DOI:10.2214/AJR.04.1893
AJR 2006; 186:1304-1313
© American Roentgen Ray Society
Benign Tumors of the Tracheobronchial Tree: CT-Pathologic Correlation
Jeong Min Ko1,
Jung Im Jung1,
Seog Hee Park1,
Kyo Young Lee2,
Myung Hee Chung1,
Myeong Im Ahn1,
Ki Jun Kim1,
Yo Won Choi3 and
Seong Tai Hahn1
1 Department of Radiology, College of Medicine, The Catholic University of
Korea, 62 Yeouido-dong, Youngdungpo-gu, Seoul 150-713, Korea.
2 Department of Pathology, Kangnam St. Mary's Hospital, College of Medicine, The
Catholic University of Korea, Seoul, Korea.
3 Department of Radiology, Hanyang University Hospital, Seoul, Korea.
Received December 13, 2004;
accepted after revision March 3, 2005.
Address correspondence to J. I. Jung
(jijung{at}catholic.ac.kr).
Abstract
OBJECTIVE. The purpose of this essay is to illustrate the CT
findings of variable benign tumors of the tracheobronchial tree and to
correlate the CT and pathologic findings in 17 patients.
CONCLUSION. The tracheal tumors were eccentric, well-defined,
polypoid masses in all cases. The endobronchial tumors were masses confined
within the bronchus in all cases, and atelectasis or pneumonia of the distal
parenchyma was frequently associated. Of the six hamartomas, one was a fatty
mass, and two were nodules with calcification. The others were
soft-tissue-density nodules. The lipomas manifested as fat density on CT scans
in both cases. The other benign tumors were low-attenuating,
soft-tissue-density masses without characteristic findings on CT scans.
Keywords: airway • chest • CT • lung
Introduction
Most tumors of the tracheobronchial tree are malignant. Benign tumors are
quite rare (ca. 1.9% of all lung tumors). Unlike malignant tumors, many benign
neoplasms are slow growing and present with symptoms related to bronchial
obstruction [1]. Benign lesions
of the trachea often go unrecognized for months or even years. As a result,
patients with such tumors often undergo prolonged treatment for obstructive
lung disease or asthma.
It is often difficult to identify benign tracheal lesions on routine chest
radiographs. The clinical and radiographic features of these tumors are often
indistinguishable from those of malignant tumors. Many of these lesions have
similar radiographic features. These radiographic findings are often
nonspecific and include atelectasis, pneumonia, bronchiectasis, and
mediastinal shifts [1]. Early
recognition and diagnosis of these benign lesions may allow conservative
treatment and excellent patient outcome.
The CT findings of benign tumors of the tracheobronchial tree are rarely
reported. Only a few case reports of the CT findings of benign tumors of the
tracheobronchial tree have appeared in the literature
[2,
3]. In those cases, CT revealed
the tumor and showed its size and extent, findings helpful for surgical
planning. More important, CT findings excluded contiguous mediastinal and
parenchymal lung involvement
[3]. In most of the reported
cases CT showed masses confined within the tracheobronchial lumen without
evidence of adjacent invasion. CT findings also aid in definitive diagnosis of
fatty tumor within the tracheobronchial lumen because CT is highly specific
and sensitive in the detection of fat
[2].
We retrospectively reviewed the CT scans of 17 patients with pathologically
proven benign tracheobronchial tumors. The nine male and eight female patients
were 16-76 years old. The chief complaints were chronic cough and sputum
(n = 10), progressive dyspnea or asthma attack (n = 3),
hemoptysis (n = 3), and fever (n = 1). One patient had no
specific complaint. The tumors were classified according to the anatomic
location of the lesions in the trachea (n = 5), bronchus intermedius
(n = 3), lobar bronchus (n = 6), and segmental bronchus
(n = 3). Pathologic diagnoses were hamartoma (n = 6),
leiomyoma (n = 3), lipoma (n = 2), schwannoma (n =
2), inflammatory polyp (n = 1), amyloidoma (n = 1),
papilloma (n = 1), and pleomorphic adenoma (n = 1). We
present the CT findings of benign tracheobronchial tumors correlated with the
histopathologic findings.
Hamartoma
Hamartoma is defined as a mass resulting from the abnormal growth and
mixture of tissue elements or an abnormal proportion of a single element of
tissue normally present in an organ
[1]. Hamartoma in the lung is
the most common benign tumor. Arrigoni et al.
