CHAPTER 17 
Neoplasms of the Lung
and Airways
Brett W. Carter, Girish S. Shroff, and Carol C. Wu
CHAPTER OUTLINE
Introduction, 265
Lung Neoplasms, 265
Imaging of Lung Cancer, 265
Malignant Neoplasms, 266
Lung Cancer Staging, 271
Molecular Profiling, 272
Benign Neoplasms, 273
■  Introduction
Primary lung cancer is the leading cause of cancer-related
mortality worldwide and accounts for more deaths than
the next three malignancies combined—pancreatic,
colorectal, and pancreatic cancer in men; breast, colorec-
tal, and pancreatic cancer in women. In 2017, it was
estimated that 222,500 new cases would be diagnosed,
with 155,870 deaths expected from the disease. Lung
cancers are generally divided into two types, non–small
cell lung carcinoma (NSCLC), which accounts for 85% of
all lung cancers, and small cell lung carcinoma (SCLC),
which represents 15% of all lung cancers. NSCLC
primarily includes adenocarcinoma and squamous cell
carcinoma, as well as less common tumors such as large
cell carcinoma, sarcomatoid carcinoma, and spindle
cell sarcoma. SCLC is an aggressive malignancy and
the most common primary pulmonary neuroendocrine
neoplasm.
■  Lung Neoplasms
Imaging of Lung Cancer
Diagnostic imaging plays a key role in the identification,
characterization, and staging of lung cancer. Suspicious
pulmonary lesions may be initially identified on chest
radiography because the modality is widely available
and relatively inexpensive. However, there are significant
limitations of radiography, including the inability to detect
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Airway Neoplasms, 273
Imaging of Airway Neoplasms, 273
Malignant Airway Neoplasms, 274
Benign Airway Neoplasms, 277
Conclusion, 278
small nodules and other abnormalities, poor delineation
of primary tumor and nodal status, and the inability to
evaluate for extrathoracic disease. It has been reported that
approximately 12% to 30% of lung cancers are missed
on chest radiography. Multidetector computed tomog-
raphy (MDCT) is the primary imaging modality used to
evaluate pulmonary nodules and masses, characterize
primary tumors, and detect intrathoracic and extrathoracic
lymphadenopathy and metastatic disease. Limitations of
MDCT include inferiority to MRI in the visualization of
mediastinal and chest wall invasion and inferiority to
fluorodeoxyglucose (FDG)–positron emission tomography
(PET)/CT in the evaluation of nodal and metastatic
disease. MRI is not routinely used in the evaluation
of lung cancer, but is complementary to conventional
imaging techniques. Advantages of MRI include the ability
to evaluate the status of the intrathoracic vasculature
when there are contraindications to contrast-enhanced
MDCT (e.g., renal failure, severe contrast allergy) and
improved evaluation of the mediastinum and chest wall
compared to MDCT. Limitations of MRI include inherent
problems with imaging the chest, although improved
MR techniques have resulted in decreased scan times
and diminished respiratory and cardiac motion. PET/
CT has demonstrated an improved ability to assess for
intrathoracic and extrathoracic lymphadenopathy and
metastatic disease, assess response to therapy, and detect
residual and/or recurrent disease. Important restrictions
of PET/CT include limited characterization of the primary
tumor, limited evaluation of mediastinal and chest wall
invasion, and false-positives and false-negatives, which
can result in misinterpretation.
265
266 SECTION 4  Entities by Pathologic Category

BOX 17.1  IASLC/ATS/ERS Classification of

Lung Adenocarcinoma
Preinvasive lesions
Atypical adenomatous hyperplasia
Adenocarcinoma in situ
Nonmucinous
Mucinous
Mixed nonmucinous/mucinous
Minimally invasive adenocarcinoma
Nonmucinous
Mucinous
Mixed nonmucinous/mucinous
Invasive adenocarcinoma
Lepidic predominant
Acinar predominant
Papillary predominant
Micropapillary predominant
Solid predominant with mucin production
Variants of invasive adenocarcinoma
Invasive mucinous adenocarcinoma
Figure 17.1  Collimated contrast-enhanced axial CT scan of a
Colloid 64-year-old man with a history of adenocarcinoma of the left upper
Fetal lobe status post–left upper lobectomy demonstrates a 9-mm ground-glass
Enteric nodule (arrow) in the left lower lobe. Wedge resection revealed atypical
adenomatous hyperplasia.
ATS, American Thoracic Society; ERS, European Respiratory Society;
IASLC, International Association for the Study of Lung Cancer.

Malignant Neoplasms

Adenocarcinoma
Adenocarcinoma is the most common subtype of NSCLC
and represents a spectrum of malignant epithelial
neoplasms characterized by glandular differentiation
or mucus production. It is more common in women
than in men and is associated with smoking in 46% of
cases. Adenocarcinoma includes a wide variety of patterns
and differentiation. An updated classification system
was introduced in 2011 by a joint working group of the
International Association for the Study of Lung Cancer
(IASLC), American Thoracic Society (ATS), and European
Respiratory Society (ERS) emphasizing the correlation
among pathology, imaging, and clinical topics such as
diagnosis, treatment, and prognosis (Box 17.1). This new
system eliminated the term bronchioloalveolar carcinoma
(BAC) and grouped lesions into the following catego-
ries: (1) preinvasive lesions; (2) atypical adenomatous
hyperplasia; (3) adenocarcinoma in situ; (4) minimally Figure 17.2  Collimated contrast-enhanced axial CT scan of a
invasive adenocarcinoma; (5) invasive adenocarcinoma; 68-year-old woman with a history of adenocarcinoma of the right
lower lobe treated with chemoradiation therapy demonstrates a part
(6) invasive adenocarcinoma; and (7) variants of invasive solid nodule (white arrow) in the right middle lobe and a ground-glass
adenocarcinoma. nodule (black arrow) in the right lower lobe. Biopsy of these nodules
Atypical adenomatous hyperplasia (AAH) represents a revealed adenocarcinoma in situ.
small proliferation of atypical type II pneumocytes, Clara
cells, and respiratory bronchioles measuring ≤5 mm. On
MDCT, AAH manifests as a focal ground-glass nodule, type represents the majority of adenocarcinomas. On
typically measuring ≤5 mm, although they may range MDCT, AIS appears as a ground-glass, solid, or part
up to 12 mm in size (Fig. 17.1). Adenocarcinoma solid nodule typically measuring ≤3 cm. These nodules
in situ (AIS), formerly referred to as BAC, represents may demonstrate slightly higher attenuation than AAH
adenocarcinoma with pure lepidic growth, no evidence (Fig. 17.2). Minimally invasive adenocarcinomas (MIAs)
of nuclear atypia, and no evidence of stromal, vascular, are characterized by lepidic predominant growth and
or pleural invasion. Nonmucinous, mucinous, and mixed invasion of ≤5 mm. On MDCT, MIAs usually manifest
types have been described, although the nonmucinous as a part solid nodule with a predominant ground-glass

