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2020 CPR II Pulm Exam 2 > Lung Path 5 - Pulm Tumors and Pleural Diseases (Singh) > Flashcards

Lung Path 5 - Pulm Tumors and Pleural Diseases (Singh) Flashcards

1
Q

What is more common primary pleural tumors or secondary tumors from metastasis?

A

Secondary from metastasis

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2
Q

The most frequent metastatic malignancies of the pleura arise from which 2 sites?

A

Breast and lung

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3
Q

In most metastatic involvements of the pleura what type of effusion follows?

A

Serous or sero-sangioneous often containing neoplastic cells

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4
Q

Which soft-tissue tumor has a propensity to occur in the pleura is often attached to the surface by a pedicle?

A

Solitary fibrous tumor

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5
Q

Which immunostaining features of solitary fibrous tumors are helpful in distinguishing these tumors from malignant mesotheliomas?

A

CD34+ and keratin-negative

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6
Q

What do solitary fibrous tumors show microscopically?

A

Whorls of reticulin and collagen fibers w/ interspersed spindle cells resembling fibroblasts

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7
Q

Solitary fibrous tumors are highly associated with a cryptic inverion of which chromosome and this creates which fusion gene unique to this tumor?

A

Chromosome 12 —> NAB2-STAT6 fusion gene

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8
Q

Homozygous deletion of which tumor suppressor gene occurs in about 80% of mesothelioma; involves which chromosome?

A

CDKN2A/INK4-α on chromosome 9p

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9
Q

How does malignant mesothelioma appear grossly?

A

Affected lung is ensheathed by a thick layer of soft, gelatinous, grayish-pink tumor tissue

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10
Q

What are the 3 variants of growth that can be seen microscopically with mesothelioma?

A
  • Epithelioid
  • Sarcomatoid
  • Mixed
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11
Q

Epithelioid type of mesothelioma resembles adenocarcinoma; how can it be differentiated via immunohistochemical stains for which 5 markers?

A
  • (+) Keratin
  • (+) Calretinin
  • (+) Wilms tumor 1 (WT-1)
  • (+) Cytokeratin 5/6
  • (+) D2-40
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12
Q

The mesenchymal type of mesothelioma (sarcomatoid type) appears as what type of sarcoma and resembles?

A

Spindle cell sarcoma, resembling fibrosarcoma

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13
Q

Sarcomatoid mesotheliomas may only stain positive for which marker?

A

Keratin

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14
Q

What are the typical presenting features of malignant mesothelioma?

A
  • Chest pain
  • Dyspnea
  • Recurrent pleural effusions**
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15
Q

Malignant mesothelioma often invades lung directly and commonly metastasizes to which LN and organs?

A
  • Hilar LN’s
  • Eventually –> liver and other distant organs
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16
Q

What is the prognosis of malignant mesothelioma?

A

50% die within 12 months; few survive longer than 2 years

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17
Q

What are found in increased numbers in the lungs of patients with mesothelioma?

A

Asbestos bodies and asbestos plaques

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18
Q

What is the major morphological pattern of damage seen with chronic rejection following a lung transplant?

A

Bronchiolitis obliterans –> fibrosis causing partial or complete occlusion of small airways WITH or WITHOUT active inflammation

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20
Q

What age is the peak incidence of lung cancer seen?

A

50-60 y/o

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21
Q

Deletions of which 3 chromosomal loci and 3 genes are seen as early events in the squamous cell carcinoma of the lung

A
  • 3p and 9p (site of CDKN2A gene –> product = p16)
  • -* 17p (site of TP53 gene)
  • -* Loss of RB tumor suppressor
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22
Q

Large areas of “benign” respiratory mucosa being mutagenized by exposure to carcinogens in tobacco smoke is known as what?

A

“Field effect“

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24
Q

Loss of function mutations in which 2 genes and chromosome are seen with small cell carcinoma of the lung?

A
  • TP53
  • RB
  • Chromosome 3p deletions
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25
Q

Amplification of which gene has been associated with squamous cell carcinoma of the lung?

A

FGFR1

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26
Q

Oncogenic gain of function mutations in which 5 RTK genes are associated with Adenocarcinoma of the lung?

Tumors without RTK mutations often have mutation in which gene?

A
  • RTK mutation = EGFR + ALK + ROS + MET and RET
  • Non-RTK = KRAS
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27
Q

Which type of lung cancer shows the strongest association with smoking?

A

Small cell carcinoma

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29
Q

Amplification of which gene is associated with small cell carcinoma of the lung?

A

MYC family

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30
Q

Which precursor lesion is characterized by dysplastic pneumocytes lining alveolar walls that are mildly fibrotic?

A

Atypical adenomatous hyperplasia

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31
Q

Which precursor lesion is composed entirely of dysplatic cells growing confluently along pre-existing alveolar septae; and may or may not hav intracellular mucin?

A

Adenocarcinoma in situ

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33
Q

Which tumor of the lung tends to spread aerogenously, forming satellite tumors and may consolidate an entire lobe mimicking lobar pneumonia?

A

Mucinous ADENOCARCINOMA

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34
Q

What is the characteristic histologly of squamous cell carcinoma?

A

Keratin pearls and/or intracellular bridges

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35
Q

Via cytology which color will the cytoplasm be if keratin is present and is indicative of SCC?

A

Orange cytoplasm

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36
Q

Lung cancers in never smokers are more common in which sex and are most often what type of cancer?

A
  • Women
  • Adenocarcinoma
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37
Q

Lung cancers in never smokers are more likely to have mutations in which gene and never have mutations in what?

A
  • More likely to have EGFR mutations; sometimes TP53
  • NEVER have KRAS mutations
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38
Q

What is the histology of the chromatin, cytoplasm, nucleus, and nucleoli like with small cell carcinoma of the lung?

