General Thoracic Surgery (General Thoracic Surgery (Shields)) [2 VOLUME SET]
Editors: Shields, Thomas W.; LoCicero, Joseph; Ponn, Ronald B.; Rusch, Valerie W.
Title: General Thoracic Surgery, 6th Edition
Copyright 2005 Lippincott Williams & Wilkins
> Table of Contents > Volume I - The Lung, Pleura, Diaphragm, and Chest Wall > Section XIII - The Trachea > Chapter 78 - Benign and Malignant Tumors of the Trachea
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Chapter 78
Benign and Malignant Tumors of the Trachea
Penfield L. Faber
William H. Warren
Despite their histologic similarity to tumors of the main-stem bronchus and lung, tumors of the trachea are approximately 100 times less common than bronchial tumors and constitute only 2% of all upper respiratory tract tumors, as reported by Perelman and Koroleva (1987). Tracheal cancer is responsible for less than 0.1% of all cancer deaths, as noted by Pearson and Gullane (1995). The most common malignant tumors are squamous cell carcinoma and adenoid cystic carcinoma.
Malignant tumors of the trachea are more common than benign tumors. Houston and associates (1969) reviewed 30 years of experience at the Mayo Clinic, and 53 of 90 tracheal tumors were malignant. Hajdu and colleagues (1970) reported a series of 41 primary malignancies of the trachea that were treated over a period of 33 years at a major cancer hospital. This number represents slightly more than one malignant tracheal tumor per year seen at a major referral center, which emphasizes the relative rarity of these tumors. In a center study of 208 patients with primary tracheal tumors reported by Regnard and associates (1996), 181 were malignant and 27 were benign; however, in a review of 43 tracheal tumors occurring in infants and children, Gilbert and associates (1953) noted that 93% were benign. Desai and associates (1998) reviewed 36 reported tracheal tumors in infants and children from 1965 to 1995; 23 were benign and 13 were malignant. Malignant fibrous tumors and mucoepidermoid carcinoma were the more common malignant variants in children. The predominant benign tumors in children are hemangiomas, fibromas, and papillomas.
Secondary tumors also involve the trachea. Direct extension into the trachea occurs from cancers of the thyroid, larynx, lung, and esophagus. Mediastinal tumors may directly invade the trachea; the most common is lymphoma. Metastasis to the trachea is uncommon, but breast cancer, melanoma, and sarcomas have all been found in the trachea.
SYMPTOMS AND FINDINGS
Dyspnea and shortness of breath on exertion are the most common presenting symptoms and, according to Perelman and Koroleva (1987), occur when the tracheal lumen has been reduced to one-third of its normal cross-sectional area. Perelman and co-workers (1996) noted inspiratory dyspnea in 80% of patients. Regnard and associates (1996) reported that 42% of 208 patients had a presenting complaint of dyspnea. Cough is a common symptom associated with tracheal neoplasms, but no particular salient features are associated with the cough to indicate that it is caused by a tracheal tumor. As the airway narrows, the classic symptom of wheezing becomes apparent. Stridor is a more prominent form of wheezing and indicates significant compromise of the airway. Often, a patient with a tracheal tumor is treated for asthma for a prolonged period.
Hemoptysis occurs in approximately 20% of patients with tracheal neoplasms and is most commonly seen in patients with squamous cell carcinoma. People with benign tumors rarely, if ever, present with hemoptysis. A change in voice quality can be related to paralysis of the vocal cord resulting from invasion of the recurrent laryngeal nerve or by direct extension of an upper tracheal tumor into the larynx. Recurrent pneumonitis, either unilateral or bilateral, can occur from obstruction of a main bronchus.
Perelman and Koreleva (1987) reported that the interval between the onset of early symptoms and diagnosis was approximately 25 months for benign tumors and 8 months for malignant tumors. Regnard and associates (1996) noted that the mean duration of symptoms for patients with adenoid cystic carcinoma was 12 months, and for those with tracheal cancers it was 4 months. The long duration of symptoms is emphasized by the finding of Perelman and colleagues (1996) that 23% of patients with tracheal tumor arrived at the Center for Surgery with life-threatening asphyxia. Regnard and associates (1996) also noted acute respiratory failure in 29% of patients.
Dysphagia is an uncommon symptom that indicates esophageal compression by a large, bulky neoplasm. Auscultation of the chest reveals a coarse wheeze that is enhanced by rapid and deep inspiration. The wheeze is more prominent on inspiration than on expiration and is different from the wheezing commonly associated with bronchial asthma.
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Obstruction from a tracheal tumor can be heard if the examiner places an ear close to the patient's open mouth during deep and forceful breathing. This is a simple but major finding associated with laryngeal or tracheal obstruction. The neck and supraclavicular fossae should be examined carefully for evidence of enlarged lymph nodes, which may indicate spread of a malignant tumor. Locations of tumors in the trachea, as reported by Perelman and associates (1996), were the thoracic trachea in 77, the cervical trachea in 26, and the carinal region in 41 patients.
DIAGNOSIS
Radiographic Features
Careful inspection of the tracheal air column on posteroanterior and lateral chest radiographs sometimes reveals a tracheal tumor (Fig. 78-1). Oblique views of the trachea and lateral neck radiographs and hyperextension reveal the presence of the tumor but do not provide specific information necessary for a planned resection and reconstruction. Linear tomography, as described by Weber and Grillo (1992), provides excellent visualization of the extent of the tumor (Fig. 78-2). Tracheal tomograms, however, are not effective for determining extraluminal extension of the tumor or lymph node invasion, and this technique is no longer available in most centers.
Computed tomography (CT) has generally replaced linear tomography as the primary method of radiologic evaluation
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of a tracheal tumor. Mediastinal extension, esophageal compression, and tracheal lumen size are all seen clearly on the CT scan (Fig. 78-3). Using thin-cut CT sections and knowing the distance between the cuts can permit a moderately accurate measurement of the length of trachea that is involved by the tumor. Gross pathologic characteristics of the tumor are also identified on CT scan. Benign lesions are frequently round and smooth and approximately 2 cm in diameter. The tumor is generally inside the lumen of the trachea, and the well-circumscribed nature of the tumor is clearly evident. Calcification is a characteristic of benign lesions and is seen in tumors such as chondromas and hamartomas (Fig. 78-4). Calcification is also present, however, in a chondrosarcoma. Malignant tumors extend up and down the trachea for several centimeters, and the surface of the tumor is irregular and possibly ulcerated. The tracheal wall is obviously invaded by the tumor at its base, and extraluminal growth may be present (Fig. 78-5). Enlarged lymph nodes usually indicate metastatic spread. The CT scan should always be done with infusion to delineate clearly the relationship of the tumor to the superior vena cava and other vascular structures in the mediastinum. No specific radiologic findings that differentiate malignant tumors of the trachea exist.Fig. 78-1. A. A chondroma can be seen on this frontal chest radiograph (arrow). B. Tracheal hamartoma is clearly identified on this lateral chest radiograph (arrow). |
Fig. 78-2. Linear tomogram clearly depicts the length of involvement of a carcinoid tumor of the trachea (arrows). |
Fig. 78-3. Computed tomogram identifies posterior mediastinal extension of an adenoid cystic carcinoma. |
Fig. 78-4. The computed tomogram demonstrates almost total calcification of a tracheal chondroma (arrow). |
Fig. 78-5. Computed tomographic scan shows adenoid cystic carcinoma extending through the tracheal cartilage (arrow). |
Magnetic resonance (MR) imaging can offer some advantages in the assessment of tracheal neoplasms. Coronal, oblique, and sagittal views can be obtained that demonstrate long lengths of the trachea and delineate a more precise length of tracheal involvement by the tumor. T1-weighted images characterize the anatomy of the trachea and adjacent soft tissues quite well. MR imaging also clearly depicts adjacent vascular structures and permits assessment of possible invasion of these structures. MR angiography can be used in place of conventional angiography when vena cava obstruction is evaluated.