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 CHAPTER 17  Neoplasms of the Lung and Airways 267

component and a solid component measuring ≤5 mm
(Fig. 17.3). Invasive adenocarcinomas are characterized
by lepidic predominant spread and invasion of ≥5 mm.
Acinar predominant, papillary predominant, micropapil-
lary predominant, and solid predominant with mucin
production types have been described. On MDCT, invasive
adenocarcinomas manifest as a solid or part solid nodule,
with a predominantly solid component (Fig. 17.4). Local
invasion of the pleura, chest wall, or diaphragm may
be present. Other findings include air bronchograms,
internal lucent regions, and masslike consolidation.
Multiple pulmonary nodules, masses, and/or regions
of consolidation may be present. PET/CT is most effec-
tive at staging and restaging aggressive tumors such as
invasive adenocarcinomas. Other malignancies such as
AIS and MIA may demonstrate little to no FDG uptake,
which may be due to poor cellularity or slow cellular
proliferation (Fig. 17.5).
Squamous Cell Carcinoma
Squamous cell carcinoma (SCC) is the second most
common subtype of NSCLC and accounts for 25% to

Figure 17.3  Collimated contrast-enhanced axial CT scan of a
73-year-old woman demonstrates a part solid nodule (arrow) in the
left lower lobe, with both ground-glass and solid components. A left
lower lobectomy was performed and revealed minimally invasive
adenocarcinoma.
Figure 17.4  Collimated contrast-enhanced axial CT scan of a 43-year-
old woman demonstrates a solid mass (white arrow) with irregular
and spiculated margins in the left upper lobe. Note the presence of
pleural tags (black arrow). Biopsy revealed invasive adenocarcinoma.

A B

Figure 17.5  FDG-PET/CT of adenocarcinoma. (A) Collimated axial fused PET/CT image of a 52-year-old
woman demonstrates a lobular nodule in the right lower lobe (arrow) with FDG uptake similar to that
of the adjacent chest wall soft tissues. Biopsy revealed minimally invasive adenocarcinoma. (B) Collimated
axial fused PET/CT image of a 67-year-old woman with a large soft tissue mass in the right lower lobe
that is markedly FDG-avid. Biopsy revealed invasive adenocarcinoma.

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268 SECTION 4  Entities by Pathologic Category

Figure 17.6  Collimated contrast-enhanced axial CT scan of a

92-year-old man demonstrates a large, thick-walled, cavitary mass
(arrows) in the right lower lobe. Biopsy revealed squamous cell carcinoma
of the lung.

30% of all lung cancers. It is strongly associated with

smoking, and 50% of tumors develop in current or former
smokers. SCC is more common in men than women.
Four histopathologic variants have been described—
papillary, small cell, clear cell, and basaloid—and 7%
to 10% of patients are asymptomatic. Clinical symptoms
are more likely to be present when lesions are centrally
located and in an advanced stage of disease. When present,
symptoms most commonly reported include cough,
dyspnea, hemoptysis, and fever. Paraneoplastic syndromes
such as hypercalcemia due to secretion of a parathyroid
hormone–like substance may be present. SCCs arising
in the lung apices, also referred to as Pancoast tumors,
can result in neuropathic pain due to involvement of
the brachial plexus or Horner syndrome due to involve-
ment of the sympathetic chain.
SCC typically arises from large bronchi in the central
aspect of the chest. Thus, on MDCT, most SCCs manifest
as a centrally located nodule or mass. An endobronchial
component may be present, and postobstructive atelectasis
and/or pneumonia may be visible. Alternatively, tumors
may appear as a peripherally located nodule or mass,
with lobulated or spiculated margins. Cavitation may be
present and is more common in larger lesions (Fig. 17.6).
Central necrosis and wall thickening may be present.
Calcification has been reported in 13% of lesions. On
PET/CT, most tumors demonstrate FDG uptake greater
than the mediastinal background. MRI can be used to
assess for chest wall invasion and involvement of the
brachial plexus in Pancoast tumors (Fig. 17.7).
Large Cell Lung Carcinoma
Large cell lung carcinoma (LCLC) represents a hetero-
geneous group of neoplasms that account for 5% to 10%
of all lung cancers. LCLC encompasses several malignan-
cies, including giant cell carcinoma, basaloid large cell
carcinoma, clear cell carcinoma, lymphoepithelioma-like
B
Figure 17.7  Pancoast tumor with chest wall invasion. (A) Collimated
contrast-enhanced axial CT scan of a 19-year-old woman shows a soft
tissue mass (asterisk) in the right lung apex, representing a primary
Pancoast tumor. (B) Sagittal T2-weighted MR image of the same patient
demonstrates invasion of the right chest wall (arrows) by the tumor
(asterisk).
carcinoma, large cell carcinoma with rhabdoid phenotype,
and large cell neuroendocrine carcinoma (LCNEC). LCLC
has been described as a diagnosis of exclusion in that
tumor cells lack histopathologic features that would
otherwise classify the lesion as an SCLC or specific subtype
of NSCLC. LCNEC is one of the most clinically significant
LCLCs and represents a high-grade neuroendocrine
malignancy characterized by neuroendocrine histologic
features. Most LCNECs occur in men with a history of
heavy smoking, and the mean age at the time of diagnosis
is 65 years. Clinical symptoms include chest pain,
hemoptysis, cough, and other symptoms such as dyspnea,
weight loss, and fever. Unlike SCC, paraneoplastic syn-
dromes are rare.
On MDCT, LCNEC is usually indistinguishable from
other lung cancers. Lesions usually appear as a large

