Chondrosarcoma of the larynx.

Chondrosarcoma of the larynx accounts for approximately 0.2% of all head and neck malignancies and approximately 1% of all laryngeal malignant tumors, although it is the most common nonepithelial neoplasm of the larynx. Men are affected more frequently than women (3:1 ratio), usually during the middle to later decades of life. Patients present with a variety of symptoms as a result of tumor growth, including dyspnea, dysphagia, hoarseness, airway obstruction, and/or pain.

The tumor most commonly arises within the cricoid cartilage (specifically, the posterior lamina), although the thyroid and arytenoid cartilages are occasionally affected. The tumors arise from hyaline cartilage rather than elastic cartilage. A popcorn-like calcification on radiographic examination is quite common. Macroscopically, the tumors are smooth, lobular, and glistening, and they are "crunchy" when cut. Their mean maximum dimension is 3 cm.

The tumor is made up of lobules of hypercellular atypical chondrocytes in a basophilic cartilaginous matrix material. Lobules of neoplastic cartilage invade bone within the cartilage (figure 1). The cells have a relatively high nuclear-to-cytoplasmic ratio and hyperchromatic nuclei (figure 2). Mitotic figures are not common, and necrosis is infrequent. Based on their cellularity and degree of anaplasia, chondrosarcomas are classified as either low-, medium-, or high-grade tumors. The differential diagnosis includes chondroma, hamartoma, and polyps, although spindle-cell (sarcomatoid) carcinoma can contain metaplastic or malignant cartilage.

Surgical excision is the treatment of choice, either as a wide excision or as voice-sparing surgery. Recurrence is common (20%), but it can be managed with additional conservative surgery until the patient reaches the point where laryngectomy may be the only resort. Patients experience an excellent long-term prognosis, with a 10-year survival rate of greater than 95%.

Acknowledgment

The author would like to thank Diana Chuong, MD, for her contribution to this Clinic.

Suggested reading

Kozelsky TF, Bonnet JA, Foote RL, et al. Laryngeal chondrosarcomas: The Mayo Clinic experience. J Surg Oncol 1997;65:269-73.

Thompson LD, Gannon FH. Chondrosarcoma of the larynx: A clinicopathologic study of 111 cases with a review of the literature. Am J Surg Pathol 2002;26:836-51.

From the Department of Pathology, Woodland Hills Medical Center, Southern California Permanente Medical Group, Woodland Hills, Calif.