Three-dimensional helical CT of the central airway can provide precise anatomic information for the planning of endobronchial and surgical procedures. Kauczor and associates (1996) evaluated 36 patients with proven airway obstruction. The majority had bronchial carcinoma or mediastinal or hilar lymphadenopathy. Bronchoscopic correlation was completed in all patients. Anatomy of the lesions was clearly depicted and closely correlated with the bronchoscopic findings. Three-dimensional helical CT provided additional information for assessing the distal airway when the bronchoscope could not be passed through the obstructing lesion. This radiologic technique will become most valuable in planning tracheal resections or in monitoring palliative efforts for tracheal neoplasms.
If the patient complains of dysphagia, a barium examination of the esophagus may demonstrate compression or possibly
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invasion. This study may also further define the extraluminal extent and size of the tracheal neoplasm.Pulmonary function studies reveal airway obstruction characterized by a reduced volume of air expired in 1 second, a significantly decreased peak flow rate, and a flattened expiratory flow volume loop. Maximum voluntary ventilation is diminished. Pulmonary function tests may also delineate the presence or absence of parenchymal lung disease.
Bronchoscopy
Bronchoscopy is a necessary step in the diagnosis and clinical evaluation of a patient with a tracheal tumor. Biopsy and manipulation of a tracheal tumor is potentially hazardous, however, because bleeding can cause complete tracheal obstruction. A sedated or anesthetized patient may be unable to maintain adequate ventilation, and the passage of an endotracheal tube may not be possible because of the obstructing tumor. The bronchoscopic examination should be carried out by an experienced endoscopist, who can insert an open tube bronchoscope through the tumor to establish an airway and manage any complications of hemorrhage.
A bronchoscopic examination is always conducted in the operating room, where ventilating bronchoscopes and biopsy forceps are available and a trained anesthesiologist is close at hand. Grillo (1989) believes that when the indications for primary tracheal resection are clear-cut, bronchoscopy can be deferred until the time of the operative procedure, and frozen sections can be used to determine histology. With the flexible fiberoptic bronchoscope, however, a careful examination and biopsy can be accomplished in the awake patient. A preresection bronchoscopy offers several advantages:
Vocal cord function is evaluated, and the entire larynx and cricoid cartilage are seen clearly. This visualization is particularly important for upper tracheal lesions in which a partial resection of the cricoid or laryngectomy may be required.
The gross characteristics of the tumor are noted, and an impression can be gained whether the tumor is benign or malignant.
The size of the tracheal lumen is clearly noted. This assessment is extremely helpful in planning anesthetic management of the airway during the initial phase of tracheal resection.
A biopsy sample can be obtained with the small biopsy forceps through the flexible instrument, and knowing the histology is of benefit in planning the treatment program.
Frequently, the small flexible fiberoptic bronchoscope can be inserted past the neoplasm, and the distal airway can be carefully examined. The length of the tumor can be carefully measured and correlated with the radiologic measurements.
These findings are extremely helpful in planning the surgical approach and resection. However, the endoscopist must be prepared to insert an open tube bronchoscope if airway obstruction does occur.
The bronchoscopy is performed with the flexible fiberscope passed through a topically anesthetized nasopharynx to a position above the larynx. Supplemental oxygen is provided through the mouth. Several aliquots of 4% lidocaine (Xylocaine) are instilled on and through the vocal cords until satisfactory topical anesthesia is achieved. The vocal cords and larynx are examined carefully, and the bronchoscope is then passed into the trachea to visualize the tumor. Decision for biopsy depends on the vascularity of the tumor and the size of the tracheal lumen. The decision for biopsy requires careful judgment, and the biopsy should not be done if there is any possibility of airway compromise (Fig. 78-6). Houston and colleagues (1969) reported that of 53 primary cancers of the trachea, the diagnosis was established by bronchoscopic biopsy without complication in 47 patients (Fig. 78-7).
When life-threatening airway obstruction occurs from a tracheal tumor or bleeding from a biopsy obstructs the airway, an adequate tracheal lumen can be established by coring out the tumor with the rigid bronchoscope and biopsy forceps, as described by Mathisen and Grillo (1989). Regnard and associates (1996) recommend the relief of tracheal obstruction to better define location and size of the tumor and to provide a more adequate airway. In their series of 208 tracheal tumors, airway obstruction was relieved by
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laser treatment in 62 of 71 patients, by bronchoscopic d bridement in 5, and by cryotherapy in 2. Tracheostomy was done to provide an adequate airway in 2 patients. Daddi and associates (1998) described several advantages associated with the endoscopic treatment of airway obstruction before attempted curative resection. They include the improvement of respiratory function, a better assessment of the exact location of the tumor by endoscopic means, more reliable CT scanning of the airway, and improvement of the patient's performance status with associated pulmonary therapy and antibiotics. Endoscopic d bridement was accomplished with ventilating rigid bronchoscopes and the neodymium:yttrium-aluminum-garnet (Nd:YAG) laser.Fig. 78-6. Adenoid cystic carcinoma partially obstructs the tracheal lumen. The flexible bronchoscope passed easily, and biopsy was done with evaluation of the distal trachea. |
Fig. 78-7. A trachea almost totally occluded by adenoid cystic carcinoma (arrow). Bleeding from biopsy or manipulation could be life threatening. |
Schneider and colleagues (2001) described endoscopic intervention in eight patients with symptomatic tracheal neoplasms. Significant airway obstruction necessitated tumor ablation with the Nd:YAG laser. Clinical stabilization was achieved prior to resection.
BENIGN TUMORS
Gilbert and associates (1953) reported that the common benign tumors of the trachea are chondroma, papilloma, fibroma, and hemangioma. Benign tumors most often occur in the upper one-third of the trachea in children and are more common in the lower one-third in adults, frequently arising from the membranous portion of the trachea (Table 78-1).
Table 78-1. Benign Tracheal Tumors | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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Chondroma
According to Mark (1983), the most common benign mesenchymal tumor of the trachea is a chondroma (Fig. 78-8). These tumors histologically duplicate normal cartilage and can exhibit vascular invasion. Endoscopically, a chondroma appears as a firm, white nodule projecting into the lumen of the trachea. The tumor occurs in a 4:1 preponderance in men, and it is more common in adults than in children. No definite etiology for this lesion has been described. A chondroma occurs more frequently in the larynx than in the trachea. Biopsy of the lesion can be difficult because of its firm consistency, and this characteristic can indicate the diagnosis. Vascularity is minimal, and the lesion can be removed easily through the bronchoscope. Recurrence after endoscopic removal has been observed, however, and Salminen and colleagues (1990) reported malignant transformation to chondrosarcoma. Recommended treatment is segmental tracheal resection.