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 CHAPTER 17  Neoplasms of the Lung and Airways 269

Figure 17.8  Collimated contrast-enhanced axial CT scan of a

74-year-old man demonstrates a large soft tissue mass (arrow) in the
right lower lobe. Note the presence of internal vascularity. Biopsy
revealed large cell neuroendocrine carcinoma.

mass in the peripheral aspect of the chest, ranging in

size from 13 to 92 mm (Fig. 17.8). Only one-fifth of
lesions are centrally located. Most tumors demonstrate
lobular margins, although tumors with spiculated borders
have been described. The attenuation of LCNEC has been B
described as similar to that of chest wall musculature.
Following the administration of IV contrast, tumors dem-
onstrate varying degrees of enhancement. Heterogeneous
or peripheral enhancement may be seen in the setting of
necrosis. Calcification is an infrequent finding and has
been reported in less than 9% of cases. LCNEC typically
demonstrates increased FDG uptake on PET/CT, and the
modality is used for staging and restaging purposes.

Small Cell Lung Carcinoma

SCLCs account for approximately 13% to 15% of all
lung cancers and are the most common primary pulmo-
nary neuroendocrine neoplasm. SCLC has the strongest
association with cigarette smoking, which is responsible
for approximately 95% of cases of SCLC. In general,
SCLC is much more aggressive than NSCLC and has a
greater propensity for the early development of widespread
metastases. SCLC has traditionally been staged with the
Veterans Administration Lung Study Group (VALSG)
systems, which divide SCLC into limited stage (LS) and
extensive stage (ES) based on radiation treatment plan-
ning. However, the IASLC has recommended that the
current seventh edition of the American Joint Committee
on Cancer (AJCC) tumor-node-metastasis (TNM) staging
system for lung cancer be used to stage SCLC.
Because 90% to 95% of SCLCs arise from lobar or
main bronchi, most SCLCs are located within the central
aspect of the chest and manifest as a mediastinal (92%) or
hilar (84%) lymphadenopathy or pulmonary lesion (Fig.
17.9). Postobstructive atelectasis and/or pneumonia may
be seen in these patients. MDCT can be useful in revealing
the presence and extent of mediastinal invasion. In 68%
C
Figure 17.9  Manifestations of small cell lung carcinoma (SCLC). (A)
Posteroanterior chest radiograph of a 52-year-old woman presenting
with chest pain demonstrates thickening of the right paratracheal stripe
(black arrow) and convexity of the right hilum (white arrow) suggestive
of lymphadenopathy. (B) Collimated contrast-enhanced axial CT scan
of the same patient confirms the presence of a soft tissue mass in the
right hilum and mediastinal lymphadenopathy. Biopsy revealed SCLC.
(C) Collimated contrast-enhanced axial CT scan of a 52-year-old man
with SCLC demonstrates a soft tissue mass (arrows) in the medial left
upper lobe, with lobular margins representing the primary tumor.
of patients, encasement of mediastinal structures such as
the trachea, esophagus, heart, and vessels, including the
superior vena cava, is evident (Fig. 17.10). Intratumoral
calcification has been reported in 23% of patients. A small
group of patients with SCLC (<5%) may have a peripheral
pulmonary nodule without associated lymphadenopathy.

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270 SECTION 4  Entities by Pathologic Category

A
Figure 17.10  Superior vena cava obstruction due to small cell lung
carcinoma (SCLC). Collimated contrast-enhanced axial CT scan of a
52-year-old man with SCLC demonstrates soft tissue in the right
paratracheal region (asterisk) obliterating the superior vena cava. Note
the collateral vessels in the left chest wall and mediastinum. A cavitary
metastasis (arrow) is present in the left lung.

These peripherally located tumors usually manifest as

well-defined nodules or masses that are homogeneous
and demonstrate lobular margins and spiculation. Sur-
rounding ground-glass opacities, representing edema or
hemorrhage, may be identified. Unusual manifestations
of SCLC on MDCT include consolidation, air space opaci-
ties, and lymphangitic carcinomatosis. On PET/CT, most
SCLCs demonstrate markedly increased FDG uptake due
to high metabolic activity. PET/CT has been shown to
stage patients more accurately than conventional imaging
and can be used for restaging purposes.
Carcinoid
Carcinoid tumors are neuroendocrine neoplasms that
are typically found in the gastrointestinal tract; however,
20% to 30% of all carcinoids arise from the respiratory
tract, and these neoplasms represent 1% to 2% of all
primary lung cancers. Carcinoid tumors are classified
based on mitotic activity—typical (low grade) and atypical
(intermediate grade). Most neoplasms are typical carci-
noids, which are well-differentiated neoplasms with
neuroendocrine features measuring ≥5 mm and exhibiting
less than 2 mitoses/10 high-power field (HPF) or 2 mm2
and no necrosis. Typical carcinoids are less aggressive,
and 13% of patients present with lymph node involve-
ment. Atypical carcinoid tumors account for 10% to 16%
of carcinoids, are characterized by 2 to 10 mitoses/HPF
or necrosis, and are more aggressive, and more patients
(57%) present with lymph node involvement.
Typical and atypical carcinoids demonstrate similar
features on imaging studies. The most common finding
on chest radiography is a well-defined hilar or perihilar
nodule or mass. In general, carcinoids are more common
in the right lung than in the left lung. The average size
is 3 cm, with a range of 2 to 5 cm, although atypical
B
Figure 17.11  Imaging manifestations of carcinoid. (A) Collimated
contrast-enhanced axial CT scan of a 70-year-old man demonstrates a
soft tissue mass in the right lower lobe, with eccentric calcification
(arrow). Biopsy revealed carcinoid. (B) Collimated contrast-enhanced
axial CT scan of a 46-year-old woman demonstrates a carcinoid tumor
in the left lower lobe (white arrow) with extensive peripheral enhance-
ment and vascularity (black arrow).
carcinoids tend to be larger than typical carcinoids and
are more likely to be located in the lung periphery. Associ-
ated findings, such as postobstructive atelectasis and/or
pneumonia, may be present. MDCT usually demonstrates
a soft tissue nodule that is often closely associated with
an airway. In some cases, the tumor may be entirely
endoluminal or partially endoluminal. Punctate, eccentric,
or diffuse patterns of calcification have been reported in
30% of cases. Associated findings such as postobstruc-
tive atelectasis and/or pneumonia, mucoid impaction,
bronchiectasis, or air trapping may be present. Carcinoid
tumors may demonstrate enhancement following the
administration of IV contrast (Fig. 17.11). PET/CT is of
limited utility in the evaluation of carcinoids because

