Primary pulmonary neoplasm

PRIMARY PULMONARYPRIMARY PULMONARY
NEOPLASMNEOPLASM
By:-
Dr Vineet Srivastava

 EpidemiologyEpidemiology
 A common malignancy.
 Incidence : in men decreasing
(102 per 100,000 in 1984 & 73.6 in 2004).
-in women increasing since 1960s; rate slowed in
1990s , now approaching a plateau
(50.2 per 100,000 in 2004).
 Annual mortality rate : in men - decreased since 1990s by
approximately 1.9% annually,
: in women - approaching a plateau
after increasing for several decades.
 Remains one of the leading causes of cancer-related deaths in
men in India and both men and women in the United States.

 CausesCauses
 cigarette smoking:cigarette smoking: strongest risk factor (attributable to up to 90% of lung
cancers in men and 80% in women)
- The risk directly proportional to duration & number of cigarettes smoked.
- Nearly all cases of squamous cell and small cell carcinoma are related to cigarette
smoking
- Adenocarcinoma is the predominant cell type in non-smokers (although its
association with tobacco smoking is increasing)
 Involuntary smoke exposure (passive smoking):Involuntary smoke exposure (passive smoking): an excess risk of 20% for
women and 30% for men among never-smokers.
 Environmental and occupational exposures:Environmental and occupational exposures: to particulate and chemical
substances. Exposure to the naturally occurring radioactive gas radon, both in
homes and in mines, is the most important risk factor after cigarette smoking.
- Asbestos, arsenic, chloromethyl ethers, chromium, isopropyl oil, mustard gas,
nickel, beryllium, chloroprene, vinyl chloride, and various smelting by-products such
as lead and copper.
 Genetic susceptibility:Genetic susceptibility: an important factor, independent of smoking
- gene mutations, such as TP53 and KRAS, are common in lung cancer,
- strong association between KRAS mutations in adenocarcinoma
- mutations of the epidermal growth factor receptor gene appear to have a strong
association with adenocarcinoma (particularly BAC subtype)

 Histologic ClassificationHistologic Classification
 Two major histologic categories:
i) non–small cell lung carcinoma (NSCLC)
ii) small cell lung carcinoma (SCLC)
 NSCLC subdivided into histologic types (such as squamous
cell, adenocarcinoma, and large cell), according to the most
differentiated portion of the tumor.
 Many tumors composed of more than one type, definitive
diagnosis requires a resected specimen.
 NSCLC also graded as well, moderately or poorly
differentiated, according to the least differentiated feature.
 Additionally, tumors with immunohistochemical or
ultrastructural features of neuroendocrine differentiation are
collectively referred as NSCLC with neuroendocrine
differentiation.

 Histologic Classification of Lung TumorsHistologic Classification of Lung Tumors
(By WHO)
i) Epithelial Tumors
ii) Lymphoproliferative Tumors
iii) Miscellaneous Tumors

 Histologic Classification of Lung TumorsHistologic Classification of Lung Tumors
(By WHO)
i) Epithelial Tumors
A) Benign
Papilloma
Adenoma
B) Malignant
Preinvasive lesions
- Squamous carcinoma in situ
- Atypical adenomatous hyperplasia
- Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
Non small cell carcinoma
Squamous cell carcinoma
- Variants: Papillary, Clear cell, Small cell, Basaloid
Adenocarcinoma
- Variants: Acinar, Papillary, Bronchioloalveolar, Mixed
subtype
Solid adenocarcinoma with mucin production

Large cell carcinoma
- Variants: large cell neuroendocrine, basaloid,
lymphoepithelioma-
like, clear cell, large cell with rhabdoid phenotype
Adenosquamous carcinoma
Small cell carcinoma
- Variant: combined small cell carcinoma
Sarcomatoid carcinoma
- Variants: pleomorphic, spindle cell, giant cell, carcinosarcoma,
pulmonary blastoma (pleomorphic carcinoma, spindle
cell carcinoma, giant cell carcinoma)
Carcinoid tumor
- Typical carcinoid
- Atypical carcinoid
Salivary gland tumors
- Mucoepidermoid carcinoma
- Adenoid cystic carcinoma
- Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

Mesenchymal tumors
- Epithelioid hemangioendothelioma
- Angiosarcoma
- Pleuropulmonary blastoma
- Chondroma
- Congenital peribronchial myofibroblastic tumor
- Diffuse pulmonary lymphangiomatosis
- Inflammatory myofibroblastic tumor
- Lymphangioleiomyomatosis
- Synovial sarcoma
- Pulmonary artery or vein sarcoma
ii) Lymphoproliferative Tumors
Marginal zone B-cell lymphoma of the mucosa-
associated lymphoid tissue (MALT)
Diffuse large B-cell lymphoma
Lymphomatoid granulomatosis
Langerhans cell histiocytosis

iii) Miscellaneous Tumors (Benign)
Hamartoma
Granular cell tumor
Sclerosing hemangioma
Clear cell tumor

 Atypical AdenomatousAtypical Adenomatous
HyperplasiaHyperplasia Considered a precursor of adenocarcinoma
 Localized proliferation of atypical cells lining alveoli and
respiratory bronchioles
 Typically less than 5 mm in diameter
 Located peripherally in the lung
 Most frequently diagnosed in patients with primary non–small cell
carcinoma, especially adenocarcinoma.
 Not significantly correlated with gender, age, smoking status,
familial history of malignancy, or preceding malignancy.
 Radiologically: AAH manifests as well-circumscribed solitary or
multifocal ground-glass nodular opacities (few mm to 2 cm)
 Tend to remain stable for several months to years.
 Because of similar radiologic manifestations, CT differentiation
between AAH and adenocarcinoma is difficult, and resection is
usually required for a definitive diagnosis.