[4] reviewed 130 benign tumors,
of which 77% were hamartomas. Only 3% of the hamartomas were endobronchial. In
a large series of benign endobronchial tumors, hamartoma was the most common
type (70%) [1]. Endobronchial
hamartoma is a special form of the usual intrapulmonary hamartoma. It
originates from a large bronchus, grows into the lumen, and obstructs the
bronchi before becoming large
[2].
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Fig. 1A —75-year-old man with endobronchial hamartoma with fat attenuation in
bronchus intermedius. Patient had had recurrent pneumonia for several years.
CT scan at level of right bronchus intermedius obtained after administration
of contrast material shows horseshoe-shaped, fat-attenuated endobronchial
lesion in right bronchus intermedius.
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Fig. 1B —75-year-old man with endobronchial hamartoma with fat attenuation in
bronchus intermedius. Patient had had recurrent pneumonia for several years.
Photomicrograph of specimen shows bland cartilage, columnar epithelium, and
interposed fat (arrows) consistent with hamartoma. (H and E,
x100)
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Fig. 2 —Endobronchial hamartoma as mass containing calcifications in
bronchus intermedius of patient with situs inversus totalis. Contrast-enhanced
CT scan shows calcified endobronchial mass (arrow) with fibrous cap
in bronchus intermedius.
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Intrapulmonary and endobronchial hamartomas contain cartilage, fat, fibrous
tissue, and an epithelial component. It is reported, however, that
endobronchial lesions tend to have relatively more fat than do parenchymal
lesions. This finding may be attributable to the relative abundance of fat in
the bronchial walls as opposed to the lung parenchyma. Unlike the more common
parenchymal hamartoma, which tends to present as an asymptomatic solitary
lesion, endobronchial hamartoma is often symptomatic because of airway
obstruction, which can cause cough, hemoptysis, dyspnea, and obstructive
pneumonia [1].
Chest radiography often shows the sequelae of endobronchial obstruction.
These include atelectasis, postobstructive pneumonia, and bronchiectasis.
Endobronchial mass lesions may be poorly depicted or not depicted at all on
chest radiographs [1].
CT scans reflect the various tissue components of hamartoma. Ahn et al.
[2] reported the CT findings of
endobronchial hamartoma in three patients. One lesion was a mass of fat
attenuation, and the pathologic examination showed the lesion was composed
mainly of fatty tissue (Figs.
1A and
1B). Another lesion exhibited
soft-tissue attenuation and contained a higher-attenuation core composed of
connective tissue stroma, islands of cartilage, and occasional areas of
calcification and ossification (Fig.
2). In the third patient, the mass attenuation was similar to that
of muscle. The lesion was composed of connective tissue stroma containing fat,
lymphoid tissue, and smooth muscle in various proportions (Figs.
3A,
3B, and
3C). Ahn et al.
[2] concluded that the CT
finding of endobronchial hamartoma is an endobronchial mass with obstructive
pneumonia. However, in the case of some masses containing fat or
calcification, a specific diagnosis of hamartoma can be made.
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Fig. 3A —62-year-old man with endobronchial hamartoma that manifested as
soft-tissue-attenuating mass. The patient had complained of cough for 6
months. Contrast-enhanced CT scan shows low-attenuation (47 H) endobronchial
mass (white arrow) obstructing right upper lobe bronchus.
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Fig. 3B —62-year-old man with endobronchial hamartoma that manifested as
soft-tissue-attenuating mass. The patient had complained of cough for 6
months. Three-dimensional shaded-surface display reconstruction image shows
bronchial obstruction.
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Fig. 3C —62-year-old man with endobronchial hamartoma that manifested as
soft-tissue-attenuating mass. The patient had complained of cough for 6
months. Excised mass is round and lobulated with firm consistency. Pathologic
diagnosis was hamartoma.
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Leiomyoma
Primary pulmonary leiomyoma is extremely uncommon, constituting
approximately 2% of benign lung tumors. Approximately 45% of pulmonary
leiomyomas are endobronchial; the other lesions occur in the lung parenchyma
and trachea [1].
The histologic findings of leiomyoma are composed of long interlacing
fascicles of spindle cells with abundant elongated eosinophilic cytoplasm.
Hypocellularity is often associated with stromal hyalinization and a marked
decrease of the vascular framework. The nuclei are oval and indented with
delicate chromatin and small inconspicuous nucleoli and are devoid of atypical
mitoses. Leiomyoma can be difficult to differentiate from fibroma,
neurofibroma, and schwannoma at light microscopy alone. However,
immunoperoxidase staining for vimentin, actin, and S-100 protein has positive
results in leiomyoma [1].
