Sarcomas

Sarcomas of the larynx are uncommon, accounting for 1-2% of all laryngeal neoplasms. Among them, chon-drosarcoma is the most frequent type, comprising 75% of all laryngeal sarcomas [209].

ICD-O:9220/3

Chondrosarcoma (CS) is the most common non-epithelial neoplasm in the larynx. It appears that laryngeal CS behaves less aggressively than its counterpart in the rest of the body. The majority of laryngeal CS are low grade CS [209, 360].

Laryngeal CS affects men more frequently than women, mostly in the 7th decade [209]. It usually presents with hoarseness; other symptoms include dyspnoea, dysphonia, a cough, a neck mass, airway obstruction and pain [209, 360]. The symptoms are frequently present for a long time before the diagnosis is established.

Chondrosarcoma arises predominantly in the cricoid cartilage, especially at the inner posterior plate; it can also arise in the thyroid and arytenoid cartilages. It very rarely arises in the epiglottis [209, 360].

The aetiology is unknown, although disordered ossification of the laryngeal cartilages and ischaemic changes in a chondroma have been suggested as possible predisposing risk factors [360]. Other possible risk factors include previous radiation exposure [134] and Teflon injection [147].

Grossly, CS is characteristically a lobulated, submu-cosal mass covered by normal mucosa; on its cut surface it is glassy, firm white or grey (Fig. 7.14a) . Radiographic findings are characteristic showing coarse or stippled calcifications [360, 377]. Microscopically, larynge-al CS is indistinguishable from CS of bone origin elsewhere in the body, and is graded according to the his-tologic criteria proposed by Evans and co-workers [99] for CS of the bones. Low-grade CS (grade I) has slightly increased cellularity, binucleation in the lacunar spaces, slight nuclear pleomorphism, and hyperchromasia (Fig. 7.14b) . High-grade CS (grade III) has remarkable cellularity, multinucleation in the lacunar spaces, nuclear pleomorphism, nuclear hyperchromasia, necrosis and mitotic activity, whereas the intermediate grade CS (grade II) has medium cellularity and less nuclear pleomorphism [128].

The vast majority of laryngeal CS are of low- or intermediate grade. High-grade CS are considered to be rare; in a large series of 111 laryngeal CS, only 6 (6%) were of a high grade [360]. Dedifferentiated (mesenchymal) CS

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