 (DIPNECH) Diffuse Idiopathic Pulmonary(DIPNECH) Diffuse Idiopathic Pulmonary
Neuroendocrine Cell HyperplasiaNeuroendocrine Cell Hyperplasia
 Widespread proliferation of pulmonary neuroendocrine cells or
neuroendocrine bodies within the epithelium of the distal bronchi or
terminal bronchioles.
 Uncommon, may be confined to the bronchial and bronchiolar
epithelium, or may extend beyond the basement membrane forming
small localized or diffuse aggregates called tumorlets (<0.5 cm in
diameter) or carcinoids (>0.5 cm).
 Fibrous obliterative bronchiolitis and peribronchiolar fibrosis of the
involved airways in 1/3rd
patients.
 More frequently in women, mostly in fifth or sixth decade
 Typical history of the insidious onset of progressive dyspnea and
chronic nonproductive cough.
 Radiologically: Bronchial wall thickening and a mosaic attenuation
pattern (ground-glass opacities and hyperlucent areas) due to
air-trapping. Solitary or multiple nodules (2 to 20 mm in dia.) are an
associated feature.

• Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia in a 63-year-
old woman with a chronic history of cough and dyspnea on minimal exertion.
• CT scan shows multiple small bilateral pulmonary nodules (arrows).
• Wedge resection biopsy of the middle lobe revealed carcinoid tumor (5 mm)
and multiple carcinoid tumorlets (<5 mm).

 Squamous Cell CarcinomaSquamous Cell Carcinoma
 Decreasing in incidence, 30% of all lung cancers.
 Typically occur in central bronchi and frequently manifest as
post obstructive pneumonia or atelectasis.
 Mucoid impaction, bronchiectasis, and hyperinflation are
uncommon radiologic manifestations.
 1/3rd
occur beyond the segmental bronchi and usually range in
size from 1 to 10 cm
 Radiologically: More likely to cavitate than the other
histologic cell types of lung cancer. (10% to 30%, commonly in
large peripheral masses and poorly differentiated tumors)
 Cavitation is typically eccentric, with thick, irregular
walls, although thin walls may occur in rare circumstances.
 Most squamous cell carcinomas grow slowly, and
extrathoracic metastases tend to occur late
 NON SMALL CELL LUNG CARCINOMANON SMALL CELL LUNG CARCINOMA

A, PA chest radiograph showing
complete atelectasis of the left
upper lobe. Convexity in the lower
portion of the atelectatic lung
(arrow) is the result of a central
mass
B, CT scan confirms an endobronchial
mass (M) that occludes the left upper
lobe bronchus and causes complete
atelectasis of the left upper lobe. Note
the enlarged subcarinal node
(asterisk) due to metastasis
 Squamous cell lung cancer manifesting as a central endobronchial
mass

A, PA chest radiograph shows a
lobular mass in the right lower
lobe and infrahilar and para
tracheal adenopathy (arrows).
B, CT scan confirms the lobular
mass in the right lower lobe and
infrahilar adenopathy (arrow).
Focal low attenuation within
the mass is consistent with
necrosis.
 Squamous cell lung cancer manifesting as a peripheral
mass

 AdenocarcinomaAdenocarcinoma
 Replaced squamous cell carcinoma as the most frequent cell type, (50% of all lung
cancers).
 Typically manifest as peripheral, solitary pulmonary nodules.
 Earlier, nodules were typically described having soft tissue attenuation and an
irregular or spiculated margin as a result of parenchymal invasion and an associated
fibrotic response
 Now, adenocarcinomas with purely ground-glass, purely solid, or mixed
(ground-glass and solid) attenuation are being detected more often (due to increased
use of CT and screening for lung cancer).
 Noguchi classification (based on tumour growth patterns):
- type A: localized bronchioloalveolar cell carcinoma (BAC)
- type B: localized BAC with foci of structural collapse of alveoli
- type C: localized BAC with active fibroblastic proliferation
- type D: poorly differentiated adenocarcinoma
- type E: tubular adenocarcinoma
- type F: papillary adenocarcinoma with a compressive growth pattern
 Ground-glass attenuation of nodular opacities is more frequent in types A to C
 Soft tissue attenuation is more frequent in types B to F
 Likelihood of invasive adenocarcinoma and a more advanced stage of lung cancer
is greater with mixed and solid opacities

•CT scan shows a nodule in the right upper lobe (arrow).
•The spiculated margin is typical of lung cancer.
•Note the diffuse emphysema
 Adenocarcinoma manifesting as a pulmonary nodule

 LYMPHANGITIC CARCINOMATOSISLYMPHANGITIC CARCINOMATOSIS :
 Although uncommon at presentation, occurs more frequently
with adenocarcinoma and typically manifests radiologically as
thickening of interlobular septa or multiple small
pulmonary nodules.
 Intrathoracic metastases to hilar and mediastinal nodes are
present more often with more centrally located
adenocarcinomas
A & B : Thin-section CT scans show thickening of the bronchial walls
and interlobular septa (arrowheads) in the left lung. Nodularity of the
septa is suggestive of malignancy.

 BRONCHIOLOALVEOLAR CELL CARCINOMABRONCHIOLOALVEOLAR CELL CARCINOMA
(BAC)(BAC):
 It is uncommon (<4% of all lung cancers), However, incidence
has increased over the past 2 decades
 younger age distribution, higher incidence in never-smokers
and women, and typically more indolent course.
 A diverse group of malignancies that share the pathologic
feature of lepidic growth - spread along alveolar septa
without associated lung destruction.
 In addition, there is absence of stromal, vascular, or
pleural invasion.
 Presentation can be either solitary or multifocal as origin of
BAC is either monoclonal (with multifocality due to
dissemination by aerosolization, intrapulmonary lymphatics, and
intra-alveolar growth) or polyclonal (with multifocality due to
de novo tumor growth at multiple sites

 Manifestations – a solitary, peripheral pulmonary nodule (43%)
– air space disease (30%),
– and multiple nodules (27%)
(a combination of manifestations may be present in a single patient)
 Solitary pulmonary nodules - typically located peripherally, can remain
stable in size for many years.
 Morphologically, BAC nodules can be purely ground-glass, mixed (ground-
glass and solid), or solid in attenuation on CT which correspond to biologic
activity : low activity for ground-glass attenuation
invasive for solid opacities
 Most pure BAC nodules are mixed with <30% of solid component (shows
collapsed alveoli.
 Cystic disease or cavitation is uncommon (<7%) but occurrence of multiple
small, focal, low-attenuation regions (pseudocavitation) or air-bronchograms
within the nodule is occasionally useful in suggesting the diagnosis.
 The diffuse form may present as multiple nodules (usually small, typically
ground-glass in attenuation, occasionally cavitary), ground-glass opacities, or
opacities resembling pneumonia (Mostly combination of these).
 Hilar and mediastinal adenopathy and pleural effusions are uncommon

