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Adapted from Grillo HC and Mathisen DJ.1

Adapted from Grillo HC and Mathisen DJ.1

tracheal tumor is histologically indistinguishable from that found in salivary glands, it is indeed rare for a single patient to present with tumors, either synchronous or metachronous, both in the salivary gland and in the trachea. The airway tumor may occur at any level of the trachea, but seems to be more prevalent in the lower trachea and carina. It occurs much less often in main bronchi and very rarely distal to that. Multiple tumors are extremely rare.

Adenoid cystic carcinoma presents grossly in a variety of ways (Figures 7-3, 7-4, 7-5, and Figures 7 through 11 [Color Plates 12, 13]). The lesions may be red and rubbery in appearance or granular and friable. One or all borders may appear sharply defined or the tumor may infiltrate diffusely. Frequently, adjacent to the main mass of tumor, which clearly projects from the mucosa, there is evidence of tumor infiltration beneath the mucosa or in the tracheal wall. The margins are often indistinct when examined with a magnifying telescope through a rigid bronchoscope. Mucosal elevation or friability is seen and new tumor vessels may be identified in the mucosa. Commonly, these cancers invade microscopically submucosally and perineurally for long distances beyond grossly visible disease. Microscopic pathology is described in Chapter 3, "Pathology of Tracheal Tumors." The lesion may be essentially circumferential within the trachea or may involve both main bronchi at the carinal level. Proximal tumors may involve the larynx in varying degrees. The extratracheal tumor mass may become bulky. Although these tumors may grow to considerable size extramurally into the mediastinum prior to recognition, they often displace adjacent structures before invading them, including the esophagus and pulmonary artery. I have seen the left pulmonary artery shown to be completely occluded by the pulmonary angiogram, but at operation found only compression, without invasion of the pulmonary arterial wall. The esophagus is more likely to be directly invaded by a posterior tumor, especially in its muscular wall. A recurrent laryngeal nerve may be defunctioned by direct invasion. Adjacent paratracheal regional lymph nodes on both sides may be involved, but apparently not as frequently as by SCC of the trachea. For a tracheal tumor, these should be considered as N1 nodes.

Late local recurrence of ACC, even 15, 20, or more years after apparent cure by surgery and irradiation, is a discouraging characteristic. Despite almost uniformly excellent early response to external irradiation treatment, recurrence usually follows if irradiation alone is used for treatment. Recurrence is seen at the prior site of the maximum bulk of tumor, usually between 3 to 7 years following treatment. Improved long-term outcome seems to follow combined surgical resection and full-dose radiotherapy, but these results must be regarded with reservation in view of the tumor's indolent clinical behavior and the small amount of information about long-term behavior.6,11,12 Metastases to the lung occur all too frequently, but

figure 7-3 Adenoid cystic carcinoma. Bronchoscopic views. A, Tumor invading the subglottic larynx in a 54-year-old man, at and above the cricoid level, with essentially circumferential extension. Resection with laryngeal salvage was impossible. The patient elected palliative neutron beam radiation to preserve laryngeal speech. Tumor recurred, as expected, in 6years. B, Adenoid cystic carcinoma confined to midtrachea, 2 x2.7cm, in a 64-year-old man. The tumor, 4.5 cm from the carina, was removed by cervicomediastinal tracheal resection. C, Tumor at the carina and in the left main bronchus, also extending into the proximal right main bronchus. Left carinal pneumonectomy was done via a "clamshell" incision.

may emerge late and enlarge slowly. Patients may remain asymptomatic for many years with multiple enlarging pulmonary metastases. Indeed, such behavior may justify surgical removal of a tracheal lesion despite the presence of numerous lung metastases, in order to prevent airway obstruction. Experience is small, but excision of pulmonary metastases has almost never produced cure or seemed to alter the course of the disease. Usually, metastases are too numerous for complete removal. Where a few metastases have been resected, other metastases have usually appeared later. Metastases to bone and other organs also occur.