Papilloma
A solitary papilloma of the trachea is rare, but this lesion does occur in adults, as described by Maxwell and colleagues (1985). Papillomas are sessile growths lined by squamous epithelium covering fibrovascular cores. Cytologic atypia may be present. A solitary benign papilloma is easily removed through the open tube bronchoscope, and
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the base of the tumor can be ablated with the Nd:YAG laser. Periodic endoscopic surveillance is indicated, and recurrence can be treated with laser ablation.Fig. 78-8. A. Linear tomogram depicts a calcified chondroma (arrows). B. The benign chondroma is removed by segmental tracheal resection. C. Microscopic examination reveals areas of ossification in a largely cartilaginous tumor. |
Juvenile laryngotracheal papillomatosis is common in children and is seen more frequently than is solitary papilloma of the trachea. It accounts for 60% of benign tracheal tumors in children, according to Beattie and associates (1992). It has been linked with the human papilloma virus types 6 and 11. Papillomatosis more commonly involves the larynx, but it is found in the tracheobronchial tree in 20% of patients. It follows a relatively benign course, requiring repeated endoscopic removal, with recurrence rates as high as 90%. Complications include chronic cavitary respiratory papillomatosis resulting from proliferation of the virus in the distal bronchial tree, as noted by Karley and colleagues (1989). Malignant transformation of papillomatosis has been reported in patients with a history of radiation therapy or smoking, but it may also occur in nonsmokers, as noted by Guillou and associates (1991). The types of therapy for the more invasive form of the disease include photodynamic therapy with sensitization of the papilloma cells using hematoporphyrin diacetate, as described by Kavuru (1990) and Basheda (1991) and their colleagues. Leventhal and associates (1991) reported successful treatment with the use of lymphoblastoid interferon- -N1.
Fibroma
Fibroma accounts for approximately 20% of all benign tumors in adults, according to Beattie and colleagues (1992). Mark (1983) reported that fibroma is more common than fibrosarcoma and can be difficult to distinguish from a fibrous histiocytoma. The tumor is well circumscribed and consists of fibroblasts embedded in intercellular collagen. A benign fibroma is easily removed through the bronchoscope, followed by careful laser ablation of the base of the tumor. Local recurrence would be unusual, but if it does occur, segmental tracheal resection would then be indicated.
Hemangioma
Mark (1983) noted that hemangioma of the trachea is similar to hemangioma of the skin in infants, with an increase in size at 1 month of age followed by a spontaneous decrease in size at 1 year. It may arise in the trachea or extend into the tracheal lumen from a hemangioma located in the mediastinum. Treatment may require tracheostomy to provide an adequate airway, followed by repeated small doses of radiation therapy to shrink the tumor. Weber and co-workers (1990) used steroids to cause regression. Many lesions require no treatment, and natural regression frequently occurs. Larger hemangiomas of the lower trachea may require direct surgical intervention. In this instance,
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a careful plan must be developed for airway control during excision of the tumor. Franks and Rothera (1990) used cardiopulmonary bypass to resect a low-lying tracheal hemangioma.
Other Benign Tumors
Granular Cell Tumor
The granular cell tumor occurs less frequently in the trachea than it does in the tongue, neck, or larynx. Burton and associates (1992) summarized the reported experience with this tumor in the trachea and identified 24 cases. The tumor is thought to be of neurogenic origin and derives from Schwann cells. Malignant transformation of the granular cell tumor does occur in other sites, but it has never been reported in the trachea. Both endoscopic and partial tracheal resection have been successful as treatment of this lesion. Burton and associates (1992), however, described a case of local recurrence after endoscopic resection that then required partial tracheal resection. Daniel and colleagues (1980) recommended removal of tumors larger than 1 cm by a segmental tracheal resection because of the increased risk for full-thickness wall involvement with tumors of this size. Smaller lesions (<1 cm) are easily ablated by endoscopic Nd:YAG laser therapy. Cunningham and co-workers (1989) recommend the use of bipolar cautery. Endoscopic therapy would certainly be applicable to large tumors as well, with resection reserved for local recurrence, because malignant transformation of this tumor in the trachea has yet to be reported.
Fibrous Histiocytoma
Fibrous histiocytoma is a histologically benign tumor in the trachea, but it can be locally infiltrative (Fig. 78-9). An associated prominent inflammatory component can cause the tumor to be termed an inflammatory pseudotumor. The behavior of this tumor in the trachea appears to be benign, but because of its local infiltration, segmental resection is the treatment of choice. Resection of a malignant fibrous histiocytoma of the trachea was reported by Randleman and associates (1990), with identification of one other case in the literature.
Glomus Tumor
Glomus tumor is a benign neoplasm arising from specialized cells surrounding arterial venous anastomoses. Garcia-Prats and colleagues (1991) reviewed the literature and found six cases of tracheal origin. This tumor can be confused histologically with the carcinoid tumor, and immunohistochemistry is recommended for confirmation. Menaissy and co-workers (2000) reviewed nine cases of glomus tumor of the trachea and noted that the tumor can extend outside the tracheal wall. Segmental tracheal resection is recommended, with no expected recurrence.
Fig. 78-9. A broad-based fibrous histiocytoma (arrows) removed by segmental tracheal resection. |
Lipoma
Tracheal lipoma is a rare lesion, with five cases reported (Fig. 78-10). Chen and associates (1990) described a patient with a tracheal lipoma requiring major tracheal resection for complete removal. Other authors describe endoscopic removal. It is only logical to approach this tumor with endoscopic removal because it is a completely benign tumor, and any local recurrence can be treated successfully with laser therapy.
Leiomyoma
A leiomyoma may occur as a primary tracheal tumor; according to Mark (1983), it usually occurs in the distal one-third of the trachea. Both tracheal resection and endoscopic removal have been described in rare case reports.
Neurofibroma
The neurofibroma can occur in the trachea as a primary tumor, but it is not associated with generalized neurofibromatosis. This tumor can invade the wall of the trachea, and segmental resection would be the treatment of choice.
Pang (1989) presented two cases of primary neurilemoma (schwannoma) of the trachea. His review identified
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14 other reported cases. These tumors derive from the Schwann cell and are typically slow growing. The tumor usually has a broad base, and complete removal by bronchoscopy would be difficult. These tumors can recur with malignant potential, and segmental tracheal resection is the treatment of choice.Fig. 78-10. A. Computed tomogram shows almost total tracheal obstruction by a lipoma (arrow). B. Tracheal resection was required for the removal of the broad-based lesion. C.On microscopic examination, the fatty tumor is seen in the submucosa of the trachea. |
Hamartoma
Hamartoma is a benign tumor composed of tissues normally found in the organ or structure in which it is located, but the normal-appearing tissues are not in their normal histologic pattern. Most hamartomas are found in the pulmonary parenchyma, but 10% are located in the main bronchus or trachea. They are polypoid in appearance and can cause symptoms of respiratory obstruction. The CT scan can be diagnostic in detecting fat and calcification in a smooth and rounded lesion. They do not become malignant, and endoscopic resection is the treatment of choice (Fig. 78-11).
