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without airway obstruction.

In 2 patients, the malformation extended from the thoracic inlet to the diaphragm and was fed by multiple arterial branches. These included thyrocervical, internal thoracic, right coronary, a branch of right subclavian, and bronchial arteries. The huge resulting shunt enlarged the azygos vein and the superior and inferior vena cava. The malformation protruded through the wall of the trachea in both, visible broncho-scopically as a plexus of pulsating vessels. One patient had undergone right upper and middle lobectomies in childhood for hemangiomatous lesions not further defined in prior records, and the other patient had had cutaneous vascular lesions removed in childhood. Both were 25 years of age when seen. In one patient, who had severe obstructive symptoms, management was commenced with an extensive program of embolic obliteration of multiple feeding vessels from the neck, mediastinum, and right coronary artery. After figure 7-11 Chondroblastoma in a 22-year-old medical student, treated for years for "asthma" Retrospectively, a tracheal tumor was identified on x-rays taken 7 years earlier. The impression of the endotracheal tube on the soft intraluminal tumor is seen.

figure 7-12 Arteriovenous vascular malformations (AVM) of mediastinum involving trachea. Angiograms show widespread involvement of the mediastinum and neck. A, A 25-year-old man with protrusion of AVM into the lower trachea (arrow) producing respiratory obstruction. Managed by multiple, repeated embolization of feeding arteries followed by successful tracheal segmental resection. His airway remained clear 19 years later. See text. B, Lateral view of the patient in A. The arrow indicates tracheal invasion. C, A 25-year-old woman with more diffuse tracheal compression by very extensive AVM. Intratracheal protrusion of vessels was also present. See text.

reducing flow in this way, meticulous dissection accomplished resection of the involved portion of the trachea. The basic malformation was not resectable in its entirety. Seven years later, multiple coronary arterial venous fistulae were ligated at another hospital. Since then, the patient has continued to do well. The second patient was not initially sufficiently symptomatic to justify such an extensive and somewhat perilous approach. Years later, she presented at another hospital where embolization failed and surgery was unsuccessful. She died there from intratracheal hemorrhage adjacent to a tracheostomy.

Glomus tumor, found most commonly in skin and subcutaneous tissue and especially in fingers under the nails, occurs in the trachea only rarely. It arises from cells related to the glomus bodies, which have smooth muscle characteristics.43 Symptoms in trachea are cough, dyspnea, and hemoptysis. The tumors are benign and treated by limited but complete resection in most cases. Fourteen tracheal cases had been collected by the year 2000.44 The tumors may be very vascular. Initial examination may suggest carci-noid or hemangiopericytoma. Immunohistology clarifies the differential diagnosis. We have also encountered a very diffuse form, which contiguously involved most of the trachea with extension into right and left main bronchi (Figure 7-13). The patient showed early response to external irradiation and brachyther-apy, but required a stent to maintain a satisfactory lumen. Long-term palliation cannot yet be assessed, although he continues to do well 3i/> years after treatment.

Granular cell tumor, formerly termed granular cell myoblastoma, is predominantly believed to arise from Schwann cells, and is of muscle origin (Figure 7-14). The tumors arise most commonly in the tongue and head and neck area. A small number occur in the airways, and most of these are in the larynx or bronchi. Burton and colleagues collected 30 tracheal instances in 1992.45 Age of occurrence was from 6 to 56 years and the tumor was more often found in women. Many patients are black.46 Granular cell tumors can be multiple in the airway or elsewhere, and malignant manifestation is only very rarely described. Tracheal lesions appear most often intraluminally, but can be located or extend extraluminally. Tumor cells are present deep in the tracheal wall in all the larger tumors.47 Endoscopic resection of the smaller tumors has produced some long-lasting clearances. However, the frequency of recurrence (over half), lack of knowledge of the tumor depth in unresected patients, the indolent course of tumor, late recurrences, and the need figure 7-13 Diffuse glomus tumor. Broncho-scopic view. Widespread nodular disease is evident. Treated by irradiation because of extent. See text. Also, see Figure 19 (Color Plate 14).

figure 7-14 Granular cell tumor of the upper trachea, posteriorly based and extending over the cricoid cartilage. An arc of posterior laryngeal mucosa and submucosa is visible at the top of the photograph. Only a slit of airway remains anterior to the tumor. The patient, a 14-year-old girl, was critically obstructed.

figure 7-14 Granular cell tumor of the upper trachea, posteriorly based and extending over the cricoid cartilage. An arc of posterior laryngeal mucosa and submucosa is visible at the top of the photograph. Only a slit of airway remains anterior to the tumor. The patient, a 14-year-old girl, was critically obstructed.

for very long-term bronchoscopic follow-up after endoscopic removal, favor primary limited but complete sleeve resection of the trachea or bronchus. Patients often present with "asthma," that is, shortness of breath and wheezing, rather than hemoptysis. One 14-year-old girl with critical obstruction urgently required laryngotracheal resection and reconstruction (see Figure 7-14). Follow-up bronchoscopy revealed a second granular cell tumor in the bronchus intermedius, which was resected without loss of lung tissue. Multiple granular cell tumors elsewhere have also been reported. The question of irradiation hardly arises with this particular pattern of behavior, and when irradiation has been given, its effect has been uncertain.

