Pathology of the Head and Neck

Larynx and Hypopharynx Chapter 7 223aFig. 7.14. Chondrosarcoma of the cricoid cartilage of the larynx.a Typical macroscopic appearance of the tumour located in thecricoid cartilage: cut section reveals a glistening, lobulated, glassybtumour. b Microscopically, there is slightly increased cellularity,binucleation in the lacunar spaces, mild nuclear pleomorphismand hyperchromasiaMacroscopically, they mostly present as submucosalmasses [335].Microscopically, they are similar to MEC in othersites, and are classified as low-, intermediate-, and highgradeMEC (see Chap. 5).The behaviour is unpredictable, and is related to thegrade and stage of the disease. The best treatment iscomplete surgical excision. Radiotherapy has been reportedto be successful in a limited number of patients[335]. Neck dissection may be necessary, as 50% of patientswith MEC have metastases in the regional lymphnodes. The 5-year survival is 90–100% for low-gradeMEC, and 50% for high-grade MEC [115].7.7.5 SarcomasSarcomas of the larynx are uncommon, accounting for1–2% of all laryngeal neoplasms. Among them, chondrosarcomais the most frequent type, comprising 75%of all laryngeal sarcomas [209].7.7.5.1 ChondrosarcomaICD-O:9220/3Chondrosarcoma (CS) is the most common non-epithelialneoplasm in the larynx. It appears that laryngeal CSbehaves less aggressively than its counterpart in the restof the body. The majority of laryngeal CS are low gradeCS [209, 360].Laryngeal CS affects men more frequently than women,mostly in the 7th decade [209]. It usually presentswith hoarseness; other symptoms include dyspnoea,dysphonia, a cough, a neck mass, airway obstructionand pain [209, 360]. The symptoms are frequently presentfor a long time before the diagnosis is established.Chondrosarcoma arises predominantly in the cricoidcartilage, especially at the inner posterior plate; it canalso arise in the thyroid and arytenoid cartilages. It veryrarely arises in the epiglottis [209, 360].The aetiology is unknown, although disordered ossificationof the laryngeal cartilages and ischaemic changesin a chondroma have been suggested as possible predisposingrisk factors [360]. Other possible risk factorsinclude previous radiation exposure [134] and Teflon injection[147].Grossly, CS is characteristically a lobulated, submucosalmass covered by normal mucosa; on its cut surfaceit is glassy, firm white or grey (Fig. 7.14a) . Radiographicfindings are characteristic showing coarse or stippledcalcifications [360, 377]. Microscopically, laryngealCS is indistinguishable from CS of bone origin elsewherein the body, and is graded according to the histologiccriteria proposed by Evans and co-workers [99]for CS of the bones. Low-grade CS (grade I) has slightlyincreased cellularity, binucleation in the lacunar spaces,slight nuclear pleomorphism, and hyperchromasia(Fig. 7.14b) . High-grade CS (grade III) has remarkablecellularity, multinucleation in the lacunar spaces, nuclearpleomorphism, nuclear hyperchromasia, necrosisand 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 intermediategrade. High-grade CS are considered to be rare;in a large series of 111 laryngeal CS, only 6 (6%) were ofa high grade [360]. Dedifferentiated (mesenchymal) CS