MALIGNANT PRIMARY TRACHEAL TUMORS
The most frequent malignant primary tracheal tumors in the adult are squamous cell carcinoma and adenoid cystic carcinoma. Manninen and associates (1991) reported a national registry study in Finland of tracheal carcinoma that demonstrated the overall rarity of this malignancy. They noted that primary carcinoma of the trachea accounted for 0.03% of all detected malignancies registered in Finland between the years 1967 and 1985. Tracheal malignancy accounted for less than 0.2% of all malignancies of the respiratory tract over the same period, and squamous cell carcinoma was the most common tumor. In a review of 198 patients with tracheal tumors, Grillo and Mathisen (1990) reported 80 patients with adenoid cystic carcinoma and 70 with squamous cell carcinoma. In a series of 144 patients who underwent surgery for primary tumors of the trachea, Perelman and associates (1996) recorded 66 adenoid cystic carcinomas and 21 squamous cell carcinomas. Squamous
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cell carcinomas seem to be more prominent in Europe. Regnard and associates (1996) reported 94 squamous cell carcinomas and 65 adenoid cystic carcinomas in 181 malignant tumors of the trachea, and Gelder and Hetzel (1993) recorded 174 squamous cell carcinomas and 34 adenoid cystic carcinomas in 272 reported cases (Table 78-2).Fig. 78-11. A. Bronchoscopic appearance of a tracheal hamartoma. B. Resection was accomplished through a rigid, open bronchoscope. C. Fibrous hamartoma. Microscopic examination reveals dense connective tissue, fat, and smooth muscle in tracheal submucosa. |
Squamous Cell Carcinoma
Squamous cell carcinoma of the trachea occurs most commonly in the distal one-third of the trachea and originates frequently along the posterior wall (Fig. 78-12). It affects men approximately four times more frequently than women, spreads to regional lymph nodes, and invades adjacent mediastinal structures. It accounts for approximately 50% of all primary tracheal malignancies, and most patients are heavy smokers. It is not unusual for these patients to develop or have had a secondary primary cancer of the larynx or lung. At the time of diagnosis, Mark (1983) states that invasion into the tracheal wall has occurred in 50% of patients, extension into the mediastinum in 33%, and metastasis to cervical lymph nodes in 33%. Grillo and associates (1992a) noted that in approximately two-thirds of patients with squamous cell carcinoma, the lesion is resectable at the time of presentation. Limitations to resectability include an excessive linear extent of the tumor, leaving insufficient trachea for reconstruction; invasion of critical mediastinal structures; and distant metastasis. In patients with squamous cell carcinoma, the status of lymph nodes in the mediastinum at the time of resection and the presence of tumor at the margin of surgical resection serve as major determinants of prognosis.
Adenoid Cystic Carcinoma
Adenoid cystic carcinoma more commonly arises in the upper one-third of the trachea, in contrast to squamous cell
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carcinoma (Fig. 78-13). In many series, it is the most common tracheal malignancy. According to Mark (1983), it is proportionately more prevalent in the trachea than in the main-stem bronchus. This tumor is frequently referred to as a cylindroma, but this terminology should be abandoned because it implies that the tumor is benign. The adenoid cystic carcinoma is a slow-growing neoplasm, and patients frequently have experienced symptoms for longer than 1 year before diagnosis is made. This tumor arises from bronchial glands and is histologically identical to those that arise in the salivary glands. This low-grade tumor is composed of uniform cells arranged in sheets and contains well-definedP.1071
glandular lumens. Individual cells are arranged in epidermoid fashion. Thus, there is an association of mucus-secreting and epidermoid cells, accounting for the designation mucoepidermoid. The high-grade form has cytologic atypia, mitoses, and areas of necrosis. Lymphatic and vascular invasion are more frequent in higher-grade tumors. Tobacco exposure is not a risk factor.Table 78-2. Numbers of Patients with Malignant Tumors of the Trachea | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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Fig. 78-12. A. Extensive tracheal squamous cell carcinoma (arrows) is seen on the radiograph of the chest. B. Computed tomogram reveals significant involvement of the tracheal lumen. C. Biopsy revealed a squamous carcinoma. A keratin pearl is seen (arrow) in the center of the microscopic section. |
Adenoid cystic carcinoma classically infiltrates the submucosa of the trachea for a distance greater than is grossly apparent. This pathologic feature accounts for an increased likelihood of a positive microscopic surgical margin at the time of tracheal resection (Fig. 78-14). The tumor grows through the tracheal cartilaginous rings and infiltrates the sheath of nerves adjacent to the trachea. It frequently pushes adjacent mediastinal structures aside rather than directly invading them. The adenoid cystic carcinoma less commonly spreads to regional lymph nodes but does metastasize to the lung or other distant organs. Local recurrence after resectional therapy can occur many years later, and these patients should be followed for the rest of their lives. Because of the relatively slow growth of these tumors, significant palliation can be achieved by resections that are associated with a positive margin. Postoperative radiation therapy is a determinant in achieving long-term control. Grillo and Mathisen (1990) reported that prognosis did not appear to depend on a positive resection margin or the presence of positive lymph nodes.
Fig. 78-13. A. Adenoid cystic carcinoma is seen on a linear tomogram (arrows). B. Six centimeters of trachea were resected for the adenoid cystic carcinoma seen in A. C. Adenoid cystic carcinoma. Microscopic examination reveals a cribriform pattern within nests of tumor cells separated by a hyalinized stroma. |
In a review of 38 patients with adenoid cystic carcinoma of the upper airway, Maziak and associates (1996) noted that lymphatic metastases were relatively uncommon, and subsequent hematogenous metastasis occurred in 17 of the 38 patients. Pulmonary metastasis occurred in 13 patients. Lin and associates (2002) carried out DNA flow cytometric analysis and oncogene expression in nine adenocystic carcinomas.
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DNA ploidy tended to correlate with the grade of the tumors, but HER2/neu, p53, and COX-2 were predominately negative and had no impact on prognosis.Fig. 78-14. A. Gross examination reveals submucosal extension of adenoid cystic carcinoma at the distal margin of resection. B. Microscopic examination reveals submucosal infiltration of the tumor at the margin of resection. |
Carcinoid Tumors
Carcinoid tumors are the third most common malignant tumors of the trachea. Briselli and colleagues (1978) published a review of the occurrence of this tumor in the trachea. Tracheal carcinoids may be of the typical or atypical histologic types. The typical carcinoids behave in a benign fashion; only minimal margins beyond the tumor are required at the time of resection. The atypical carcinoids are of greater malignant potential and may invade tissues beyond the trachea. Lymph node metastases may be present. In such cases, more aggressive resection is required.
Other Primary Malignant Tumors
Tracheal Adenocarcinoma
Tracheal adenocarcinoma accounts for approximately 10% of all primary tracheal malignancies, as reported by Mark (1983) (Fig. 78-15); this does not include adenocarcinomas arising from a main-stem bronchus that extend to the lower trachea or carina. Adenocarcinoma carries a poor long-term prognosis because of its propensity to spread directly into the mediastinum and to metastasize to regional lymph nodes. Therapy is primary resection, if technically feasible.
