figure 7-8 A, Bronchoscopic view of pleomorphic adenoma. This true adenoma of the trachea is very rare. B, Another pleomorphic adenoma, with areas of adenocarcinoma present.
Squamous papilloma of the trachea may occur as a solitary, moderate-sized lesion, which should be removed by segmental tracheal resection. Naka and colleagues in 1993 found reports of 5 solitary tracheal papillomas, 7 in the main bronchus, and 41 in a lobar bronchus.25 They proposed surgical resection for wide-based or poorly-defined tumors or for suspected malignancy. Tracheal polyps of inflammatory origin which occur as solitary lesions may also be obstructive.26
Multiple squamous papillomatosis also occurs in many locations in the trachea and at the carina. A fortunately-rare form produces multiple papillomas throughout the distal tracheobronchial tree, with obstructive septic consequences. In children, squamous papillomas of the trachea behave in benign fashion and tend to regress with adolescence (see Chapter 6, "Congenital and Acquired Tracheal Lesions in Children").27 Their occurrence is related to human papilloma virus and antiviral treatment has been employed. These tumors were treated by repeated endoscopic removal using, in various eras, electrocoagulation, cryosurgery, and now, laser—all seemingly effective. Multiple lesions in adults, on the other hand, may be verrucous lesions, which cover large areas. The process may be so extensive that surgical resection is not possible. This is one of the few clear-cut indications for repetitive laser treatment. The role of photody-namic therapy remains to be clarified. Invasive carcinoma is not uncommon deep within these lesions.
Cartilaginous tumors, including osteochondroma, chondroma, chondroblastoma, and chondrosarcoma, are very rare in the trachea and only somewhat more frequent in the larynx.28 Huizenga and Balogh found 10 cartilaginous tumors in 5,000 laryngeal neoplasms.29 Peak incidence is from 40 to 70 years of age. In the larynx, about 70% arise from the cricoid, most frequently from the posterior plate. The tumor is submucosal and produces obstructive symptoms (Figure 7-9, and Figures 16,17 [Color Plate 13]). A mass may be palpated in the neck. Biopsy is difficult because the tumor is firm and normal mucosa is likely to be obtained. The recurrent nerve is not initially affected, but voice change occurs due to tumor bulk. Hemoptysis is very rare. These lesions are usually chondrosarcomas of low grade and often are indolent in behavior. Treatment is surgical excision with every effort made to save the larynx. Techniques must be individualized. The reconstructive approach is outlined in Chapter 25, "Laryngotracheal Reconstruction." With conservative approach to maintain laryngeal function, recurrence must be watched for over a long term, with likelihood of later re-resection necessary years later. Salvage laryngectomy is eventually needed in some cases. With recurrences, atypical areas and dedifferentiation are seen.28,30,31 Chondrosarcoma does not respond to radiotherapy.
Tracheal chondral tumors seem to be even less common than laryngeal tumors. Limited experience suggests that these are most common in older males.32 They may vary from benign chondroma to chondrosar-coma of increasing malignancy (Figure 7-10). Lesions may be largely intraluminal or extend through the tracheal wall.33-35 Nonproductive cough is followed by dyspnea, first on exertion and then at rest, with wheezing—especially on recumbency—late in the course. With chest x-rays initially "clear," the diagnosis of asthma is frequently carried for a long period. A medical student with a tracheal chondroblastoma was retrospectively identified as having had tumor present radiologically for 7 years (Figure 7-11). Cartilaginous tumors are radiologically defined on all imaging modalities. Calcification is frequently seen as well as destruction of normal cartilages, especially in the larynx. A bulky extraluminal mass may be visible. Treatment is surgical excision with tracheal reconstruction. The approach is directed by the location and extent of tumor (see Chapter 24, "Tracheal Reconstruction: Anterior Approach and Extended Resection," Chapter 25, "Laryngo-tracheal Reconstruction," and Chapter 28, "Reconstruction of the Lower Trachea [Transthoracic] and Procedures for Extended Resection"). Complete excision is necessary to prevent recurrence and hence should be a patient's first procedure. Bronchoscopic debulking is not definitive treatment. Further, tumors apparently progress in degree of malignancy or transform from being benign to malignant.36
True cartilaginous tumors are distinguished from hamartomas by the absence of other tissue elements that are seen in hamartomas: lipomatous, epithelial, and lymphoid. Our patients with chondroma
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