In a minority of cases, although no unusual histologic characteristics were identified, ACC proved to be rapidly and widely aggressive. Manifestations in these few patients included local invasion of the central pulmonary artery, intrapericardial extension, multiple pleural metastases, and multiple rapidly-growing pulmonary or osseous metastases. The primary tumor in such a patient is not necessarily large.

figure 7-4 Radiologic presentation of adenoid cystic carcinoma (ACC). A, Small, well-defined tumor of the upper trachea, delineated by tomography. The vocal cords and subglottic larynx are well outlined. The involved right wall of trachea is deformed. The right recurrent laryngeal nerve was involved. This 25-year-old man underwent cervicomediastinal resection and also received 5,000 cGy of irradiation postoperatively. He remained well and without recurrence 30 years later. B, Tomographic detail. Broad-based ACC in a 40-year-old woman extending into the cricoid cartilage on the left (arrow). The left side of the lower larynx was included in the resection. The left recurrent laryngeal nerve had to be resected. The trachea was bevelled to fit the line of laryngeal resection. Laryngeal structures are visible above. Vocal cords are widely abducted. C, Computed tomography scan in a 39-year-old woman shows the midtrachea encircled by ACC. She was treated by segmental tracheal resection. D, Large tumor at the carina obstructing the left main bronchus totally and the right bronchus subtotally. Left carinal pneumonectomy was necessary.

figure 7-5 Gross surgical specimens of adenoid cystic carcinoma. A, Tumor with large extratracheal component and involved peritracheal lymph nodes. B, A 6.5 cm resection for an extensive tumor of the midtra-chea in a 43-year-old man. Complete sternotomy plus extension into the right thorax was necessary for removal. Laryngeal release was also required. A tentative initial distal incision in the trachea was made, but was too close to the tumor. C, Open specimen of B, showing the intraluminal extent of tumor. D, Another extensive tumor in a 27-year-old man, involving distal trachea, carina, and left main bronchus. Left carinal pneumonectomy was necessary via bilateral thoracotomy, with elevation of the right main bronchus to proximal trachea. Laryngeal release helped because of the high level of anastomosis. Irradiation followed. The patient is now 22 years after resection.

Over 40 years, between 1962 and 2002, 135 patients with ACC and 135 with SCC were seen at MGH.6 In the second 20-year period, 97 with ACC and 98 with SCC were seen. Of these, 101 (75%) with ACC and 90 (67%) with SCC were resected.

A miscellaneous group of other primary tracheal carcinomas has also been encountered, usually with so few examples of each type that little can be said about general behavior patterns. These neoplasms include adenocarcinoma in the carina of a child at the base of a bronchogenic cyst. Carinal excision following prior removal of the cyst at another hospital led to cure. Adenosquamous carcinoma was encountered, involving both the trachea and the lower larynx, and was treated by cervical mediastinal excision with mediastinal tracheostomy. Another such tumor, involving the right main bronchus and carina, was treated successfully (15-year survival) by carinal resection and right upper lobectomy with anastomosis of the bronchus intermedius to the left main bronchus and the left main bronchus to the trachea. Two instances of small cell carcinoma of the trachea were encountered, reconfirmed on pathological review, with no involvement of adjacent lung and no nodal metastases. These patients remained disease free over many years after surgical resection plus conventional adjuvant therapy for small cell carcinoma. The rarity of primary tracheal small cell carcinoma was observed elsewhere.13 A greater frequency of small cell carcinoma in the trachea, which was described in the past, probably included secondary invasion from the lung.14

Lymphoepithelial carcinoma (Schmincke tumor), more often occurring in the nasopharynx, has been seen in the trachea.15 Treatment was resection and irradiation.

It appears that melanoma can occur primarily in the trachea. As in the patient reported by Duarte and colleagues,16 our single patient had undergone removal of a small cutaneous lesion, without histologic record, many years before. Lower tracheal resection was done. However, the patient had no subsequent recurrence of melanoma in 13-year follow-up.

Except for 11 patients with carcinoid tumor of the trachea and carina, which we noted in 1978, carci-noid has not often been reported in the trachea.17 Behavior of carcinoid in the trachea and carina appears to be much the same as in the bronchi, where it is much more common (Figure 7-6, and Figures 12 through 15 [Color Plate 13]).18 The rarer atypical variety may be highly malignant. Typical carcinoids, when resected with limited but negative histologic margins, did not recur. In these cases, no regional nodes were involved. Carcinoid syndrome is seen only rarely with bronchial carcinoid, often, but not uniformly, in the presence of hepatic or other metastases. One patient with carcinoid syndrome from a tracheal tumor was referred with massive mediastinal lymph node metastases following resection of an atypical tracheal carci-noid. Block removal of lymph nodes temporarily controlled the syndrome until tumor recurred. Carcinoid appears to respond poorly to irradiation. We have not encountered Cushing's syndrome with tracheal car-cinoids, possibly a reflection of the low incidence of this hormonal effect in these tumors.19