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Figure 17.12  Collimated axial fused PET/CT image of a 47-year-old
woman with a history of breast cancer demonstrates a well-circumscribed
nodule (arrow) in the right lower lobe. FDG uptake within the nodule
is similar to background. Biopsy revealed typical carcinoid.
tumors may demonstrate little to no FDG uptake, and
a high false-negative rate has been reported (Fig. 17.12).
Lung Cancer Staging
The IASLC has recommended that the current eighth
edition of the AJCC TNM staging system be used to stage
NSCLC, SCLC, and bronchopulmonary carcinoid. Tra-
ditionally, SCLC has been staged with the VALSG and
modified VALSG systems, which divide SCLC into LS
and ES based on radiation treatment planning.
VALSG and Modified VALSG Staging Systems
Although it has been recommended that the TNM system
replace the VALSG staging systems for the staging of SCLC,
a brief review of the latter is beneficial because they are
still commonly used in clinical practice. In the original
VALSG system, LS-SCLC was characterized by tumor
involvement limited to one hemithorax (with or without
local extension) and no distant extrathoracic metastatic
disease (Box 17.2). Regional and ipsilateral supraclavicular
lymph nodes were considered to be LS-SCLC if these
groups could be included in a single safe and adequate
radiation port. ES-SCLC applied to all other patients;
this included specific features such as malignant pleural
and pericardial effusions, contralateral hilar or supracla-
vicular lymph nodes, and metastatic disease that could
not be treated in a single radiation port. In 1989, the
IASLC proposed key changes to the VALSG system (Box
17.3), the most significant of which included contralateral
mediastinal and supraclavicular lymph nodes and ipsi-
lateral pleural effusions (benign and malignant, regardless
of cytology) into LS-SCLC. ES-SCLC disease was defined
as disease beyond that of LS-SCLC. A retrospective study
at one institution indicated that the IASLC system had
better prognostic value than the VALSG system. In clinical
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CHAPTER 17  Neoplasms of the Lung and Airways 271
BOX 17.2  Veterans Administration Lung
Study Group Staging System
Limited Stage
Confined to single radiation port
Confined to ipsilateral mediastinum
Ipsilateral mediastinal or supraclavicular lymph
nodes
Extensive Stage
Disease not confined to single radiation port
Contralateral mediastinal or supraclavicular lymph
nodes
Malignant pleural or pericardial effusion
Metastatic disease
BOX 17.3  Modified Veterans Administration
Lung Study Group Staging System
Limited Stage
Confined to single radiation port
Ipsilateral mediastinal or supraclavicular lymph
nodes
Contralateral mediastinal or supraclavicular lymph
nodes
Ipsilateral pleural effusion (regardless of cytology)
Extensive Stage
Disease not confined to single radiation port
Metastatic disease
practice, features of the VALSG and IASLC staging systems
are often combined. For example, LS-SCLC can include
contralateral mediastinal and ipsilateral supraclavicular
lymph node involvement. However, certain findings, such
as contralateral supraclavicular or hilar lymph node
involvement, remain controversial, and clinical manage-
ment is typically based on the ability to include these
regions in a safe and adequate radiation port.
TNM System
The eighth edition of the AJCC TNM staging system has
made key changes to the system based on significant
differences in 5-year survival, as determined by the IASLC
Lung Cancer Staging Project. Highlights of the eighth
edition of the TNM staging system will be discussed here;
the complete scheme is outlined in Table 17.1.
One of the key changes made in the eighth edition
included the further subdivision of primary lung cancers
into tumor (T) descriptors based on size. The analysis
of the new database showed significant separation of T1
tumors from T2 lesions based on a threshold size of 3 cm,
as well as a progressive reduction in patient survival for
each 1-cm threshold (≤1 cm, >1–2 cm, >2–3 cm, >3–4 cm,
>4–5 cm, >5–6 cm, >6–7 cm, and >7 cm). T1 lung cancers
are subdivided into three groups at 1-cm thresholds,
including T1a lesions measuring ≤1 cm, T1b nodules
measuring >1 and ≤2 cm, and T1c tumors measuring >2
and ≤3 cm. T2 lesions have been subdivided into two
groups, including T2a tumors measuring >3 and ≤4 cm
272 SECTION 4  Entities by Pathologic Category

TABLE 17.1  Eighth Edition of the TNM Staging System (TNM-8)

T DESCRIPTOR DEFINITION
TX Primary tumor cannot be assessed or tumor proven by the presence of malignant cells in sputum or bronchial
washings but not visualized by imaging or bronchoscopy.

T0 No evidence of primary tumor

Tis Carcinoma in situ

T1 Tumor ≤ 3 cm in greatest dimension, surrounded by lung or visceral pleura, without bronchoscopic evidence of
invasion more proximal than the lobar bronchus
 T1a Tumor ≤ 1 cm in greatest dimension
 T1b Tumor > 1 cm and ≤ 2 cm in greatest dimension
 T1c Tumor > 2 cm and ≤ 3 cm in greatest dimension

T2 Tumor > 3 cm and ≤ 5 cm or tumor with any of the following features: involvement of a main bronchus regardless
of distance from the carina; invasion of the visceral pleura; partial or complete lung atelectasis or pneumonitis
 T2a Tumor > 3 cm and ≤ 4 cm in greatest dimension
 T2b Tumor > 4 cm and ≤ 5 cm in greatest dimension

T3 Tumor > 5 cm or directly invading any of the following structures: parietal pleura, chest wall, phrenic nerve,
parietal pericardium, or separate tumor nodule(s) in the same lobe as the primary lesion

T4 Tumor measuring >7 cm that invades any of the following structures: mediastinum, diaphragm, heart, great
vessels, trachea, recurrent laryngeal nerve, esophagus, vertebral body, carina; or separate tumor nodule(s) in a
different lobe of the same lung.

