurrence.

Extraskeletal osteosarcoma of the larynx: an extremely unusual tumour