•CT scan shows a well-marginated ground-glass opacity in the left upper
lobe (arrow).
 Bronchioloalveolar cell carcinoma (BAC) manifesting
as
solitary pulmonary nodule

•CT scan shows small pulmonary nodules and ground-glass opacities
(Cavitary nodules with central areas of cavitation are occasionally
referred to as the “Cheerio” sign)
 Bronchioloalveolar cell carcinoma manifesting as
multiple pulmonary nodules

 Bronchioloalveolar cell carcinoma opacities mimicking pneumonia
A, PA chest radiograph shows a poorly
marginated, homogeneous,
consolidative opacity in the right
lower lobe.
B, CT scan confirms right lower
lobe consolidation and reveals
ground-glass opacities in the
right lower lobe. CT scan also
revealed diffuse ground-glass
opacities in the middle lobe (not
shown).

CT scan shows an opacity in the right upper lobe that resembles
pneumonia, as well as numerous small solid and ground-glass nodules in
the right lung
 Bronchioloalveolar cell carcinoma manifesting as nodules
and consolidation.

 Large Cell CarcinomaLarge Cell Carcinoma
10% to 20% of all lung cancers.
Most are peripheral, poorly marginated masses (>7
cm) in size.
Typically rapid growth
cavitation is uncommon.
Hilar and mediastinal adenopathy occurs in up to one
third of patients at presentation.
Early extrathoracic metastases are common

A, PA chest radiograph shows a
large lobular mass in the right
upper lobe. Cavitation is present,
with an air-fluid level in the upper
aspect of the mass.
B, CT scan shows a well-
circumscribed mass with eccentric
cavitation and thick walls. Note the
nonenlarged paratracheal and left
lower paratracheal lymph nodes
(arrowheads).
 Large cell lung cancer manifesting as a large cavitary mass

 SMALL CELL LUNG CARCINOMASMALL CELL LUNG CARCINOMA
 15% to 20% of all lung cancers.
 1° tumor is typically small & central in location.
 Associated with marked hilar and mediastinal adenopathy
 Distant metastases to liver, bone marrow, adrenals, and
brain
 Pleural effusions occur in 5% to 40% of patients.
 5% of SCLCs manifest as small, peripheral, solitary
pulmonary nodules without intrathoracic adenopathy,
disseminated extrathoracic disease, or pleural effusions

 Small cell lung cancer with
intrathoracic adenopathy
A: PA chest radiograph shows
marked right hilar adenopathy
B: CT scans shows marked right
hilar and subcarinal adenopathy
C: CT scans shows a small primary
malignancy (arrow) in rt. upper lobe

A, PA chest radiograph shows
-a large, poorly marginated right mass
-multicompartmental mediastinal
adenopathy
-obstructive consolidation &
atelectasis of the right lung
B, CT scan confirms
-marked hilar and mediastinal
adenopathy
-marked narrowing of the bronchus
intermedius (asterisk).
-consolidative and atelectatic
opacities distal to the large right hilar
mass
-small right effusion
 Small cell lung cancer manifesting as extensive hilar and
mediastinal adenopathy

 Clinical ManifestationsClinical Manifestations
 Most patients in fifth or sixth decade and symptomatic at presentation.
 Variable symptoms depending upon :-
- Local effects of the primary mass,
- Presence of regional or distant metastases,
- Coexistence of paraneoplastic syndromes.
 Central endobronchial carcinomas: manifest as fever, dyspnea, hemoptysis,
and cough.
 Cough with copious amounts of watery sputum (bronchorrhea) typically in
patients with BAC and extensive parenchymal disease (rare).
 Symptoms as a result of local growth and invasion of adjacent structures:
- Chest pain (peribronchial nerve involvement);
- vocal cord paralysis and hoarseness (recurrent laryngeal nerve involvement)
- dyspnea due to diaphragmatic paralysis (phrenic nerve involvement)
- Horner's syndrome — ptosis, mycosis, anhidrosis (sympathetic chain and
stellate ganglion involvement by superior sulcus tumors)
- Facial swelling, headaches, enlarged collateral chest wall vessels (superior vena
cava obstruction)
- Dysphagia (esophageal invasion)

 Non–small cell lung cancer in a 61-year-old woman presenting
with
hoarseness caused by involvement of the recurrent laryngeal nerve
A, PA chest radiograph shows a left
perihilar mass (arrow).
[Note a small effusion and pneumothorax
following transthoracic needle aspiration
biopsy (arrowheads)]
B, CT scan reveals mediastinal
invasion, with extension of the
mass into the aortopulmonary
window. Arrows show the mass in
the recurrent laryngeal nerve.
(Note the small pleural effusion)

PA chest radiograph shows a large lobular mass in the left lower lobe and
elevation of the left hemidiaphragm
 Small cell lung cancer in a 50-year-old woman with
diaphragmatic paralysis resulting from phrenic nerve
involvement.