N DESCRIPTOR DEFINITION
NX Lymph nodes cannot be assessed.

N0 No regional lymph nodes

N1 Ipsilateral peribronchial and/or hilar lymph nodes and intrapulmonary nodes; includes lymph nodes affected by
direct extension

N2 Ipsilateral mediastinal and/or subcarinal lymph node(s)

N3 Contralateral mediastinal, contralateral hilar, ipsilateral or contralateral scalene, or supraclavicular lymph node(s)

M DESCRIPTOR DEFINITION
M0 No metastases

M1 Presence of metastasis
 M1a Intrathoracic metastases: separate tumor nodule(s) in contralateral lung; malignant pleural effusion or pleural
thickening, nodules, or masses; malignant pericardial effusion or pericardial thickening, nodules, or masses
 M1b Single extrathoracic metastasis in single distant organ
 M1c Multiple extrathoracic metastases in single or multiple distant organs

and T2b lesions measuring >4 and ≤5 cm. Lung cancers
measuring >5 cm and ≤7 cm are classified as T3 tumors,
and those measuring >7 cm are classified as T4 lesions.
Other significant modifications to the T descriptor
include the grouping of lung cancers resulting in atelectasis
of a lobe or the entire lung as T2 disease, the grouping
of lung cancers involving a main bronchus regardless of
distance from the carina as T2 disease, reclassification
of diaphragmatic invasion as T4 disease (previously T3
disease), and elimination of mediastinal pleural invasion
as a descriptor. No changes were made to lymph node
descriptors in the eighth edition. However, metastatic
disease was further subdivided into M1a, M1b, and M1c
components based on the location of metastases. For
example, intrathoracic metastatic disease, including
contralateral tumor nodules and pleural or pericardial
metastases, is designated M1a. Extrathoracic metastatic
disease is classified as M1b disease if there is a solitary
metastasis or a single distant organ is involved and is
classified as M1c disease if there are multiple metastases
in one or more distant organs.
Molecular Profiling
The improved molecular and genetic characterization of
lung cancer has revealed certain driver mutations that
may confer susceptibility or resistance to specific treat-
ments. Some of the most common driver mutations in
NSCLC include epidermal growth factor receptor (EGFR),
Kirsten rat sarcoma virus (KRAS), and anaplastic lym-
phoma kinase (ALK) mutations, which are almost always
mutually exclusive. Never-smokers with lung adenocar-
cinoma have the highest incidence of certain mutations,
such as EGFR and ALK. Activation of EGFR by EGF leads
to cellular growth, proliferation, and decreased apoptosis,
and these mutations are more common in adenocarci-
nomas, Asian patients, female patients, and never-smokers.
The most common EGFR mutations include deletion in
exon 19 and a point mutation in exon 21. The ALK gene
codes for a receptor tyrosine kinase and is fused to the
echinoderm microtubule-associated, protein-like 4 (EML4)
gene, producing the EML4-ALK fusion oncogene in 3%
to 7% of patients with NSCLC. The EML4-ALK fusion

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oncogene (or ALK rearrangement) promotes cell growth
and proliferation and is more likely to be seen in younger
patients who are light smokers or never-smokers with
adenocarcinoma of the lung. The KRAS gene codes for
RAS proteins, guanosine nucleotide binding proteins
located at the inner surface of the plasma membrane
that are involved in the transduction of growth signals
from receptor tyrosine kinases (RTKs). KRAS accounts
for most of the RAS mutations, are present in 25% to
40% of NSCLCs, and are more common in whites, current
or former smokers, and adenocarcinomas.
Benign Neoplasms
Hamartoma
Hamartoma is the most common benign pulmonary
neoplasms and is composed of connective tissue contain-
ing cartilage and varying amounts of fat, smooth muscle,
bone, and lymphovascular structures. Approximately 6%
of all solitary pulmonary nodules identified on imaging
studies represent hamartomas. Most lesions are identified
in patients in the sixth decade of life, and the incidence
is two or three times greater in men than women. Most
hamartomas are detected incidentally on radiographs or
MDCT performed for other reasons. These tumors are
characterized by slow growth, and malignant transforma-
tion is incredibly rare.
The most common appearance of hamartoma on
MDCT is a well-circumscribed solitary pulmonary
nodule or mass, with smooth or lobular borders in
the lung periphery. Calcification is more common as
lesions increase in size and is much better visualized on
MDCT than radiography. The characteristic “popcorn”
configuration of calcification is only present in 10% to
15% of cases. Internal fat (−40 to −120 Hounsfield units
on MDCT) is present in 60% of cases. The identification of
internal fat and calcium is typically considered diagnostic
of hamartoma (Fig. 17.13). Following the administration
of IV contrast, heterogeneous enhancement is typical.
Regions of internal enhancing septations may be present.
Although features on MDCT are typically sufficient to
make the diagnosis, lesions may be visible on MRI.
Hamartomas are typically of intermediate signal intensity
on T1-weighted imaging and high signal intensity on
T2-weighted imaging. Following the administration of
IV contrast, enhancement of the capsule and internal
septations may be present. On PET/CT, approximately
20% of lesions demonstrate FDG uptake; larger lesions
tend to be more FDG-avid than smaller lesions.
■  Airway Neoplasms
Tumors of the tracheobronchial tree are uncommon,
accounting for 2% of all respiratory malignancies in the
United States, with an estimated incidence of 1/1,000,000
individuals. Neoplasms affecting the bronchi are 400
times more common than tracheal tumors. Most (90%)
of tracheal tumors in adults are malignant, whereas most
in children are benign. Clinical symptoms reported at
the time of presentation are typically nonspecific and
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CHAPTER 17  Neoplasms of the Lung and Airways 273
Figure 17.13  Collimated contrast-enhanced axial CT scan of a
39-year-old woman demonstrates a left lower lobe nodule with internal
fat and calcification (arrow), characteristic of a pulmonary hamartoma.
may include stridor, wheezing, adult-onset asthma,
hemoptysis, and recurrent pneumonia. Because of the
relative rarity of these tumors and nonspecific symptoms,
diagnosis is often delayed.
Imaging of Airway Neoplasms
Radiography is typically the initial radiologic examination
that is performed when patients present with respiratory
symptoms; careful evaluation of the trachea and proximal
bronchial air columns is essential to detect abnormalities.
However, only 18% to 28% of tracheal tumors are visible
on radiography. The most common findings include a
lobular or rounded opacity in the tracheal or bronchial
air columns. Postobstructive atelectasis or pneumonia
may be present. Endoluminal lesions may produce a
ball valve mechanism and resultant hyperinflation and
air trapping. Chronic airway obstruction may lead to
regions of bronchiectasis or bronchiolectasis.
MDCT is the imaging modality of choice, optimally
demonstrates the presence and extent of disease, and
can be used for surgical planning. MDCT has the advan-
tages of fast acquisition times and widespread availability,
and imaging of the entire tracheobronchial tree can be
performed in seconds. Nonspecific findings such as
endoluminal lesions and airway wall thickening may be
identified. Features such as extraluminal extension,
mediastinal invasion, and irregular margins suggest
malignancy, whereas well-circumscribed margins suggest
a benign lesion. Several specific imaging features can be
used to narrow the differential diagnosis, including fat,
calcification, and enhancement. When fat is present,
hamartoma, lipoma, and liposarcoma should be included
in the differential diagnosis. In the setting of internal
calcification, hamartoma, carcinoid, chondroma, and
chondrosarcoma should be included. When enhancement
is present, carcinoid, hypervascular metastases from
274 SECTION 4  Entities by Pathologic Category