Small Cell Carcinoma
Small cell carcinoma of the trachea is rare compared with its more common bronchial presentation. Gelder and Hetzel (1993) reported a 6% incidence of small cell carcinoma in 321 patients. Careful histochemical analysis of this neuroendocrine tumor is required to be certain that it is not an atypical carcinoid. Therapy and prognosis are much different. Prognosis is extremely poor, and the tumor's natural history parallels that of small cell carcinoma of the lung. Primary treatment is chemotherapy and local radiation therapy.
Other Uncommon Malignant Tumors
Other malignant tumors include mucoepidermoid tumor, which was reported by Heitmiller and associates (1989),
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mixed tumor, and various mesenchymal tumors, including chondrosarcoma and fibrosarcoma. Thedinger and co-workers (1991) reported a patient with a tracheal leiomyosarcoma. Kaplan and associates (1992) described primary lymphoma of the trachea. Treatment of lymphoma depends on the stage of the disease, as well as histologic subtype. A localized lymphoma responds to primary radiation therapy. Plasma cell tumor of the trachea has been reported, and when the diagnosis is established, therapy is initial endoscopic removal and subsequent radiation therapy. The patient should be monitored closely for the late development of multiple myeloma.Fig. 78-15. Tracheal adenocarcinoma (arrows) involving the main-stem bronchus and carina. |
SECONDARY MALIGNANT TRACHEAL TUMORS
The trachea is subject to invasion by adjacent malignancies, including laryngeal, thyroid, lung, and esophageal cancer. Metastasis from distant primary tumors is also possible, including melanoma and tumors of the breast, kidney, and stomach.
Laryngeal Carcinoma
Extension of laryngeal carcinoma into the upper portion of the trachea is a common phenomenon; it is treated by surgical excision during the course of laryngectomy with end tracheostomy formation. Recurrence of the cancer at the stoma is rarely resectable for cure and is best treated with palliative radiation therapy or chemotherapy, or both. Sisson (1975) and Krespi (1985) and their colleagues used an aggressive surgical approach in the treatment of tracheal stomal recurrences. The morbidity and mortality rates, however, were high. Ujiki and associates (1987) recorded the major and frequent problems that accompany the associated reconstruction of gastrointestinal continuity, with transposition of the stomach and a pharyngogastric anastomosis in a previously irradiated field.
Thyroid Carcinoma
In a review of thyroid cancer invading the trachea, Zannini and Melloni (1996) noted that the incidence of this occurrence varied from 0.5% to 21%. The variation of incidence was attributed to the criteria used to define airway invasion and the number of cases of undifferentiated thyroid cancer included in the various series. The American Joint Commission on Cancer, as reported by Greene and colleagues (2002), recognizes invasion of the trachea as a T4 tumor. Ozaki and associates (1995) examined the spread of thyroid cancer into the tracheal wall and found that circumferential spread on the mucosal side of the trachea was greater than the extent of thyroid cancer on the adventitial side.
Thyroid cancer invading the airway is frequently asymptomatic because the tumor has not protruded into the tracheal lumen. Symptoms include wheezing, stridor, hemoptysis, and dyspnea on exertion. Recommended diagnostic evaluation includes high-resolution and spiral CT scans to define the upper airway and search for metastatic pulmonary disease, endoscopic examination of the larynx and trachea to assess vocal cord mobility and possible tracheal invasion, and assessment for bony metastatic disease. The most frequent well-differentiated thyroid carcinomas are the papillary and follicular types, and there is no difference in their tendency to invade the trachea or larynx. Undifferentiated and antiplastic tumors have a greater tendency to invade the trachea. Frequently, invasion of the trachea by thyroid cancer is an intraoperative finding. The decision on the extent of resection must be based on expected morbidity, extent of tracheal invasion, and ability to achieve airway reconstruction. The shave technique of resection has been advocated by McCarty and associates (1997). Nishida and associates (1997) also noted that well-differentiated thyroid carcinomas with limited tracheal invasion could be treated successfully by nonresectional management of the trachea. Postoperative adjuvant irradiation is recommended for these patients. Patients with deep invasion of the trachea or intraluminal extension require segmental tracheal resection.
Patients with locally recurrent thyroid cancer after a previous resection are usually symptomatic (Fig. 78-16). In a review by Grillo and associates (1992b), the most common presentation was that of a patient with previous thyroid surgery in whom the cancerous gland had to be shaved off the underlying trachea. This group of patients was often treated with radioactive iodine or external radiation therapy postoperatively. Recurrence at the site of prior resection is particularly troublesome, with recurrent airway bleeding and eventual suffocation from tumor progression. Therefore, as emphasized by Grillo and associates (1992b), palliation and, in some instances, cure can be achieved by segmental tracheal resection or by laryngotracheal resection and reconstruction at the site of recurrence.
Fig. 78-16. Recurrent thyroid carcinoma (arrow) invading the trachea. Clinical signs were hemoptysis and severe stridor. |
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Carcinoma of the Lung
Carcinoma of the lung involves the trachea and tracheal carina by proximal extension from tumors arising from a main-stem bronchus or extrinsic compression and invasion from disease in the paratracheal or subcarinal lymph nodes. Most, if not all, patients with local invasion from metastatic disease in mediastinal lymph nodes are not candidates for resection. Electrocautery or laser ablation and brachytherapy, however, may be palliative in the management of intraluminal obstructive growth. The role of neoadjuvant multimodality therapy followed by excision must be evaluated. Direct involvement of the tracheal carina by proximal extension of a main-stem bronchial tumor without mediastinal lymph node involvement may be considered for possible resection (tracheal sleeve pneumonectomy), especially if the postoperative mortality can be kept in the range of 10% (see Chapter 30).
Esophageal Cancer
Invasion of esophageal malignancy into the trachea often results in esophagorespiratory fistula. Burt and co-workers (1991) reviewed the extensive experience with this problem at Memorial Hospital in New York. The approach to this problem has varied, with many people advocating esophageal stents or gastrostomy for feeding purposes. Esophageal exclusion with gastric or colon bypass is a major operative procedure that is associated with a high operative mortality and, most often, only brief postoperative survival.
Other Malignancies
Metastasis to the trachea from distant sites is usually treated with endoscopic resection and Nd:YAG laser ablation to debulk the lesion. Systemic chemotherapy or radiation therapy is used as indicated.
PRINCIPLES AND RESULTS OF SURGICAL TREATMENT
History
Belsey (1950) reported experience with tracheal resection and listed 2 cm as the maximal length of trachea that could be circumferentially resected. Rob and Bateman (1949) reported resection of part of the tracheal wall with reconstruction by autologous or synthetic material. These procedures frequently were complicated by fistula, mediastinal infection, and stenosis. In subsequent years, some authors described successful resection of longer lengths of trachea. The door to extended and aggressive tracheal surgery was opened by Grillo and associates (1964) when they thoroughly and systematically described the amount of trachea that could be removed from thoracic and cervical mediastinal approaches. The landmark conclusion was that approximately one-half of the trachea could be removed and reapproximated. Many reports by Grillo (1983, 1989) and colleagues (1964, 1990, 1992a) on techniques and results of resection of tracheal tumors and stenosis paved the way for modern techniques of tracheal resection. Pearson and associates (1975) were among the first to describe resection techniques for high tracheal lesions that involved the cricoid. In 1995, Pearson and Gullane updated their experience with these techniques.