A lesion that occurs infrequently in the lung and sometimes in the gastrointestinal tract is variously termed postinflammatory tumor, pseudotumor, plasma cell granuloma, xanthoma, fibrous histiocytoma, and by composite names formed from these terms (xanthomatous pseudotumor, plasma cell tumor, fibroxanthoma, xanthogranuloma). Histologically, the lesions show proliferative mature plasma cells, reticuloendothelial cells, granulation tissue stroma with proliferative fibroblasts, and contain lymphocytes and fat-laden mononuclear cells. What is still not clear is whether these lesions are true tumors or in fact inflammatory in nature (Figure 7-15). Tumors which might be considered inflammatory on bronchoscopic biopsy may prove to be neoplastic and invasive on final examination.48 Depth of invasion into and through the cartilage varies. Matsubara and colleagues have subdivided these lesions in the lung into three groups.49 They question the relationship of these lesions, which they consider to be of inflammatory origin, with malignant fibrous histiocytoma. In the airway, the tumor causes cough, dyspnea, wheeze, hemoptysis, and pulmonary changes due to obstruction.50,51 Tumors occur at any age but more often in children and young adults. Intraluminal tumor may be polypoid or sessile.

Criteria for benignity or malignancy do not seem to be well established.52 The lesions can extend into the mediastinum and the thyroid gland, giving rise to diagnosis of malignant fibrous histiocytoma.53,54 This term is also used to describe high-grade sarcoma, adding to confusion. Laryngeal tumors may more often appear malignant. On the other hand, complete excision of tracheal histiocytoma usually results in figure 7-15 Inflammatory pseudotumor of the trachea in a 39-year-old woman, extending into the posterior subglottic larynx. A, Endoscopic view. The cricoid is visible anteriorly, tracheal rings distally, and proximal extension of lesion posteriorly. B, Magnetic resonance scan and reconstructions showing localization of the lesion. From left to right: 1) subglottic and linear extent, anterior view; 2) lateral view showing the posterior mass high in the airway; 3) cross section of airway partly obstructed by bulky mass, with thyroid lobes on either side. Removal of tumor required resection of 4 cm of trachea with posterior laryngeal mucosa and submucosa, and sacrifice of invaded right recurrent laryngeal nerve. Temporary tracheostomy was done. There has been no recurrence.

cure.47,48,53-55 Incomplete excision is likely to result in local recurrence. Increasing the etiologic mystery are reports of resolution of pulmonary lesions following treatment with cortisone or spontaneously.56,57 This last raises a question about the suggested beneficial effect of irradiation or of cortisone.58

In 2 patients with very large pseudotumors of the trachea, excision produced relief without recurrence. Extension of the lesion proximally over the posterior cricoid plate submucosally required excision of the lesion anterior to the cartilage and reconstruction with a tongue of distal tracheal membranous wall (see Figure 7-15). Sleeve resection of an obstructed left main bronchus for another lesion allowed salvage and gradual effective recovery of the chronically inflamed lung. A measured decision must always be made on whether a long obstructed lung can be usefully salvaged.

Primary neurogenic tracheal tumors are also uncommon. Horowitz and colleagues found reports of 3 neurofibromas (not with von Recklinghausen's disease) and 12 neurilemomas (Schwannoma), to which they added another.59 Most were in the lower trachea and produced cough, symptoms of tracheal obstruction, or hemoptysis. Segmental resection was curative. Endoscopic resection may well result in long-delayed recur-

rence.59 Such a recurrent tumor was dumbbell in form and necessitated carinal resection and reconstruction. Stack and Steckler added an upper tracheal case in 1990, the nineteenth reported patient.60 All were benign. A patient with recurrent plexiform neurofibroma of the lower trachea, following previous local enucleation elsewhere, attained cure (24-year follow-up) by extended resection of the trachea, including a portion of esophageal wall and an isolated area of metastatic seeding in the prior thoracotomy wound (Figure 7-16). Local enucleation of tumors from the tracheal wall is generally not an appropriate method of management.

Two patients with tracheal paraganglioma, which involved the posterior wall of the trachea intimately adjacent to the cricoid cartilage, were treated by segmental excision of the upper trachea, removal of a portion of the posterior cricoid plate, and in one, a portion of the muscular wall of the esophagus. There was no local recurrence. One, however, affected with familial incidence of multiple tumors, later presented with bilateral carotid body tumors. His trachea remained clear. This tumor is not common and was first reported in the trachea in 1956.61