A B
Figure 17.14  Squamous cell carcinoma of the trachea. (A) Collimated contrast-enhanced axial CT
scan demonstrates thickening of the anterior wall of the trachea and an adjacent soft tissue mass (black
arrow). (B) Collimated axial fused PET/CT image of the same patient shows marked FDG uptake in the
lesion (white arrow), typical of squamous cell carcinoma.

primary malignancies such as thyroid and renal cell
carcinomas, melanoma, choriocarcinoma, and heman-
gioma should be considered.
Two-dimensional (2D) multiplanar reconstruction
(MPR) images and three-dimensional (3D) reconstruction
images can be used to provide additional information.
2D MPRs can improve delineation of the craniocaudal
extent of tumor and can be used to obtain accurate
measurements from a tumor to important anatomic
landmarks. 3D volume rendering or virtual bronchoscopy
provides an endoluminal perspective, the advantages of
which include the evaluation of patients in whom
bronchoscopy is contraindicated due to comorbidities
and bronchoscopes cannot be advanced secondary to
an obstructing tumor.
With the advent of MDCT with MPRs, there is no
distinct advantage to using MRI. The long acquisition
times associated with most studies may be difficult for
those with respiratory symptoms. However, it can be
useful for follow-up imaging of children and young adults
by limiting cumulative radiation doses.
FDG-PET/CT has been increasingly used in the evalu-
ation of a wide variety of neoplasms. The presence and
extent of FDG uptake in airway neoplasms depend on
tumor histology because highly malignant tumors such
as SCC demonstrate intense FDG uptake, but more
indolent lesions such as carcinoid demonstrate little to
no FDG uptake. PET/CT effectively stages patients by
demonstrating metastatic disease and can provide guid-
ance for tissue biopsy.
Malignant Airway Neoplasms
Squamous Cell Carcinoma
SCC is the most common primary malignancy of the
trachea and constitutes approximately 50% of all tracheal
malignancies. It is strongly associated with cigarette
smoking and typically affects men 50 to 60 years of age.
Nearly 40% of patients develop synchronous or meta-
chronous malignancies of the head and neck or lung.
Mediastinal lymphadenopathy and/or pulmonary
metastases are present in one-third of patients at the
time of diagnosis.
The most common appearance of SCC on MDCT is
a polypoid intraluminal mass, the borders of which may
be highly variable and demonstrate smooth, lobular, or
irregular characteristics (Fig. 17.14). Although tumors
may arise from any portion of the tracheobronchial tree,
there is preference for the posterior wall of the lower
two-thirds of the trachea. Lesions may be confined to
the affected portion of the airway or result in extraluminal
extension into the adjacent mediastinum and affect
structures such as the esophagus. These cases may be
complicated by the development of a tracheoesophageal
fistula. Alternatively, SCC may manifest as airway wall
thickening, which can be focal or circumferential. SCCs
typically demonstrate increased FDG uptake on PET/CT
(see Fig. 17.14).
Adenoid Cystic Carcinoma
Adenoid cystic carcinomas are the second most common
primary malignancy of the trachea and arise from the
submucosal minor salivary glands. Unlike SCCs, there
is no association with cigarette smoking. Men and women
are affected equally and patients are typically diagnosed
in the fourth decade of life. Regional lymph nodes are
affected in approximately 10% of patients at the time of
diagnosis.
The most common imaging manifestation of adenoid
cystic carcinoma is a focal intraluminal soft tissue mass.
Alternatively, tumors can result in diffuse or circumfer-
ential thickening of airway walls, which may or may not
result in luminal stenosis (Fig. 17.15). The lower trachea