Prosthetic Replacement of the Trachea
A significant number of animal experiments have been carried out in an attempt to develop a prosthesis that could replace a long segment of the trachea. Tube grafts of stainless steel, plastic, and glass have all met with failure. This failure is related to granulation tissue that obstructs or results in strictures at the anastomosis and to migration of the prosthesis, leading to erosion of mediastinal vessels. Neville and associates (1990) reported 19 years of experience with the use of a silicone tube to reestablish airway continuity in cases of malignant and benign tracheal obstruction. In some patients, the silicone tube was used as a stent, and in others, resection of the trachea with primary anastomosis of the tube to the proximal and distal trachea was accomplished. Successful use of this prosthesis has not been duplicated in other centers, and its use is not recommended.
Selection of Surgical Procedure
Tracheal resection and end-to-end anastomosis using various release techniques to minimize tension is the primary form of therapy for tracheal neoplasms. The standard techniques are discussed in Chapter 76.
Benign tumors must be approached with a full knowledge of the pathology of the tumor to be treated. Benign tumors that are broad based and have a likelihood of local recurrence are best managed by tracheal resection. These tumors usually involve the trachea for a distance of 1 or 2 cm, and the resection and anastomosis is accomplished without difficulty. Benign tumors, such as lipoma, solitary papilloma, and hamartoma, can be removed through the rigid bronchoscope. The Nd:YAG laser has facilitated the total ablation of these tumors, and after endoscopic removal, the base of the tumor is ablated with the laser. Endoscopic surveillance is indicated to be certain that local recurrence does not occur.
Malignant primary tumors of the trachea should be treated by primary resection when the clinical findings indicate that the tumor most likely can be removed and tracheal
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reconstruction can be accomplished safely. Grillo and Mathisen (1990) reported 147 resections in 198 tracheal tumors seen from 1962 to 1989. These resections included laryngotracheal resection, staged reconstruction, and carinal resection. There were 82 pure tracheal resections; of these, 32 were for squamous carcinoma and 22 for adenoid cystic carcinoma. The operative mortality in the tracheal reconstructions was 1% (1 in 82).Perelman and associates (1996) reported 48 sleeve resections of the trachea with anastomosis for malignant tumors. Operative mortality was 4.2% (2 in 48 patients). A silicone prosthesis was used in five patients when the length of trachea resected was too long to permit an anastomosis. Mortality in this group was 40% (2 in 5 patients). Regnard and associates (1996) noted that postoperative mortality was three times greater for tracheal squamous cell or adenocarcinoma than for adenoid cystic carcinoma and other miscellaneous tumors. In their series, the best prognostic factor for tracheal cancers was the completeness of resection. Postoperative complications and mortality were not affected by the age of the patient, positive margins at resection, or lymph node involvement. Maziak and associates (1996) reported a mortality rate of 6.3% in 32 tracheal resections for adenoid cystic carcinoma. However, 4 of these patients had reconstruction with a Marlex mesh prosthesis. Rafaely and Weissberg (1997) reported 22 resections for tracheal tumors, with 19 segmental resections and 3 that included the carina. Mortality was 4.5% (1 in 22).
Squamous Cell Carcinoma
Grillo and associates (1992a) evaluated 70 patients with squamous cell carcinoma of the trachea. Fifty patients were deemed suitable for attempted resection. The tumor was excised in 44 patients, and sleeve resections of the trachea were done in 32, carinal resection in 9, and other types of procedures in 3. In 98 resected tracheal cancers reported by Regnard and associates (1996), the resection margin was positive in 26%. Grillo and Mathisen (1990) noted a 23% rate of positive resection margins in resected tracheal cancers. These findings confirm the necessity of performing frozen-section analysis on resected margins during the operative procedure as well as the difficulty of achieving a complete resection in patients with tracheal cancers. Because resection margins are frequently limited and preservation of residual tracheal blood supply negates the ability to carry out a wide en bloc dissection, Grillo and Mathisen (1990) and Regnard and co-workers (1996) recommend postoperative radiation therapy for all patients undergoing tracheal resection for squamous cell carcinoma.
Adenoid Cystic Carcinoma
Prognosis does not depend on a positive resection margin or the finding of positive lymph nodes. This factor is important to keep in mind at the time of the surgical resection because patients who have grossly negative resection margins but a positive frozen-section margin do not require more aggressive resection, with its attendant higher-risk anastomotic separation. Adenoid cystic carcinomas are radiosensitive and slow growing, so it is justified to accept a positive margin. Additional tracheal tissue should be resected if the microscopic margin is positive on frozen section and a satisfactory anastomosis can still be safely accomplished. The best long-term results are achieved in patients who have had a complete resection (Fig. 78-17). Maziak and associates (1996) reported excellent long-term results after both complete and incomplete resections for adenoid cystic carcinoma of the trachea. Grillo and Mathisen (1990) and Maziak and colleagues (1996) recommend postoperative irradiation for all patients with adenoid cystic carcinoma, whether or not the margins are positive.
Other Malignant Tumors
All other types of primary tracheal malignant tumors should be resected, if it is technically feasible. The decision regarding postoperative radiation therapy is based on the completeness of the surgical resection and the histologic type of tumor. Atypical carcinoid tumor with negative margins at resection is not a candidate for adjuvant radiation.
Prognosis
Squamous Cell Carcinoma
Grillo and Mathisen (1990) reported that 20 (49%) of 41 patients with squamous cell cancer were alive without evidence of tumor after resection. These results included patients who also had a carinal resection. Negative factors that affected long-term survival in patients with squamous cell carcinoma were positive nodes and microscopic tumor at the resection margin. Grillo and Mathisen (1990) state that resection combined with irradiation provides a tripled survival time for squamous cell carcinoma compared with radiation alone. Median survival of resected squamous cell carcinoma with postoperative irradiation was 34 months. Perelman and Koroleva (1987) reported a survival for resected tracheal squamous cell carcinoma of 27% at 3 years and 13% at 5 and 10 years. Pearson and co-workers (1984) identified four of nine patients with tracheal squamous cell carcinoma who were alive from 6 to 56 months after resection.