 Non–small cell lung cancer in a 54-year-old man with a superior
sulcus (Pancoast) tumor and a 2-month history of neck pain
B, CT scan confirms the left
superior sulcus tumor
and destruction of the left
first rib
A, PA chest radiograph shows a left
apical mass, with destruction of
the first rib (arrowheads)

 Symptoms related to extrathoracic metastases are mostly bone pain
or central nervous system (CNS) abnormalities.
 Paraneoplastic syndromes occur in 10% to 20% of lung cancer patients
and are usually associated with SCLC.
 Antidiuretic and adrenocorticotropic hormones are the more
frequently excreted hormones resulting in hyponatremia and in
Cushing's syndrome respectively.
 Other hormones that may be elevated are calcitonin, growth hormone,
human chorionic gonadotropin, and, rarely, prolactin and serotonin.
 Neurologic paraneoplastic syndromes (Lambert-Eaton myasthenic
syndrome, paraneoplastic cerebellar degeneration, paraneoplastic
encephalomyelitis, paraneoplastic sensory neuropathy) are rare and are
usually associated with SCLC.
 The neurologic symptoms typically precede the diagnosis of lung
cancer by up to 2 years & progress rapidly.
 Miscellaneous paraneoplastic syndromes associated with lung cancer
include acanthosis nigricans, dermatomyositis, disseminated
intravascular coagulation, and hypertrophic pulmonary
osteoarthropathy (most common, upto 17%).

 Non–small cell lung cancer in an asymptomatic 64-year-old woman
A, CT scan shows a large right upper lobe mass
B, Technetium 99m–labeled methylene diphosphonate
bone scintigram shows linear areas of increased
radiotracer uptake in the femurs and tibias. This
appearance is characteristic of hypertrophic
pulmonary osteoarthropathy.

 DiagnosisDiagnosis
 Because most lung cancer have advanced disease at presentation, diagnosis is
usually not difficult.
 Nevertheless, 20% to 30% of patients with lung cancer present with a
solitary pulmonary nodule that may be difficult to differentiate from a benign
nodule.
 Certain morphologic and physiologic features, however, suggest a diagnosis
of lung cancer :-
 Size:-Size:-
 The larger the nodule, the more likely it is to be malignant.
 Small size, does not exclude lung cancer (up to 42% of resected lung cancers
< 2 cm)
 Margins:-Margins:-
 Malignant typically have irregular or spiculated margins
 Benign nodules typically have smooth margins.
(however, presence of one does not exclude the other)

 Internal Morphology.Internal Morphology.
 Fat ( 40 to 120 [HU]) and− − calcification, only features that are reliable in
distinguishing lung cancer from a benign nodule.
 Calcification in malignant lesions is typically amorphous in appearance (found in
up to 14% of lung cancers ).
 Calcification in benign lesions is diffuse, solid, central punctate, laminated,
or popcorn-like.
 Likelihood of malignancy varies according to the degree of soft tissue attenuation.
- partially solid nodular opacities (63%),
- ground-glass opacities (18%)
- solid nodules (7%)
 Cavitation occurs in benign nodules and lung cancer.
Malignant nodules : typically thick, irregular walls,
Benign nodules : smooth, thin walls.
 GrowthGrowth.
 Lung cancers typically double in volume between 30 and 400 days (avg. 240 days)
 The measurement of serial volumes, rather than diameters, and the computer-
calculated doubling of volume of small nodules have been suggested as accurate and
potentially useful methods to assess growth

 Non–small cell lung cancer manifesting as a calcified mass
CT scan shows a large right upper lobe mass with mediastinal invasion.
Scattered areas of amorphous calcification in the mass are typical of,
but not diagnostic for, malignancy

Low-Risk Populations (Little or No History of Smoking and NoLow-Risk Populations (Little or No History of Smoking and No
Other Risk Factors)Other Risk Factors)
Nodular size reassessment If stable If stable
≤4 mm not required
>4 mm but ≤6 mm CT at 12 months No further evaluation
>6 mm but ≤8 mm CT at 6 to 12 months CT at 18 to 24 months No further evaluation
>8 mm CT scans at 3, 9, and 24 months or further evaluation with contrast-
enhanced CT, CT PET, biopsy, or resection
High-Risk Populations (History of Smoking or Other Exposure orHigh-Risk Populations (History of Smoking or Other Exposure or
Risk Factor)Risk Factor)
Nodular size reassessment If stable If stable
≤4 mm CT at 12 months No further evaluation
>4 mm but ≤6 mm CT at 6 to 12 months CT at 18 to 24 months No further evaluation
>6 mm but ≤8 mm CT at 3 to 6 months CT at 9 to 12 months again 24 months
>8 mm CT scans at 3, 9, and 24 months or further evaluation with contrast-
enhanced CT, CT PET, biopsy, or resection
 Fleischner Society Guidelines for the Evaluation ofFleischner Society Guidelines for the Evaluation of
Incidentally Discovered NodulesIncidentally Discovered Nodules

 Blood SupplyBlood Supply
 qualitatively and quantitatively different
 In CECT - malignant nodules enhance > 20 HU,
benign nodules enhance <15 HU
A, NCCT scan shows a left lung
nodule with an attenuation value of
24 Hounsfield units (HU).
B, CECT scan shows nodule
enhancement of 70 HU and central
necrosis.
* Resection revealed non–small cell cancer

 MetabolismMetabolism
 Metabolism of glucose is typically increased in lung cancer cells
 FDG (fluorodeoxyglucose ) uptake can be assessed visually on PET images by comparing the
activity of the lesion with that of the background.
 FDG uptake is quantified by using standardized uptake value (SUV) or local metabolic rate of
glucose (Ki = influx constant).
 SUV > 2.5 is considered indicative of malignancy.
 The probability of malignancy is high when a solid nodule ≥ 1 cm shows increased FDG uptake
 These nodules should be biopsied or resected and thus FDG PET can reduce the number of
benign nodules resected.
A, CT scan shows a spiculated nodule
in the right upper lobe. The irregular
margin suggests malignancy.
B, Axial fused CT PET image with FDG
shows increased uptake within the nodule
compared with the adjacent mediastinum.
These findings are suspicious for
malignancy.
* Transthoracic needle aspiration biopsy revealed adenocarcinoma