Tracheal leiomyoma arises from the smooth muscle in the tracheal wall,
typically along the membranous portion of the lower third of the trachea
because of the abundant smooth-muscle fibers in this area
[3]. Most patients with
tracheal leiomyoma are 15-72 years old, and there is no sex predominance. Even
though it is benign, tracheal leiomyoma can be lethal because of tracheal
obstruction [3].
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Fig. 5A —57-year-old woman with endobronchial leiomyoma. Contrast-enhanced CT
scan shows low-attenuation (42 H) endobronchial mass (arrow)
obstructing apical segmental bronchus of right upper lobe.
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Fig. 5C —57-year-old woman with endobronchial leiomyoma. Photomicrograph
shows spindle-shaped cells with oval nuclei arranged in parallel bundles and
whorls typical of leiomyoma. (H and E, x400)
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CT findings of tracheal leiomyoma are a smooth, intraluminal soft-tissue
mass limited to the tracheal wall with occasional areas of cystic degeneration
due to poor vascularization (Fig.
4) [3]. Like those
of other benign tracheal masses, the CT characteristics of leiomyoma are not
specific, but CT is helpful in determining the extent of tracheal involvement
and involvement of adjacent structures
[3]. Diagnosis requires biopsy,
which must be performed with caution because death due to tracheal obstruction
or bleeding during endoscopic resection has been reported. Thoracotomy with
wide tracheal resection may be needed for complete excision of the typically
broad base of this neoplasm. Incomplete excision often results in recurrence
[3]. CT findings of
endobronchial leiomyoma have not yet been reported in the literature. Our
patient had a soft-tissue-density nodule that was confined to the segmental
bronchus and occluded the lumen (Figs.
5A,
5B,
5C, and
5D).
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Fig. 8A —34-year-old-woman with endobronchial schwannoma in bronchus
intermedius. The patient had chronic cough, and sputum was present.
Contrast-enhanced CT scan shows low-attenuation (45 H) endobronchial mass
(arrow) obstructing bronchus intermedius. Atelectasis of right middle
and lower lobes is evident.
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Fig. 8B —34-year-old-woman with endobronchial schwannoma in bronchus
intermedius. The patient had chronic cough, and sputum was present. Cut
surface of resected lung specimen shows yellowish white endobronchial mass
(black arrow) obstructing bronchus intermedius. Distal bronchi are
dilated and filled with mucous plugs (white arrows).
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Fig. 8C —34-year-old-woman with endobronchial schwannoma in bronchus
intermedius. The patient had chronic cough, and sputum was present. Scanning
microscopic view shows mass (M) with intact overlying respiratory
epithelium.
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Fig. 8D —34-year-old-woman with endobronchial schwannoma in bronchus
intermedius. The patient had chronic cough, and sputum was present. High-power
view shows fascicles of wavy nuclei with tapered ends and palisading
consistent with schwannoma. (H and E x400)
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Lipoma
Tumors composed exclusively or almost exclusively of mature fat rarely
occur within the lungs (0.1% of benign lung tumors)
[1]. Intrathoracic lipomas are
classified into five groups: endotracheobronchial, parenchymal, pleural,
mediastinal, and cardiac. Most such neoplasms are endobronchial (Figs.
6A,
6B, and
6C). Tracheal lipoma is rare.
In a review of reports, only three of 50 endobronchial lipomas were located in
the trachea (Figs. 7A and
7B)
[5].
Tracheobronchial lipoma arises from the submucosal fat of the
tracheobronchial tree and usually is pedunculated with a narrow stalk. The
tumor may extend between the cartilaginous rings into the peritracheal tissues
and may recur after endoscopic resection.
Patients with tracheobronchial lipoma are generally in late middle age, and
there is a definite male preponderance (ca. 90%). Lipoma, like other
endobronchial tumors, produces symptoms and signs of obstruction, including
productive cough, hemoptysis, wheezing, recurrent pneumonia, and
bronchiectasis [1].
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Fig. 9A —65-year-old woman with endobronchial inflammatory polyp. The patient
had had chronic cough. Contrast-enhanced CT scan shows low-attenuating
endobronchial mass (arrow) obstructing right upper lobe bronchus.
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Fig. 9B —65-year-old woman with endobronchial inflammatory polyp. The patient
had had chronic cough. Scanning microscopic view of excised polyp shows
fibrovascular core lined by respiratory-type epithelium consistent with
inflammatory polyp. (H and E, x1)
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Fig. 10A —50-year-old man with tracheobronchial amyloidosis and endobronchial
amyloidoma. Contrast-enhanced CT scan shows nodular thickening of trachea and
ovoid soft-tissue-density mass obstructing bronchus of right upper lobe.