Regnard and associates (1996) reported 5- and 10-year survival rates of 47% and 36%, respectively, for resected tracheal cancers in their multicenter retrospective analysis. Their data revealed that postoperative radiation therapy did not improve survival in patients with completely resected tracheal cancers. Postoperative irradiation did significantly improve survival in a patient with an incomplete resection. An interesting finding in their study was that lymph node involvement in resected tracheal cancers did not affect
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long-term survival. However, 80% of these patients did receive postoperative radiation therapy when lymph nodes were positive.Fig. 78-17. A. Polypoid adenoid cystic carcinoma. Arrows depict distal resection margin. B. Positive margin at proximal resection site. A portion of the trachea was used for frozen-section analysis. Additional tracheal resection achieved a tumor-free margin. Central portion of polypoid tumor mass has the pattern of a mixed salivary gland tumor. C. The majority of tumor is the typical pattern of adenoid cystic carcinoma. D. Cells are arranged in ribbons and cords. |
Adenoid Cystic Carcinoma
Adenoid cystic carcinoma is a malignant tumor of the trachea that can recur many years after resection. Therefore, reporting accurate final results is somewhat difficult given the possibility of local recurrence at 10 or 15 years postoperatively. Despite an increased incidence of tumor at the resection margin, however, Grillo and Mathisen (1990) reported that 39 (75%) of 52 patients were alive and free of disease, with a median survival of 118 months. Perelman and Koroleva (1987) reported an overall survival for resected adenoid cystic carcinoma of the trachea of 71% at 3 years, 66% at 5 years, and 56% at 10 and 15 years. Maziak and associates (1996) reported 32 resections for adenoid cystic carcinoma, of which 16 were complete and potentially curative. There were two operative deaths in this group, and mean survival in the 14 remaining patients was 9.8 years. The median mean survival in 15 surviving patients undergoing an incomplete resection was 7.5 years. Calculated actuarial survival was 51% at 10 years in the 32 patients treated by primary resection. Metastatic tumor was found in regional lymph nodes in 5 patients. Regnard and associates (1996) reported 5- and 10-year survival rates of 73% and 57%, respectively, for resected adenoid cystic carcinomas of the trachea. There was not a significant difference in survival in patients with a complete resection compared with those with an incomplete resection. It also was noted that postoperative irradiation did not improve survival in either complete or incomplete resections.
Synchronous metastatic pulmonary disease decreases median survival time and also occurs late in the course of the disease. The long-term prognosis for patients with adenoid cystic carcinoma is obviously better than that for patients with squamous cell carcinoma. Late metastatic disease occurs most commonly in the lung but can also be found in the bones and liver, as described by Maziak and colleagues (1996).
RADIATION THERAPY
In patients with a primary malignant tumor of the trachea that does not meet the criteria for resection, radiation therapy can be an alternative form of treatment, although it does not appear to be as effective as primary resection for providing long-term control. Rostom and Morgan (1978) reported radiation therapy for 39 patients with primary malignant tumors of the trachea, of which 28 were squamous cell carcinomas and 3 were adenoid cystic carcinomas. In these 31 patients, 5 were reported to be free of disease at 4 to 11 years after treatment, 6 died of unrelated causes, and the remainder expired from local recurrence or metastatic cancer. Radiation dose varied from 5,000 to 7,000 cGy. Patients with disease limited to the trachea had a better prognosis, and 58% (11 of 19) with tumors confined to the trachea were controlled locally at the time of death or at the last follow-up evaluation (3 to 16 years).
Fields and colleagues (1989) reported on 24 patients with primary malignant tumors of the trachea who received radiation therapy as all or part of their treatment. This group included 13 patients with squamous cell carcinomas and 4 with adenoid cystic carcinomas. The median actuarial survival was 10 months, with 5-year and 10-year survival rates of 25% and 13%, respectively. For patients treated with radiation therapy alone, response was related to dose, and a dose of more than 6,000 cGy was statistically significant in achieving a complete response. Five patients developed serious complications, however, including innominate artery rupture, tracheoesophageal fistula, and esophageal stricture. In this series, it appeared that radiation therapy had no survival advantage over surgery for localized lesions and that primary tumor control was infrequent with the more advanced lesions. Survival was significantly better for patients with adenoid cystic carcinoma than for those with squamous carcinoma. Median survivals were 126 months and 6.5 months, respectively.
Grillo and Mathisen (1990) compared their patients who underwent resection and postoperative radiation therapy with those who underwent irradiation alone. Significantly better results were achieved with resection and postoperative irradiation. Maziak and associates (1996) reported six patients with adenoid cystic carcinoma of the trachea who had irradiation as primary therapy. Mean survival was 6.2 years, with two patients developing airway obstruction as a result of local recurrence 7 and 8 years after radiation. Grillo and Mathisen (1990) reported on 12 patients with adenoid cystic carcinoma treated with radiation alone. Only 3 patients were alive without evidence of disease. However, these tumors were extensive at the time of initial diagnosis.
Jeremic and co-workers (1996) treated 22 inoperable patients with squamous cell carcinoma of the trachea by radiotherapy alone. Nine patients were treated with 60 Gy and 13 with 70 Gy. Median survival time was 24 months, and the 5-year survival rate was 27%. Survival rates were slightly higher in the 70-Gy group than in the 60-Gy group, but the difference was not statistically significant. When compared with other series of lower dosages of radiation, these results emphasize the need for a minimal dose of 60 Gy to provide potential tumor control. Treatment-related toxicity does occur with higher dosages of radiation. These complications include dysphagia with esophagitis, cartilage softening, necrotizing tracheitis, and tracheal stricture. Jeremic and co-workers (1996) noted delayed toxicity in 5 of
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the 13 patients receiving 70 Gy. Patients with enlarged mediastinal nodes had a worse prognosis.Doses higher than 70 Gy with standard fractionation are not recommended. Gelder and Hetzel (1993) reported that high-dose radiotherapy was more effective than low-dose radiotherapy in squamous carcinoma and in upper lesions of the trachea. Patients with adenoid cystic carcinoma had no difference in survival between treatment with radiotherapy and surgery.
Treatment planning by CT and neutron therapy with modern techniques of deliverance and hyperfractionation may enhance the radiation treatment of primary tracheal malignancies. Chemoradiation has been successful in controlling clinically advanced lung cancer and can also be utilized to treat cancers of the trachea. At present, however, irradiation is to be considered only as a primary form of therapy when the tumor cannot be technically resected or the patient is medically unfit for surgery.
ENDOSCOPIC MANAGEMENT
Palliation of obstructing or bleeding tracheal malignancies can be achieved with current endoscopic procedures. Modern intravenous anesthetic techniques permit spontaneous ventilation during open tube bronchoscopy, which provides safety for the compromised airway. Dumon and colleagues (1982) described the use of a specially designed bronchoscope through which the Nd:YAG laser fiber is passed, along with d bridement forceps. Nd:YAG laser resection is particularly valuable in establishing an open airway in a patient with an obstructing tracheal neoplasm. The laser controls hemorrhage after tumor d bridement with biopsy forceps or the end of the open tube bronchoscope. Gelb and colleagues (1988) described 13 patients who underwent palliative Nd:YAG laser treatment for relief of malignant tracheal obstruction. Tracheal diameter was significantly increased after single or multiple laser treatments, with improvement of inspiratory flow rates. Symptomatic relief occurred for 4 to 48 months.
Venuta and associates (2002) reported 351 Nd:YAG laser resections in 273 patients; the trachea was involved in 36. In the entire group, 3 patients expired, for a 1% mortality. Demonstrated benefits of Nd:YAG laser resection were palliation of symptoms, safety, improved ventilation with clearance of distal infection, and patient stabilization prior to major resections. Morbidity is reduced during radiation or chemoradiation.
The Nd:YAG laser can penetrate deeply into tumor tissue, with resultant photocoagulation of superficial and deep blood vessels with thermal necrosis. Tumor shrinkage, reduction in blood supply, and vaporization occur. Careful use of this energy source is necessary to avoid bronchial or pulmonary artery rupture. It is practical to use the Nd:YAG laser for devascularization and tumor shrinkage, with subsequent debulking of the tumor through the rigid bronchoscope with biopsy forceps. Cavaliere and associates (1996) reported on 2,610 Nd:YAG laser resections in 1,838 patients with obstructing lung cancer. There were 10 deaths, and the median time to regrowth and stenosis was approximately 3 months.