 International Staging System for Lung CancerInternational Staging System for Lung Cancer
Primary Tumor (T)Primary Tumor (T)
TxTx Primary tumor cannot be assessed, or tumor proved by the presence of malignant cells in sputum or bronchial washings
but not visualized by imaging or bronchoscopy
T0T0 No evidence of primary tumor
TisTis Carcinoma in situ
T1T1 Tumor ≤3 cm in greatest dimension, surrounded by lung or visceral pleura, without bronchoscopic evidence of invasion
more proximal than the lobar bronchus[*]
(i.e., not in the main bronchus)
T2T2 Tumor with any of the following features of size or extent: >3 cm in greatest dimension; Involves main bronchus, ≥2 cm
distal to the carina; Invades the visceral pleura; Associated with atelectasis or obstructive pneumonitis that extends to
the hilar region but does not involve the entire lung
T3T3 Tumor of any size that directly invades any of the following: chest wall (including superior sulcus tumors), diaphragm,
mediastinal pleura, or parietal pericardium; or tumor in the main bronchus <2 cm distal to the carina, but without
involvement of the carina; or associated atelectasis or obstructive pneumonitis of the entire lung
T4T4 Tumor of any size that invades any of the following: mediastinum, heart, great vessels, trachea, esophagus, vertebral
body, or carina; or tumor with a malignant pleural or pericardial effusion[†]
or with satellite tumor nodule(s) within the
ipsilateral primary-tumor lobe of the lung
Regional Lymph Nodes (N)Regional Lymph Nodes (N)
NxNx Regional lymph nodes cannot be assessed
N0N0 No evidence of regional lymph node metastasis
N1N1 Metastasis to : ipsilateral peribronchial and/or ipsilateral hilar lymph nodes
: intrapulmonary nodes involved by direct extension of the primary tumor
N2N2 Metastasis to : ipsilateral mediastinal and/or subcarinal lymph node(s)
N3N3 Metastasis to : contralateral mediastinal or hilar lymph node(s)
: ipsilateral or contralateral scalene, or supraclavicular lymph node(s)
Distant Metastasis (M)Distant Metastasis (M)
MxMx Presence of distant metastasis cannot be assessed
M0M0 No distant metastasis
M1M1 Distant metastasis present[‡]
* Superficial tumor of any size limited to the bronchial wall, is also classified as T1.
† In a few patients, cytopathologic examinations of pleural/pericardial fluid show no tumor, is nonbloody and non-exudative. Then it should be
excluded as a staging element, and the patient's disease should be staged T1, T2, or T3.
‡ Separate metastatic tumor nodule(s) in the ipsilateral nonprimary-tumor lobe(s) of the lung are also classified M1.

 Primary Tumor (T Status)Primary Tumor (T Status)
 Imaging is performed to assess the degree of pleural, chest wall, and mediastinal
invasion.
 CT: used for gross chest wall invasion (inaccurate in differentiating between
anatomic contiguity and subtle invasion)
 Findings suggestive of chest wall invasion include :
•  Tumor-pleura contact extending over > 3 cm
•  Obtuse angle at the tumor-pleura interface
   • Thickening of the pleura or increased attenuation of the extrapleural fat
adjacent to the tumor
A: PA chest radiograph shows a large
cavitary right lower lobe lung mass
(arrows) and destruction of the posterior
aspect of the right seventh rib (arrowheads).
B: CT scan confirms the cavitary mass (M)
and destruction of the adjacent rib (asterisk).
Note the extension of the mass into the
chest wall (arrowheads)

 Although MRI offers superior soft tissue contrast resolution, its sensitivity
and specificity in identifying chest wall invasion are similar to that of CT.
 MRI is particularly helpful in evaluating superior sulcus (Pancoast) tumors and to
assess invasion of the brachial plexus, subclavian vessels, and vertebral bodies.
A, CT scan shows a large, well-
circumscribed peripheral lung mass
with invasion of the chest wall
B, Coronal fast-spin echo MRI
shows the mass abutting the chest
wall and the surrounding ribs
(asterisk) and extending through
the intercostal space into the soft
tissues of the chest wall (arrow).
Coronal T1-weighted image shows a mass (M) in the
apex of the right hemithorax, with invasion into
the neck. The mass surrounds the subclavian
artery (asterisk) and the brachial plexus. (R, first

 Findings suggestive of subtle mediastinal invasion include :
•   Tumor-mediastinum contact extending over > 3 cm
   •   Obliteration of the fat plane between the mediastinum and the tumor
   •    Tumor contacting > 90° of the aortic circumference
CT scan shows a large left upper lobe mass,
with invasion of the anterior mediastinum.
Broad abutment and loss of the soft tissue
plane between the mass and the transverse
aorta suggest vascular invasion
Double inversion recovery, contrast-enhanced,
single breath-hold coronal MRI shows an
enhancing right upper lobe mass (M) that
extends via the right superior pulmonary vein
into the left atrium (LA).

 Primary lesions associated with malignant pleural effusions or pleural
metastases are classified as T4 lesions.
 Up to 33% of NSCLC show pleural metastases at presentation.
 Pleural thickening and nodularity on CT scans suggest metastatic pleural
disease.
Contrast-enhanced CT scan shows a large right pleural effusion and enhancing nodular
pleural metastases (arrowheads). Arrow indicates compressive atelectasis of the right lower
lobe and a metastatic left rib lesion

 To enable a consistent and standardized description of N status, nodal
stations are defined by the American Thoracic Society in relation to
anatomic structures or boundaries that can be identified before and during
thoracotomy.
 The IASLC has proposed six zones within the current N1 and N2 patient
subsets for further evaluation.
 The zones consist of
- peripheral (stations 12, 13, 14)
- hilar (stations 10, 11)
&
- upper mediastinal (stations 1, 2, 3, 4)
- lower mediastinal (stations 8, 9)
- aortopulmonary (stations 5, 6)
- subcarinal (station 7)
 Regional Lymph Nodes (N Status)Regional Lymph Nodes (N Status)
N1
N2

 Nodal stationsNodal stations

 NSCLC commonly show extrathoracic metastases to the adrenal glands, liver, brain,
bones, and lymph nodes at presentation.
 FDG PET - higher sensitivity and specificity than CT
 Adrenal glands metastases : Common, detected in up to 20% of patients at presentation.
 Features favouring malignancy :
   • Size > 3 cm
   • Poorly defined margins
   • Irregularly enhancing rim
   • Invasion of adjacent structures
   • High signal intensity on T2
 Metastatic Disease (M Status)Metastatic Disease (M Status)
A: CT scan shows a nodule in the left lower lobe.
There is no hilar or mediastinal adenopathy
B: CT scan of the abdomen reveals a left adrenal
mass (arrow). Biopsy confirmed metastasis

 CNS metastases –
 Detected in up to 18 % of patients at presentation.
 Routine CT of the brain suggested in the initial staging evaluation of all patients with
NSCLC
 Not recommended as : CNS metastases usually associated with neurologic signs and
symptoms & not cost-effective.
 ASCO recommends brain CT or MRI for asymptomatic patients in stage III being
considered for aggressive local therapy (viz. thoracic surgery or radiation).
A: CT scan shows a 3.5 cm
spiculated left upper lobe mass.
B: Axial contrast-enhanced cranial MR image shows
an enhancing metastasis in the cerebellum (arrow).