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Figure 17.15  Sagittal reformatted image from a contrast-enhanced
CT scan demonstrates irregular thickening of the anterior and posterior
walls (arrows) of the trachea. Biopsy revealed adenoid cystic carcinoma.
and main bronchi are the most commonly affected
regions. Because of the tendency for adenoid cystic
carcinoma to result in submucosal and perineural spread,
the full extent of disease can be underestimated on
cross-sectional imaging; thus sagittal and coronal MPR
imaging is effective at demonstrating the longitudinal
spread of tumor. Adenoid cystic carcinoma commonly
shows extraluminal extension. Because most tumors
demonstrate slow growth, FDG uptake on PET/CT can
be highly variable, with one study showing a maximum
standardized uptake value (SUVmax) of 3.7 to 3.8. High-
grade tumors tend to demonstrate greater FDG uptake.
Mucoepidermoid Carcinoma
Mucoepidermoid carcinomas are rare primary malignan-
cies of salivary gland origin that represent 0.1% to 0.2%
of all pulmonary neoplasms. Metastatic disease is present
in 10% of patients at the time of diagnosis. Patients
younger than 40 years are most frequently affected.
The most common imaging appearance of mucoepi-
dermoid carcinoma is an intraluminal soft tissue nodule
with mild to marked heterogeneous enhancement fol-
lowing the administration of IV contrast. Thus these
lesions may be indistinguishable from carcinoid tumors
on MDCT. Punctate internal calcifications have been
reported in up to 50% of cases. Although any portion
of the tracheobronchial tree can be affected, 45% arise
from the central airways (the main bronchi more com-
monly than the trachea) and 55% arise from the distal
airways (usually a segmental bronchus; Fig. 17.16).
Variable FDG uptake on PET/CT has been reported;
low-grade tumors tend to result in mild FDG uptake,
whereas high-grade tumors show high FDG uptake.
Carcinoid
Carcinoid tumors are the most common endobronchial
neoplasm diagnosed in young adults; most patients
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CHAPTER 17  Neoplasms of the Lung and Airways 275
Figure 17.16  Collimated noncontrast axial CT scan of a 51-year-old
man demonstrates a soft tissue mass (arrow) in the left main bronchus
and complete atelectasis of the left lung (asterisk). Endobronchial biopsy
revealed mucoepidermoid carcinoma.
present during the fourth decade of life. Men and women
are affected equally. Carcinoid tumors are neuroendocrine
tumors that are classified based on mitotic activity—typical
(low grade) and atypical (intermediate grade). Clinically,
patients may present with paraneoplastic syndromes
related to the secretion of adrenocorticotropic hormone
(ACTH) or serotonin.
Carcinoid tumors may manifest as postobstructive
atelectasis or pneumonia resulting from the endobronchial
location of these lesions. On MDCT, an intraluminal
soft tissue mass with smooth or lobulated margins is
the most common appearance. Typical carcinoids tend
to be centrally located in the main, lobar, or segmental
bronchi, whereas atypical carcinoids tend to develop in
the lung periphery. The iceberg phenomenon has been
described, in which large lesions may manifest as hilar
or perihilar masses with a large extraluminal component
and a small intraluminal component. Carcinoid tumors
may demonstrate intense enhancement following the
administration of IV contrast (Fig. 17.17). Internal cal-
cification has been described in up to 20% of cases.
Lesions demonstrate variable uptake on FDG-PET/CT.
In general, FDG uptake is lower than in other tracheo-
bronchial malignancies, and tumors may demonstrate
no FDG uptake or uptake less than that of the mediastinal
background.
Sarcomas and Lymphoma
Sarcomas and lymphomas represent an uncommon,
heterogeneous group of malignancies that may involve
the tracheobronchial tree. The most common types of
sarcoma include spindle cell sarcoma, chondrosarcoma,
leiomyosarcoma, fibrosarcoma, and synovial sarcoma.
A wide variety of primary lymphomas, including Hodgkin
lymphoma and various non-Hodgkin lymphomas, the
latter of which include mucosa-associated lymphoid tissue
(MALT), anaplastic large cell, unspecified T cell, mantle
276 SECTION 4  Entities by Pathologic Category

A B
Figure 17.17  Endobronchial carcinoid tumor. (A) Collimated contrast-enhanced axial CT scan on
lung windows demonstrates a bilobed lesion (black arrow) in the left main bronchus. (B) Collimated
contrast-enhanced axial CT scan of the same patient on soft tissue windows shows marked enhancement
of a portion of the lesion (white arrow). Biopsy revealed carcinoid tumor, which was entirely endoluminal
at the time of resection.

cell, B cell, lymphocytic, diffuse large histiocytic, and

diffuse large cell immunoblastic lymphomas, have been
described. In general, histopathologic analysis is necessary
to differentiate sarcomas and lymphomas from other
primary airway malignancies. Patients may be asymp-
tomatic or may present with dyspnea, cough, wheezing
or stridor, and hoarseness. Hemoptysis may complicate
sarcomas but is only rarely reported with lymphoma.
The most common finding on chest radiography is a
lobulated or rounded opacity in the tracheal or bronchial
air columns. Nonspecific findings such as atelectasis or
recurrent or nonresolving pneumonia may be the only
radiographic abnormality. On MDCT, sarcomas usually
manifest as intraluminal soft tissue masses, although
extraluminal extension into the mediastinum or lung
may occur. The presence of stippled or amorphous internal
calcification suggests chondrosarcoma, although it is
important to remember that benign lesions such as
carcinoid, hamartoma, and chondroma may demonstrate
calcification and are much more common. Lymphoma
typically results in a solitary intraluminal soft tissue Figure 17.18  Collimated contrast-enhanced axial CT scan demon-
nodule or mass. Alternatively, lobulated thickening of strates a hypodense lesion in the right thyroid lobe representing
the affected airway, representing submucosal infiltration, medullary thyroid carcinoma (asterisk). Note the invasion of the right
may be the predominant finding. and anterior aspects of the adjacent trachea (arrow).

Metastatic Disease
Metastases affecting the airways are more common than
primary tumors and may be the result of direct invasion
or hematogenous spread. Tumors that tend to spread
directly through to the airways include primary malignan-
cies of the larynx, thyroid gland, lung, mediastinum, and
esophagus. Tumors that tend to spread hematogenously
include melanoma, sarcoma, and colorectal, breast, and
lung cancers.
Direct invasion of the trachea manifests as a primary
tumor extending into the airways, with an intraluminal
mass and/or airway thickening (Fig. 17.18). Destruction
of tracheal or bronchial cartilage may be present. These
lesions may be complicated by tracheoesophageal or
bronchoesophageal fistulas. For hematogenous metastases,
the imaging characteristics of metastases typically mirror
those of the primary tumor. For example, vascular tumors
such as melanoma and renal cell carcinoma may dem-
onstrate intense enhancement following the administra-
tion of IV contrast.