Other mesenchymal tumors occur in the trachea. These include leiomyoma, lipoma, and fibroma (Figure 7-17).62-64 Eleven tracheal leiomyomas have been reported, with calcification in one, just as occasionally occurs in esophageal leiomyomas.63 The majority of hamartomatas are found in pulmonary parenchyma, with less than a quarter presenting endobronchially and rarely in the trachea.65,66 Since these lesions rarely produce hemoptysis, the patients often present with a high degree of obstruction after prolonged treatment for "asthma." In a rare case where a benign connective tissue tumor is based on a narrow pedicle, endoscopic removal alone may produce cure. Such patients, however, should be observed bron-choscopically for years for recurrence. Where there is any degree of involvement of tracheal wall, segmental tracheal resection is advisable. Resection is definitive, and given the limited resection required, carries little morbidity in experienced hands.

figure 7-16 Plexiform neurofibroma of the lower trachea, in an 11-year-old boy. Resected by enucleation 6'A years previously. There was also an incisional recurrence. The patient is tumor-free 24 years after sleeve resection. A, Resected 4.5 cm specimen with surrounding tissue. Lobule of tumor protrudes from the cut end of the trachea. B, Opened specimen showing the extent of recurrent tumor.

figure 7-16 Plexiform neurofibroma of the lower trachea, in an 11-year-old boy. Resected by enucleation 6'A years previously. There was also an incisional recurrence. The patient is tumor-free 24 years after sleeve resection. A, Resected 4.5 cm specimen with surrounding tissue. Lobule of tumor protrudes from the cut end of the trachea. B, Opened specimen showing the extent of recurrent tumor.

Extramedullary solitary plasmacytomas occur most often in the head and neck. In 1995, Logan and colleagues reported the eleventh such tracheal patient.67 It is not known how likely these patients are to develop multiple myeloma. Resection and subsequent irradiation are advised for the apparently solitary tracheal lesion.

Non-Hodgkin's lymphoma (NHL) is rarely confined primarily to the trachea or bronchi. In addition to lymphocytic lymphoma, mucosa associated lymphoid tissue, and anaplastic histologic subtypes of NHL, lymphoplasmacytoid lymphoma has been found in the trachea.68 Symptoms of tracheal lymphoma are cough and those of airway obstruction, frequently misdiagnosed as asthma. When other anatomic sites are not involved, the disease is often of lower grade histology. Fidias and colleagues identified 5 patients with primary tracheal disease, variously treated with chemotherapy, resection, and irradiation.69 An additional 36 were found to have secondary tracheal involvement. The tumors may arise from mucosal lymphoid tissue, forming "lymphoepithelial" lesions. Since the cases are few, treatment regimens have varied widely. Long-term freedom from disease (12 to 64 months) resulted in 4 of 5 patients followed. One patient required tracheal resection for malacia following complete response to chemotherapy. Irradiation was then added. Maeda and colleagues reported survival over 5 years after tracheal resection only for primary tracheal malignant lymphoma.70 Although experience is sparse, appropriate treatment for stage I extranodal tracheal lymphoma would seem to be chemotherapy, surgery where feasible, and irradiation. Resection may not prove to be necessary in all cases, but when needed, it is best done prior to irradiation. Recurrence may follow after a long interval. Thus, one of my patients, who had tracheal resection with laryngotracheoplas-

ty for diffuse mixed cell and large cell malignant lymphoma, followed by irradiation, suffered recurrence retroperitoneal^ 11 years later, without airway disease.

Hodgkin's lymphoma in mediastinal lymph nodes has affected the trachea in late stages from recurrence with perforation (tracheoesophageal or tracheomediastinal fistula) or from fibrous stenosis years after chemotherapeutic and irradiation treatments.71,72 In the latter case, surgical resection seems contraindicated (see Chapter 42, "The Omentum in Airway Surgery and Tracheal Reconstruction after Irradiation").

Fortunately, sarcomas are uncommon. This group includes leiomyosarcoma, fibrosarcoma, and rhabdomyosarcoma.73-75 All are uncommon in the trachea and bronchi. In 1999, Vinod and colleagues discovered in the literature 8 cases of leiomyosarcoma, 6 of malignant fibrous histiocytoma, and 1 each of rhabdomyosarcoma, fibrosarcoma, liposarcoma, and non-human immunodeficiency virus (HIV) Kaposi's sarcoma, all primary in the trachea.54 Of these 18 cases followed for varied time periods, only 3 died of disease and 1 of other causes. The behavior of sarcomas is variable (see Figures 7-17B, 7-18). Although in our series a low-grade spindle cell sarcoma and a fibrosarcoma did not recur after resection, other sarcomas have been of high malignancy, and, in several cases, have been too extensive for resection when first identified. Irradiation and chemotherapy should probably be considered after surgical extirpation, but data are few for each cell type. In the case of a highly malignant sarcoma, postoperative irradiation has not appeared to delay recurrence. Pulmonary metastases seemed to occur early. Obstructing Kaposi's sarcoma was also identified exclusively in the subglottic region and trachea of an HIV-positive patient, and treated by laser and irradiation.76 Endobronchial lesions are often present in acquired immunodeficiency syndrome (AIDS) patients with hilar adenopathy and perihilar infiltration, who show Kaposi's sarcoma of the skin and mucous membranes.77,78 Obstruction may occur. Prognosis is very poor.

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