 Skeletal metastases :
 Usually symptomatic or have laboratory abnormalities indicating bone
metastases.
 Bone radiographs, MRI, and bone scintigraphy : performed to evaluate h/o focal
bone pain or elevated alkaline phosphatase.
 FDG PET help in detecting occult osseous metastases.
A: PA chest radiograph
shows a right upper lobe
mass. There is a small right
pleural effusion.
B: CT scan reveals a small right
adrenal mass (arrow) and confirms a
small right pleural effusion.
C, Whole-body FDG PET shows increased uptake in the primary lung mass (small arrow) and in the
adrenal metastasis (arrowhead). Focal areas of increased uptake in the upper aspect of the left
hemithorax and pelvis (large arrows) were unsuspected bone metastases.

 Stage Grouping: Tumor-Node-MetastasesStage Grouping: Tumor-Node-Metastases
(TNM) Subsets(TNM) Subsets
Stage
TNM Subset
T N M
IA T1 N0 M0
IB T2 N0 M0
IIA
T1 N1 M0
T2 N1 M0
IIB T3 N0 M0
IIIA
T1 N2 M0
T2 N2 M0
T3 N1 M0
T3 N2 M0
IIIB
T4 N0 M0
T4 N1 M0
T4 N2 M0
T1 N3 M0
T2 N3 M0
T3 N3 M0
T4 N3 M0
IV Any T Any N M1

 Staging of Small Cell Lung CarcinomaStaging of Small Cell Lung Carcinoma
 SCLC staged according to Veterans Administration Lung Cancer Study Group (VALCG)
recommendations :
 i)i) Limited disease (LD)Limited disease (LD) :: confined to a hemithorax and the regional lymph nodes.
Unlike TNM classification for NSCLC, metastases to both ipsilateral & contralateral
supraclavicular and mediastinal lymph nodes are considered local disease.
 ii)ii) Extensive disease (ED)Extensive disease (ED) :: includes noncontiguous metastases to the contralateral lung
and distant metastases.
 Most patients present with ED.
 Common sites of metastasis: liver (30%), bone (30%), bone marrow (17 to 34%), brain (10
to 27%), and retroperitoneal lymph nodes (11%).
 Evaluation includes bone marrow aspiration, bone scintigraphy and MRI of the brain and
abdomen.
A: PA chest radiograph shows a lobular
3-cm right upper lobe nodule (arrow)
and hilar adenopathy (arrowhead).
B: CT scan shows a left
adrenal metastasis
(arrow).
C, Axial contrast-enhanced cranial MR
image shows an enhancing cerebellar
metastasis (arrow).

 Contains carcinomatous and sarcomatous components.
 Includes pulmonary blastoma, carcinosarcoma, and carcinomas with spindle
or giant cells.
 Rare - 0.3% to 1%
 Most patients are men, mean age at presentation :65 yrs (range, 44 to 78 yrs).
 Usually localized at presentation, distant metastases occur frequently, prognosis is
poor.
 Mostly present with cough, dyspnea, hemoptysis, chest pain, or weight loss.
 Radiologically manifest as either
- large peripheral masses or
- polypoid endobronchial lesions with atelectasis or
- postobstructive pneumonia.
 Calcification and cavitation are uncommon
 Necrosis and hemorrhage can manifest as heterogeneous attenuation on CT.
 Hilar or mediastinal adenopathy is uncommon.
 Pleural effusion can occur as a result of local invasion.
 Metastases involve sites similar to those associated with lung cancer: lungs, liver,
bones, adrenals, brain
 Sarcomatoid CarcinomaSarcomatoid Carcinoma

 Rare, 0.25% to 0.5% of primary lung tumors.
 Named so for histologic resemblance to fetal lung tissue.
 Thought to arise from primitive pluripotential stem cells and may represent a
variant of carcinosarcoma.
 Mainly in adults, peak incidence between 40 and 60 yrs of age.
 Often symptomatic at presentation; cough, hemoptysis, and chest pain are
frequent manifestations.
 Pediatric patients typically present with fever and respiratory distress.
 Aggressive with poor prognosis owing to frequent relapses and metastases.
 Radiologically: Typically manifest as
- large (2.5 to 26 cm),
- well-marginated masses
- located peripherally in the lung
 Multiple masses, cavitation, and calcification are rare.
 Local invasion : mediastinum (8%) and pleura (25%)
 Metastases to hilar and mediastinal lymph nodes (30%)
 Extrathoracic metastases common with distribution similar to that of lung cancer
 Pulmonary BlastomaPulmonary Blastoma

 Pulmonary blastoma manifesting as a large pulmonary
mass
A: PA chest radiograph shows complete
homogeneous opacification of the left
hemithorax and displacement of the
mediastinum to the right.
B: CT scan reveals a large lung mass and
shows heterogeneous attenuation
consistent with necrosis. There is
subcarinal adenopathy due to
metastatic disease (asterisk). Note the
complete compressive atelectasis of the
left lung.