Histological examination of the wall of the trachea from which a typical carcinoid tumor arises usually demonstrates deep enough origin that it is impossible to remove the tumor definitively intraluminally with a laser or by other means (see Figure 3-12 [Color Plate 2] from Chapter 3, "Pathology of Tracheal Tumors"; Figure 7-6F). Only transient relief of obstructive symptoms can be obtained by debulking the tumor within the lumen. Full-thickness excision of the tracheal wall is required for complete removal. On the membranous wall, a carcinoid tumor of moderate size often extends through the posterior wall. Claims of cure appear to be either an illusion or such a rarity that harm may be done by encouraging delay in accomplishing simple curative surgical extirpation.

Mucoepidermoid tumor presents in the trachea and main bronchi, with malignant behavior in a small proportion of cases in most series.20,21 In Heitmiller and colleagues' series from MGH, 3 of 18 patients with mucoepidermoid tumors of the trachea and bronchi were high grade and fatal within 16 months.21 All the others survived (Figure 7-7). Nodal spread was infrequent in low-grade tumors. Two tracheal tumors were treated by sleeve resection, one by laryngotracheoplastic resection, and another underwent carinal

figure 7-6 Carcinoid tumors. A, Bronchoscopic view. Carcinoids are often smooth, rounded, and vascular in appearance. B, Irregular carcinoid located just above the carina. C, Radiographic details of the carina of a 31-year-old woman treated for "asthma" with progressive dyspnea for over a year. Anteroposterior view on left radiograph and lateral view on right radiograph. Both show the lower tracheal tumor clearly.

figure 7-6 Carcinoid tumors. A, Bronchoscopic view. Carcinoids are often smooth, rounded, and vascular in appearance. B, Irregular carcinoid located just above the carina. C, Radiographic details of the carina of a 31-year-old woman treated for "asthma" with progressive dyspnea for over a year. Anteroposterior view on left radiograph and lateral view on right radiograph. Both show the lower tracheal tumor clearly.

pneumonectomy. Bronchial sleeve resection sufficed for another 5 patients, and lobectomy was needed in 7. Jensik, Faber, and colleagues made similar observations earlier. Irradiation treatment of high-grade tumors seemed ineffective.20 In 31 children and adolescents, in whom the tumor was located in the bronchi, prognosis appeared to be better than in adults, with no recurrence seen after resection alone.22

figure 7-6 (continued) D, Computed tomography scan of the patient described in C. E, Gross specimen of the patient in C, exhibiting a typical carcinoid in character and appearance. F, Carcinoid invading the carina in a 19-year-old female. Operative photograph shows protrusion of tumor through the membranous wall of the trachea. The left-hand tape encircles the trachea. The two on the right encircle the right main (above) and left main (below) bronchi. The futility of laser treatment, which she had on several occasions, is obvious.

Two pleomorphic adenomas in the trachea were excised in our series (Figure 7-8, and Figure 18 [Color Plate 13]). These tumors are rare, may occur in children, and produce obstructive symptoms. They are mixed tumors of salivary gland type with varied mixtures of epithelial and stromal components. By 1992, 19 cases had been gathered.23 Similar tumors in the bronchus were first described by Payne and colleagues.24 Complete surgical excision and reanastomosis is the proper treatment. One of our patients had a similar tumor excised years before from a salivary gland, but there was no evidence then or since of metastatic disease. It was, therefore, classified as a primary tracheal tumor. Malignant features evidently occur, but were not present in these patients.

figure 7-7 Mucoepidermoid tumors. A, Lateral neck roentgenogram in a 28-year-old woman showing a posterior lesion (arrows) involving the subglottic larynx and upper trachea. Laryngoplasty was done, advancing the posterior membranous tracheal wall to resurface the posterior larynx after tumor excision. Both recurrent nerves remained intact. B, Carinal resection for mucoepidermoid tumor in a 34-year-old man, viewed from the tracheal end of the specimen. Twelve year follow-up was without recurrence.

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