Cryotherapy uses a probe passed through an open tube bronchoscope that freezes the tissue for the purpose of destroying it. Necrosis of the tumor and thrombosis of vessels do occur, but the basic architecture of the bronchus is maintained. A second open tube bronchoscopic procedure is necessary to remove sloughed necrotic material. Cryotherapy does not appear to have significant advantages over Nd:YAG laser resection or endoscopic d bridement.
Photodynamic therapy involves the use of a porphyrin-based photosensitizing agent (hematoporphyrin derivative) that, when exposed to a light of proper wavelength, forms toxic oxygen radicals and destroys the tumor. It has been used primarily for the treatment of patients with early-stage lung cancer limited to the mucosa or submucosal areas. However, it is also used for large obstructing cancers of the trachea and main-stem bronchi. Newer photosensitizing agents with fewer side effects are being developed, and photodynamic therapy may prove to be efficient in d briding obstructing neoplasms of the airway. Technique and results were described by Edell and Cortese (1995).
Brachytherapy is effective in providing high-dose radiation in a localized radiation field, as described by Spratling and Speiser (1996). The high-activity isotope iridium 192 (192I) is placed into a catheter that is appropriately positioned in the lumen of the tumor after endoscopic d bridement. Specific afterloading radiation machines are also available for this type of therapy. Hetzel and Smith (1991) reported that brachytherapy is complementary to laser resection, and effective palliation of the airway can be achieved. Fritz and associates (1991) described a new applicator to position the afterloading catheter centrally to minimize irregular dose distribution.
After endoscopic ablation of an intraluminal tumor, an endobronchial stent can be used to maintain an adequate airway, as suggested by Cooper and co-workers (1989). Tojo and associates (1996) reported on 25 patients with life-threatening tracheobronchial stenosis in whom expandable metal stents or silicone stents were placed. Tumor ingrowth occurred through the uncovered metal stents, and the authors' conclusion was that expandable metal stents are effective for external compression of the trachea or major bronchus, but covered metal stents or silicone stents are preferable for intraluminal tumors. Silicone stents can be positioned properly through the open tube bronchoscope. They are easily removed and prevent tumor ingrowth. However, recurrent cancer can grow over the end of the stent, necessitating tumor d bridement and repositioning of the stent. Silicone-covered metal stents can now be inserted through a small, commercially available catheter, with insertion adjacent to the fiberoptic bronchoscope. The uncovered stainless steel stents have been used for maintenance
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of a major airway, but placement and repositioning are particularly difficult, and the tumor can grow through the interstices of the stent. The stainless steel stent is difficult to remove once it has been expanded into position. Wasserman and co-workers (1996) reported that 10 patients with severe malignant obstruction of the trachea benefited significantly from placement of a silicone prosthesis. Median survival from stent insertion was 8 months, with stent replacement necessary in 5 patients (Fig. 78-18).Fig. 78-18. A. Obstructing squamous carcinoma of trachea. B. Silicone stent placement after neodymium:yttrium-aluminum-garnet laser resection of the tumor. |
MANAGEMENT OF SECONDARY TUMORS INVOLVING THE TRACHEA
Grillo and associates (1992b) evaluated 52 patients with thyroid cancer invading the upper airway between 1964 and 1991. There were 34 patients in whom resection could be accomplished: 27 patients had resection and reconstruction, and 7 underwent cervical exenteration. In the patients who had reconstruction, there were 16 papillary cancers, 5 follicular cancers, 4 tumors of mixed histology, and 2 poorly differentiated thyroid cancers. In the reconstruction group, 17 patients had undergone prior resection, and 9 patients were identified as having tracheal involvement before primary resection of the thyroid cancer. One patient had been treated only with iodine 131. Thirteen patients were referred because of recurrence, and it was specifically noted in this group of patients that the tumor had been shaved off the trachea at the time of the original resection. There were 10 tracheal sleeve resections, and the remainder had complex laryngotracheal resection and reconstruction. Mortality was 7.4% (2 of 27). Thirteen of 25 patients survived, and their long-term survival averaged 5 years and 9 months. Twelve patients were free of disease, and 1 patient had pulmonary metastasis. Eleven patients died of their cancer, with an average duration of survival of 3 years and 7 months. It is important to note that only 2 patients in this group had local recurrence, which attests to the value of radical resection for thyroid cancer invading the airway. Seven patients in the reconstruction group had metastatic disease, and the average survival for this entire group was 4.2 years, which illustrates the slow progression of pulmonary metastasis. This finding does not negate resection of recurrent thyroid cancer, if technically feasible. Grillo and associates (1992b) stressed that complete resection of local disease, including the trachea, provides the best long-term therapy. It is their opinion that the shave procedure followed by radiation therapy does not provide satisfactory long-term management. Successful long-term palliation can also be achieved when microscopic margins remain positive.
In contrast, McCarty and associates (1997) reported on 35 patients with superficial invasion of thyroid cancer who underwent the shave procedure followed by adjuvant radiation therapy. They reported that 25 patients were alive and free of recurrent disease at a mean follow-up of 81 months. Six patients developed local regional recurrence, and they underwent a second surgical procedure or repeat radiation therapy. All these patients remained free of disease at a mean follow-up of 5 years. Five patients in this series had intraluminal invasion of thyroid cancer, and all underwent laryngotracheal reconstruction. Four patients in this group were free of disease at a mean follow-up of 5 years.
Nishida and co-workers (1997) reported on 13 patients who had superficial invasion of the trachea by thyroid cancer in whom nonresectional management was carried out (the shave procedure). In these patients, the carcinoma was
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histologically present on the tracheal side of the resected thyroid gland, but gross disease was not left behind. At a mean follow-up of 7.2 years, 9 of 13 patients were alive without evidence of recurrence. Three patients died of their cancer, and there was one operative death. These results were compared with a group of 40 patients who underwent tracheal resection for a deep tracheal invasion: There were no statistical differences identified in local, regional, or distant recurrence. Nishida and colleagues concluded that superficial invasion of thyroid cancer into the trachea can be treated by nonresectional management. They also concluded that deep invasion was best treated by tracheal resection.Antiplastic thyroid cancer invading the trachea has to be considered as a separate issue because these patients frequently expire from metastatic disease soon after an extensive resection. Ishihara and colleagues (1991) described 60 patients with advanced thyroid cancer in whom the tumor was resected along with a portion of the trachea. A complete resection was achieved in 34 patients, and 5-year actuarial survival in this group was 78%. In the patients undergoing incomplete resection, the 5-year actuarial survival was 44%.
Resection of a portion of the trachea or larynx, or both, in patients with invading thyroid carcinoma can offer significant palliation from bleeding and obstruction and, possibly, cure. In a rare instance, tracheal involvement is the only extramural spread of the esophageal malignancy, and partial tracheal resection with musculocutaneous flap reconstruction of the trachea has been accomplished. This operation, as reported by Sodeyama and associates (1990), is often done in association with total laryngoesophagectomy, in which the reconstruction is part of a cervical or mediastinal tracheostomy construction.
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