 Low-grade malignancies, constitute 1% to 2% of primary lung tumors.
 Classified histologically depending on the degree of cellular atypia as :
i) Typical (80% to 90%) or ii) Atypical (10% to 20%)
 TypicalTypical : equal frequency in men and women;
- Mean age at diagnosis is 35 to 50 years.
- Usually arise in lobar, segmental, or proximal subsegmental bronchi
- generally measure 1 to 4 cm
- Rarely metastasize to regional nodes or beyond the thorax
 AtypicalAtypical : slightly older age (mean, 53 to 60 years),
- Often larger
- Occur more frequently in the peripheral aspect of the lungs.
-More aggressive, frequent metastasis to regional nodes, lung, liver &
bone. N status most important prognostic factor.
 Clinical manifestations depend on histologic type and location
 Peripheral tumors usually asymptomatic, whereas central one manifest as
cough, hemoptysis, or recurrent infection.
 Paraneoplastic manifestations, viz. carcinoid syndrome and Cushing's syndrome,
are rare and more common with atypical carcinoid tumors.
 Carcinoid TumorCarcinoid Tumor

 Radiologically : carcinoid tumors manifest most commonly as central
endobronchial masses, with or without atelectasis or consolidation.
A peripheral, well marginated pulmonary
nodule is a less common manifestation.
A: PA chest radiograph shows complete
atelectasis of the right lower lobe
(arrowheads), with compensatory
hyperinflation of the left lung. Note the
displacement of the anterior junction line
(arrows).
B: CT scan confirms atelectasis of the right
lower lobe and reveals an endobronchial
mass (M), with marked narrowing of the
bronchus intermedius (arrow)
CT scan shows a small, well-circumscribed nodule
in the left lower lobe (arrow).

 The tumors are usually < 3 cm, although occasionally they may be as large as 10
cm in diameter.
 Calcification is detected by CT in approximately 25% of carcinoid tumors
 Hilar and mediastinal adenopathy and extrathoracic metastases are
uncommon at presentation in patients with typical carcinoid tumors; they occur
CT scan shows a large
endobronchial mass with
dense punctate calcification

 Formerly categorized as bronchial adenoma
 A rare tracheobronchial tumor composed of distinct areas of
squamoid cells, mucus-secreting cells, and cells of
intermediate type.
 More common in adults, peak incidence between 35 and 45 yrs.
 Typically occur in the main or lobar bronchi
 Rarely may be located in the trachea and periphery of the lung.
 Slow-growing, low-grade neoplasms with a benign clinical course.
 Occasionally exhibit aggressive local behavior,
 Metastases are uncommon.
 Radiologically the tumor manifests:
- Usually as a central endobronchial mass
- less commonly, as a polypoid intraluminal tracheal mass
or a peripheral lung nodule or mass
 Mucoepidermoid CarcinomaMucoepidermoid Carcinoma
 SALIVARY GLAND TUMORSSALIVARY GLAND TUMORS

 Formerly categorized as bronchial adenoma.
 Uncommon 1° tumor of the lung, occurs mainly in the trachea &
main bronchi, only 10%-15% found in peripheral lung field.
 Equal frequency in men and women; the mean age at diagnosis is 45
to 51 years
 Adenoid Cystic Carcinoma (Cylindroma)Adenoid Cystic Carcinoma (Cylindroma)
They typically exhibit slow,
progressive growth.
Metastases to regional lymph
nodes, lung, bone, liver, and brain
occur late.
Radiologically : usually manifests
as an lobulated or an polypoidal
endotracheal or endobronchial mass
encroaching the airway lumen.
Masses can be circumferential
and may manifest as diffuse stenosis.
A less common manifestation is a
peripheral lung nodule or mass.
CT scan shows a circumferential
soft tissue tracheal mass (T) and
narrowing of the trachea

 Most Primary lung sarcomas with a vascular origin eg. Angiosarcomas &
epithelioid hemangioendotheliomas, are lung metastases, and the
existence of primary pulmonary angiosarcoma are rare.
 The Angiosarcomas usually occurs in young adults where as
Epithelioid hemangioendothelioma is typically seen in women
younger than 40 years.
 Most patients are asymptomatic at presentation
 Complaints include weight loss, dyspnea, chest pain, and cough.
 Usual radiologic manifestation is multiple 1 to 2 cm bilateral
pulmonary nodules.
 Calcification is rarely detected.
 Hilar adenopathy and pleural effusions occur in 9% of patients.
 Multiorgan involvement, most commonly the liver, occurs occasionally
and may represent metastatic disease or multicentric origin of the
tumor.
 MESENCHYMAL TUMORSMESENCHYMAL TUMORS
((Primary Pulmonary Sarcomas)Primary Pulmonary Sarcomas)

 Epithelioid hemangioendothelioma appearing as multiple
pulmonary nodules
A: PA chest radiograph shows
numerous small, bilateral pulmonary
nodules. Note the absence of hilar
and mediastinal adenopathy.
B: CT scan confirms the numerous
small, bilateral, well-circumscribed
nodules
C: CT scan of the abdomen reveals small,
bilateral, low-attenuation hepatic epithelioid
hemangioendotheliomas.

 Primary lung sarcomas with a spindle cell origin are rare (< 0.5%)
 Spindle cell sarcomas (malignant fibrous histiocytoma,
hemangiopericytoma, fibrosarcoma, leiomyosarcoma, synovial sarcoma)
are the most common primary pulmonary sarcomas.
 Heterogeneous group of tumors with morphologic similarities to their
extrathoracic counterparts.
 Diagnosis established after excluding metastasis and sarcoma-like primary lung
malignancies (sarcomatoid carcinomas).
 Peak incidence between fifth and sixth decades.
 Usually slow growing with late metastases, prognosis generally better than lung
cancer.
 Radiologically: - Commonly located peripherally
- Lesions range in size from 0.6 to 25 cm
- Sharply marginated and occasionally calcified
- Cavitation is uncommon,
- heterogeneous attenuation from necrosis on CT
-Pathognomonic hypervascularity are seldom seen on CT or
MRI scans of primary pulmonary hemangiopericytomas

 Malignant fibrous histiocytoma manifesting as a large
lung mass.
A: PA chest radiograph shows a left
upper lobe mass
B: CT scan shows a large lobular mass and
narrowing of the left main pulmonary artery
(arrowheads).