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Fig. 10B —50-year-old man with tracheobronchial amyloidosis and endobronchial
amyloidoma. Shaded-surface display 3D image shows multiple nodular
indentations in tracheobronchial tree and occlusion of right upper lobe
bronchus.
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The chest radiographic features of lipoma are typically related to
obstruction [1]. Bronchoscopy
often does not provide enough information for a diagnosis because of the
fibrous capsule surrounding the lesion. Bronchoscopic findings can be
misleading because the simultaneous detection of atypical cells associated
with chronic irritation and infection leads to an incorrect diagnosis of
bronchogenic cancer.
CT is highly specific and sensitive in the detection of fat, and CT
findings can lead to a definitive diagnosis of tracheobronchial lipoma (Figs.
6A,
6B,
6C,
7A, and
7B). However, endobronchial
hamartoma also can appear as a fatty mass on CT
[1]. Therefore, whenever a
fatty endobronchial mass is identified on CT, the differential diagnosis
should include lipoma and hamartoma. Whenever possible, the treatment of
patients with tracheobronchial lipoma is laser resection by means of
bronchoscopy.
Neurogenic Tumor
Intrathoracic neurogenic tumors, schwannoma and neurofibroma, occur most
often in the posterior mediastinum and only rarely in the lungs. In a large
series of pulmonary neoplasms, neurogenic tumor constituted 0.2% of the
tumors. According to results of most studies, neurofibroma is more common than
schwannoma by a ratio of 3:1. Most neurogenic tumors are intraparenchymal and
manifest as asymptomatic masses; however, 25% of tumors are endobronchial and
often cause obstructive symptoms. Most tumors are solitary and not associated
with Reckling-hausen's disease. The clinical course depends on tumor size and
the degree of bronchial obstruction
[1].
The histologic findings of both schwannoma and neurofibroma are similar to
those of their extrapulmonary counterparts. Schwannoma is encapsulated and has
two patterns: Antoni type A (cellular pattern composed of compactly arranged
spindle cells with elongated, wavy nuclei disposed in a parallel palisade
arrangement) and Antoni type B (fewer cellular zones with elongated cells
arranged in a haphazard pattern and separated by a poorly staining matrix).
Neurofibroma is composed of haphazardly arranged spindle cells with wavy
nuclei, eosinophilic cytoplasm, and variably collagenous and myxoid matrix.
Schwannoma exhibits ultrastructural evidence of Schwann cell derivation,
including prominent interdigitating cell processes, basal lamina, and Luse
bodies. Both schwannoma and neurofibroma have positive results for S-100
protein [1].
Chest radiography shows that neurogenic tumor is typically related to
endobronchial obstruction. A few surgical reports of endobronchial neurogenic
tumors describe the tumors as round, ovoid, or lobulated and as sharply
demarcated, homogeneous masses on CT (Figs.
8A,
8B,
8C, and
8D)
[6]. Treatment is usually
resection, either endoscopic or by thoracotomy. The prognosis is excellent in
the absence of neurofibromatosis.
Inflammatory Polyp (Fibroepithelial Polyp)
Inflammatory polyp typically occurs in the large airways. Many of these
lesions are thought to be related to some form of underlying irritation or
inflammatory process, including foreign body, inhalation of hot or corrosive
gas, and, rarely, bronchiolitis. Removal of the causative factor often leads
to regression of the polyp.
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Fig. 12A —68-year-old woman with endobronchial pleomorphic adenoma. Patient
had had recurrent pneumonia and chronic cough for 4 years. CT scan shows
low-attenuating endobronchial mass (arrow) and distal pneumonia.
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Fig. 12B —68-year-old woman with endobronchial pleomorphic adenoma. Patient
had had recurrent pneumonia and chronic cough for 4 years. Endobronchial
adenoma with juxtaposed, solid, cellular zones and myxoid stroma. Ductlike and
solid myoepithelial component is adjacent to myxoid stromal component. (H and
E, x200)
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Radiographs may reflect the endobronchial obstruction caused by the polyp,
and CT may elucidate the cause in the setting of a foreign body or broncholith
(Figs. 9A and
9B). The histologic features
of inflammatory polyp are similar to those of a skin tag and include a
fibrovascular core lined by respiratory-type or metaplastic squamous
epithelium [1].
