Arytenoid Chondrosarcoma

Chondrosarcoma of the arytenoid cartilage is a rare malignant neoplasm. Fewer than 10 % of all laryngeal chondrosarcomas arise from the arytenoid rather than the cricoid (most common) or thyroid cartilages. Almost all chondrosarcomas are low grade, and therefore slow-growing. For practical purposes, they do not metastasize.

Most cases occur in older adults (50–70 years), with a male predominance. The usual presenting symptom is progressive hoarseness; less commonly, in patients who delay medical evaluation, this tumor can present with noisy breathing and airway compromise. Because the lesion expands submucosally with pushing margins, the mucosa is typically intact but “bulging.”

CT or MRI demonstrates a lobulated submucosal mass arising from the arytenoid cartilage, possibly with coarse calcifications or a “popcorn-like” chondroid matrix. Tumor margins are typically well-circumscribed, remaining confined to the arytenoid cartilage and not invading the cricoid cartilage or soft tissue.

Most laryngeal chondrosarcomas are low- to intermediate-grade (Grade I–II) with relatively bland chondrocytes in lacunae, mild atypia, and low mitotic activity. High-grade (Grade III) lesions with marked atypia and necrosis are distinctly uncommon in the larynx.

Management

• Definitive treatment is surgical excision with clear margins, while preserving laryngeal framework and function when possible.
• Endoscopic laser arytenoidectomy, while technically challenging, is theoretically possible for smaller, low-grade lesions.
• An approach via anterior thyrotomy might be an option, but would require tracheotomy.
• Removal from laterally, by creating a lateral thyroid cartilage window can also be an option and was used in the case below.
• Total laryngectomy is reserved for bulky, invasive, or recurrent tumors not amenable to conservation surgery.
• Radiation therapy has traditionally been viewed as of limited efficacy but there are reports of its use to arrest tumor progression. In fact, we have used this with surprising long term effectiveness in a few cricoid chondrosarcomas in elderly or medically fragile patients who are poor surgical candidates, or if surgery would devastate residual laryngeal function.

Prognosis

This is generally favorable, due to the low grade, indolent biology of most cases. Local recurrence is reported to occur in up to 30 % of laryngeal chondrosarcomas, often many years after initial treatment, particularly when margins are close or incomplete. Distant metastasis is rare. Long-term surveillance is essential due to the potential for late recurrence.