 Synovial carcinoma of left lung manifesting as a large
mass
A, CT scan shows a large
mass in the left lower lobe,
with a small focal
calcification (arrow). A small
pleural effusion can also be
seen
B and C, Coronal T1-weighted (B) and fast-spin echo (C)
images show a large heterogeneous mass (M) abutting
the diaphragm, without signs of invasion. There is a small
pleural effusion (P). L, liver

 Hemangiopericytoma appearing as a large hypervascular
mass
A, Contrast-enhanced CT scan shows
a large right lower lobe mass. Large
vessels can be seen within the mass
B and C, Axial T1-weighted (B) and fast-spin echo (C) images show a
heterogeneous mass in the right hemithorax. Flow voids can be seen
within the intratumoral vessels.

 Primary pulmonary lymphomas account for <1% of all lymphomas.
 Diagnosis made if lymphoid proliferation is monoclonal and there are no sites
of extrathoracic lymphoma at presentation or for at least 3 months after
diagnosis.
 1° pulmonary lymphomas encompass non-Hodgkin‘s & Hodgkin's lymphoma,
and lymphoproliferative disorders associated with immunodeficiency states.
 Most primary extranodal lymphomas arise from mucosa-associated
lymphoid tissue (MALT)
 Typically infiltrate the bronchiolar mucosal epithelium, forming
lymphoepithelial lesions.
 Primary pulmonary lymphomas tend to remain localized to the lung
 Recurrence after treatment - up to 50% (often at extrapulmonary sites)
 Radiologic manifestations :
- a solitary nodule or mass - multiple nodules or masses,
- focal or multifocal consolidation - reticulonodular opacities
- atelectasis - Hilar adenopathy (rare)
- pleural effusions (in 7% to 25% )
 LYMPHOPROLIFERATIVE DISORDERS:LYMPHOPROLIFERATIVE DISORDERS:
(Primary Lymphomas)(Primary Lymphomas)

 Primary pulmonary lymphoma manifesting as bilateral
pulmonary opacities
A and B, CT scans show focal, poorly marginated nodular opacities in both lungs. Air-
bronchograms within opacities are suggestive of the diagnosis.

iii) Miscellaneous Tumors
Hamartoma
Granular cell tumor
Sclerosing hemangioma
Clear cell tumor

 Constitute 0.25% of all primary lung tumors
 Most common benign tumor of the lung
 Term hamartoma initially used to describe lesions with abnormal composition or
disorganized arrangement of normal lung tissue.
 Now considered true neoplasms containing a mixture of epithelial and
mesenchymal tissues.
 Typically occur in patients >30 years (peak incidence in the sixth decade)
 Most patients are asymptomatic
 Symptoms are typically present with central endobronchial lesions and
include hemoptysis, recurrent pneumonia, and dyspnea.
 Hamartomas are typically solitary, well-marginated, slightly lobular nodules
or masses < 4 cm
 Most are located peripherally within the lung.
 Endobronchial hamartomas are less common (< 20%)
 Calcification < 5%
 “PopcornPopcorn” calcification pathognomonic of hamartomas.
 Fat occurs in up to 50% of cases and is a diagnostic feature
 Cavitation and multiple pulmonary hamartomas are rare
 HAMARTOMASHAMARTOMAS

 Hamartoma manifesting as a large mass
 Hamartoma appearing as a solitary pulmonary nodule
CT scan shows a left upper lobe nodule
(arrow) anterior to the left pulmonary artery.
The diagnosis is suggested by small areas
of fat (attenuation, 41 Hounsfield units)−
within the nodule
CT scan shows a right lower lobe mass that has
focal calcified regions as well as focal regions
of low attenuation ( 60 Hounsfield units),−
consistent with fat.
The diagnosis is suggested by small areas
of fat within the mass

 Formerly called granular cell myeloblastomas
 Uncommon benign mesenchymal neoplasms, thought to arise from
Schwann cells
 Usually present as a small solitary skin or submucosal nodule.
 Pulmonary granular cell tumors – rare (6 - 10% of all granular cell tumors)
 Patients are usually adults (peak incidence, 30 to 50 years)
 Higher incidence in African Americans.
 Mostly manifest as small (0.3 to 6.5 cm in diameter), central
endobronchial masses.
 Multiple lesions, typically in the larger bronchi, are found in up to 25% of
cases.
 Common radiologic findings include atelectasis and obstructive
pneumonia
 Slow-growing, solitary pulmonary nodules or masses occur in 12% of
cases
 GRANULAR CELL TUMORGRANULAR CELL TUMOR

 Formerly called pneumocytoma or papillary pneumocytoma
 A benign tumor consisting of thin-walled vessels and connective
tissue.
 Originally thought to represent a vascular tumor, now believed to arise
from primitive respiratory epithelium.
 Most patients are asymptomatic women between 30 and 50 years of
age.
 However, cough and hemoptysis can occur in few.
 Radiologically, usually manifest as peripheral, well-marginated,
solitary pulmonary nodule or mass 0.4 to 8 cm in diameter (average,
3 cm).
 Multiple pulmonary nodules and calcification are rare.
 Marked contrast enhancement is typical on CT, and tumors are
typically heterogeneous in attenuation due to the angiomatous,
solid and sclerotic, and cystic composition of the tumor.
 MRI appearance is variable showing both homogeneous and
heterogeneous signal intensity.However, useful in diagnosis when regions
of different signal intensities are present in the tumor.
 SCLEROSING HEMANGIOMASCLEROSING HEMANGIOMA

 A rare neoplasm of the lung.
 Most patients asymptomatic, between 50 and 60 years of age
 Almost all behave in a benign nature, although extrathoracic metastases have been
reported.
 Manifest radiologically as well-marginated, solitary pulmonary nodules < 3
cm in diameter.
 CLEAR CELL TUMORCLEAR CELL TUMOR
CT scan shows a well-circumscribed left upper lobe nodule with heterogeneous
enhancement

Primary pulmonary neoplasm

More Related Content

What's hot

Similar to Primary pulmonary neoplasm

More from Vineet Srivastava

Recently uploaded

Primary pulmonary neoplasm

Editor's Notes