Amyloidoma
Amyloidosis includes several diseases characterized by the presence of
extracellular deposits of the insoluble protein fibril amyloid that
histochemically binds with Congo red, thus showing birefringence in polarized
light. Primary amyloidosis of the lung was classified by Spencer
[7] as follows: localized
bronchial deposits, diffuse bronchial deposits, localized parenchymal
deposits, and diffuse parenchymal deposits. Bronchial type amyloidosis occurs
as an isolated mass or as a diffuse infiltration within the wall. Diffuse
tracheobronchial amyloidosis is the most common form of primary lung
amyloidosis. Radiologic findings of tracheobronchial amyloidosis reflect the
pathologic findings and vary from a solitary mass to diffuse tracheal
narrowing. CT shows nodules, plaques, or circumferential thickening of the
trachea, main bronchus, and lobar and segmental bronchi with partial or
complete luminal narrowing (Figs.
10A and
10B). Mural nodulation and
calcification of the thickened tracheobronchial wall may be visible.
Papilloma
Squamous cell papilloma is the most common benign tracheobronchial
neoplasm. When it involves the tracheobronchial tree, this lesion has two
forms: multiple and solitary
[1]. The multiple form of
airway papilloma, known as juvenile laryngotracheal papillomatosis, is a
neoplastic growth secondary to infection with human papillomavirus types 6 and
11.
Solitary papilloma of the tracheobronchial tree is less common than the
multiple form and usually occurs in adults. Unlike the multiple form, solitary
papilloma is associated with cigarette smoking or arises de novo. Squamous
cell papilloma usually is benign in children and may regress. In adults,
however, this tumor can infiltrate and become malignant.
Gross examination shows papillomas are polypoid or sessile masses within
the airways. The lesions are characterized histologically by a proliferation
of well-differentiated squamous epithelium with a central fibrovascular core.
Radiologic findings include polypoid intraluminal masses, atelectasis, and
obstructive pneumonitis (Figs.
11A,
11B,
11C, and
11D). Involvement of the
distal airway can produce pulmonary nodules that frequently have
cavitations.
Pleomorphic Adenoma (Benign Mixed Tumor)
Primary pulmonary tumors histologically similar to salivary gland tumors
are exceedingly rare, and most are malignant. Of the benign bronchial gland
tumors that mimic salivary tumors, the most common is probably pleomorphic
adenoma (benign mixed tumor). Although it is the most common form of salivary
gland tumor, pleomorphic adenoma is exceedingly rare in the lung
[1].
Pulmonary pleomorphic adenoma has been reported in patients 11-74 years
old, but it occurs most often in the sixth and seventh decades. The tumor
occurs equally in men and women. Most tumors are polypoid endobronchial
masses, but peripheral tumors do occur. Endobronchial lesions are often
polypoid and exhibit some degree of luminal occlusion. The lesions are
circumscribed, unencapsulated, and exhibit a grayish white, rubber or myxoid
cut surface.
The CT finding in reported cases of pleomorphic adenoma was an endotracheal
polypoid mass or mass in the peripheral aspect of the lung
[8]. In our case, the adenoma
was located within the apical segmental bronchus and caused recurrent
pneumonia (Figs. 12A and
12B). The prognosis and
behavior of pulmonary pleomorphic adenoma are similar to those of salivary
gland pleomorphic adenoma. Circumscription and small size are associated with
a benign course when this tumor is managed by lobectomy. Infiltrative or
poorly circumscribed lesions are more likely to recur or metastasize
[1].
Other Benign Tumors
Benign tumors of the tracheobronchial tree not found among our cases
include granular cell tumor, hemangioma, and fibroma. Granular cell tumors are
uncommon benign neoplasms of neurogenic origin. Within the respiratory tract,
the larynx is most frequently affected, followed by the bronchi. The trachea
is rarely affected [9].
Approximated 2-6% of all granular cell tumors occur in the lung with equal sex
incidence and at a median age of 40 years (range, 8-59 years)
[1]. Chest CT is useful for
identifying the endobronchial mass, but there are no specific imaging features
for the diagnosis of granular cell tumor
[1].
Hemangiomas in adults occur more commonly in the larynx. In children, they
occur predominantly in the subglottic area
[9]. The radiologic finding of
subglottic hemangioma is a sharply marginated, rounded soft-tissue mass often
associated with considerable airway narrowing
[9].
Fibroma of the tracheobronchial tree is rare. In a study of 185 benign
tumors of the tracheobronchial tree, 2.2% (4 cases) of the lesions were
fibroma [10]. Tracheobronchial
fibromas are histologically identical to localized fibrous tumors of pleura
[11]. Like other benign
mesenchymal tumors, tracheal fibroma appears as a round or oval, well-defined,
submucosal mass with projection into the tracheal lumen
[12].
Acknowledgments
We thank Bonnie Hami, Department of Radiology, University Hospitals of
Cleveland, Cleveland, OH, for her editorial assistance in the preparation of
the manuscript.
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Abstract
Introduction