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Benign Airway Neoplasms
Squamous Cell Papilloma and Tracheobronchial
Papillomatosis
Squamous cell papilloma is the most common benign
neoplasm of the tracheobronchial tree and is composed
of a core of fibrovascular tissue surrounded by stratified
squamous epithelium. Men are affected more than
women, and the mean age at the time of diagnosis is 51
years. Papillomas are associated with cigarette smoking.
These lesions may be found in the solitary form as a
papilloma or tracheobronchial papillomatosis when
multiple lesions are present. Tracheobronchial papillo-
matosis is the result of infection with human papilloma
virus types 6 and 11, typically acquired from an affected
mother during vaginal delivery.
The most common imaging manifestation of squamous
cell papilloma is a solitary small nodule projecting into
the airway lumen, without extraluminal extension (Fig.
17.19). Most papillomas are present in a lobar bronchus,
whereas involvement of a main bronchus or the trachea
is less common. Malignant transformation has been
described. Tracheobronchial papillomatosis manifests as
multiple intraluminal polypoid nodules of various size
that usually occur in the trachea. The central airways are
involved in 5% of cases, and the small airways and lungs
are involved in less than 1%. When it spreads to the
lungs, nodules may cavitate and demonstrate air-fluid
levels. These nodules typically present in the posterior
half of the chest. Malignant transformation to SCC can
occur.
Hamartoma and Lipoma
Hamartomas are benign neoplasms consisting of cartilage,
fat, bone, smooth muscle cells, and connective tissue;
1.4% to 3% of hamartomas are endobronchial lesions,
Figure 17.19  Sagittal reformatted image from a contrast-enhanced
CT scan demonstrates a soft tissue mass (arrow) with lobular margins
arising from the posterior wall of the trachea. Biopsy revealed solitary
papilloma.
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CHAPTER 17  Neoplasms of the Lung and Airways 277
and neoplasms involving the trachea are even less
common. Men are more commonly affected than women,
and the median age at the time of diagnosis is 62 years.
Hamartomas of the airway typically contain less fat and
more cartilaginous components than parenchymal
hamartomas. The most common manifestation of
hamartoma is an exophytic, polypoid, or sessile mass
with smooth margins. The presence of fat and/or internal
calcification on MDCT suggests the diagnosis. MRI may
be beneficial in some scenarios because the fat compo-
nents demonstrate high signal intensity on T1- and
T2-weighted imaging.
Lipomas are rare tumors that arise from the submucosal
adipose tissue of the tracheobronchial tree. These lesions
are slightly more common in men than women and are
typically seen in patients 60 years of age. Lipomas
demonstrate fat attenuation on MDCT and signal intensity
on T1- and T2-weighted MRI. In most cases, the identifica-
tion of fat is enough to suggest the diagnosis.
Hemangioma
Hemangiomas of the trachea are classified as benign
neoplasms of mesenchymal origin and usually occur in
children. Specifically, capillary hemangioma is the most
common subglottic mass in children. On MDCT, hem-
angiomas manifest as well-marginated, soft tissue masses
located in the posterior or posterolateral aspect of the
subglottic trachea. Involvement of the distal airways is
much less common.
Glomus Tumor
Glomus tumor is an uncommon soft tissue neoplasm
that usually occurs in the extremities, but may also affect
the airways. It represents a neoplastic proliferation of
smooth muscle arising from the glomus body. Although
glomus tumors may mimic carcinoid tumors histologi-
cally, immunohistochemical stains readily differentiate
between these entities. Glomus tumors are more common
in men than women by a ratio of 2:1 to 7:1. These lesions
are classified based on biologic activity, and most are
benign. The World Health Organization has divided them
into benign glomus tumors, malignant glomus tumors,
and glomus tumors of uncertain malignant potential.
On MDCT, the most common imaging feature is a
polypoid nodule or mass in the trachea that arises from
the airway wall, usually the posterior wall of the lower
two-thirds of the trachea (Fig. 17.20). Tumor may dem-
onstrate marked enhancement on contrast-enhanced CT
and mimic vascular tumors such as carcinoid and
hypervascular metastases.
Myofibroblastic Tumor
Myofibroblastic tumor is an uncommon primary tumor
that usually affects the lungs but has been described in
the airways. It is known by a number of other names
such as inflammatory pseudotumor and plasma cell
granuloma and typically affects children and young
adults. Histologically, these tumors are composed of
myofibroblastic spindle cells, an inflammatory infiltrate
of plasma cells, lymphocytes, and eosinophils. It has low
to intermediate malignant potential, and the prognosis
is excellent in the setting of complete tumor resection.
278 SECTION 4  Entities by Pathologic Category

A B
Figure 17.20  Glomus tumor of the trachea. (A) Collimated contrast-enhanced axial CT scan demonstrates
a large soft tissue mass (arrow) arising from the trachea. (B) Collimated axial fused PET/CT of the same
patient shows increased FDG uptake in the peripheral aspect of the lesion (arrow). Biopsy revealed primary
glomus tumor.

On MDCT, these lesions manifest as well-circumscribed
polypoid endotracheal or endobronchial soft tissue
nodules or masses.
■  Conclusion
Worldwide, lung cancer remains the leading cause of
cancer-related mortality. Therefore it is imperative that
the radiologist understand specific concepts regarding
lung cancer, from imaging manifestations based on tumor
subtype to overall staging, to guide appropriate manage-
ment. Although neoplasms of the airways are, in contrast,
relatively uncommon, the radiologist plays an essential
role in identifying and characterizing these lesions to
facilitate further imaging and treatment.
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 CHAPTER 17  Neoplasms of the Lung and Airways 279

Questions 3. In the eighth edition of the TNM staging system

1. What is the most common histologic subtype of (TNM-8), what is the tumor descriptor for complete
lung cancer? lung atelectasis?
A. Small cell A. T1
B. Squamous cell B. T2
C. Adenocarcinoma C. T3
D. Large cell D. T4
Answer: C Answer: B
2. What is the appearance of AAH on MDCT? 4. What is the most common primary malignancy of
A. Solid pulmonary nodule the trachea?
B. Nonsolid (ground-glass) pulmonary nodule A. SCC
C. Part solid pulmonary nodule B. Adenoid cystic carcinoma
D. Consolidation C. Carcinoid
Answer: B D. Lymphoma
Answer: A

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