A
  • Small cells w/ scant cytoplasm
  • Finely granular (BLUE) nuclear chromatin (salt and pepper pattern)
  • Absent nucleoli
  • Necrosis is COMMON and often extensive
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39
Q

Which type of lung cancer is more commonly arising in the peripheral lung and which in the central/hilar lung?

A
  • Peripheral = adenocarcinoma
  • Central/hilar = squamous cell carcinoma
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40
Q

Basophilic staining of vascular walls due to encrustation by DNA from necrotic tumor cells (Azzopardi effect) is common with what lung tumor?

A

Small cell carcinoma

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43
Q

Majority of adenocarcinomas of the lung express which transcription factor required for normal lung development?

A

Thyroid transcription factor-1

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47
Q

Which type of lung tumor is almost always metastatic at time of presentation and is best treated with specific chemotherapies/radiation?

A

Small cell *neuroendocrine* carcinoma

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48
Q

What is the most aggressive lung tumor with no known preinvasive phase?

A

Small cell *neuroendocrine* carcinoma

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50
Q

What is a more ominous change squamous metaplasia or dysplasia?

A

Dysplasia

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52
Q

Which 3 markers are found in the neurosecretory granules of small cell carcinomas of the lungs?

A
  • Chromogranin
  • Synaptophysin
  • CD57
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53
Q

Via immunohistochemistry high levels of which anti-apoptotic protein can be demonstrated in small cell carcinomas of the lung?

A

BCL2

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54
Q

Early distant spread of lung carcinoma generally occurs via which 2 pathways; which carcinoma specifically metastasizes late?

A
  • Lymphatic and hematogenous
  • Squamous cell carcinoma spreads late
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55
Q

What characteristic of Lambert-Eaton Syndrome distinguishes it from Myasthenia Gravis?

A

Sx’s IMPROVE w/ more movement throughout the day

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56
Q

Apical lung cancers (pancoast tumors) in the superior pulmonary sulcus tend to invade which neural structures around the trachea and produce what signs/sx’s?

A
  • Invade cervical sympathetic plexus
  • Severe pain in the distribution of the ulnar nerve
  • Horner syndrome –> enophthalmos + ptosis + miosis + anhidrosis
57
Q

What are the 4 favored sites of metastasis for lung carcinomas?

A
  • Adrenals
  • Liver
  • Brain
  • Bone
58
Q

Which 3 LN’s are common sites of metastasis for lung carcinoma?

A
  • Bronchial
  • Tracheal
  • Mediastinal
59
Q

What may partial or total obstruction caused by a lung carcinoma cause?

A
  • Partial –> focal emphysema
  • Total –> atelectasis
60
Q

Impaired drainage of the airways caused by lung carcinomas is a common cause of what?

A

SEVERE suppurative or ulcerative bronchitis or bronchiectasis

61
Q

What is the main difference between Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH) and Carcinoid tumors?

A
  • SIZE!!!
  • DIPNECH = very small, <5mm —> “Tumorlets”
  • Carcinoid tumor = 5mm or larger
62
Q

Venous congestion of the head and arm w/ circulatory compromise caused by an underlying lung carcinoma is known as what?

A

SVC syndrome

63
Q

Which tumor of the lungs grows as fingerlike or spherical polypoid masses that commonly project into the lumen of the bronchus and may produce so-called collar-button lesions?

A

Carcinoid tumor

64
Q

Which lung tumor is characterized by organoid, trabecular, palisading, ribbon, or rosette-like arrangements of cells w/ uniform round nuclei and abundant cytoplasm separated by a delicate fibrovascular stroma?

A

Carcinoid tumor

65
Q

What are features of atypical carcinoid tumors (neuroendocrine tumor grade 2) which differ from typical carcinoid tumors (grade 1)?

A
  • ↑↑↑ mitotic activity
  • NECROSIS (typical carcinoids lack necrosis)
  • Disordered growth
  • ↑↑↑ rate of metastasis w/ LOWER survival
66
Q

Phrenic nerve invasion by lung tumor is associated with what?

A

Diaphragm paralysis

67
Q

Activating mutations of which gene in lung cancers is associated with a worse prognosis regardless of tx?

A

KRAS

68
Q

Release of PTHrP leading to paraneoplastic hypercalcemia is associated with what type of lung tumor?

A

Squamous cell carcinoma

69
Q

Which 2 hormones may be secreted by small cell carcinomas and what does each cause

A
  • ADH —> SIADH
  • ACTH —> Cushing Syndrome
70
Q

What is a feature that can be seen on CXR and CT that points to metastasis TO the lung?

A

Multiple discrete nodules (cannonball lesions) scattered throughout all lobes

71
Q

How are lung hamartomas most often discovered and how do they appear?

A
  • Incidental finding on CXR
  • Appear as rounded, radio-opacity (“coin lesion”)
72
Q

What do pulmonary hamartomas consist of?

A

Nodules of CT; most often cartilage, also fat and fibrous tissue; intersected by epithelial clefts

73
Q

Which 2 chromosomal aberrations are associated with lung hamartomas?

A
  • 6p21
  • 12q14-q15
74
Q

What are 2 systemic nervous system related syndromes that may be associated with lung tumors?

A
  • Lambert-Eaton myasthenic syndrome –> auto-abs against Ca2+ channels –> muscle weakness
  • Peripheral neuropathy –> usually purely sensory
75
Q

What is a dermatologic manfiestation that may be associated with lung tumors?

A

Acanthosis nigricans

76
Q

Lymphangioleiomyomatosis is characterized by proliferation of which cells and what does this create morphologically?

A

Perivascular epithelioid cells –> CYSTIC, emphysema-like dilation of terminal air spaces, thickening of interstitium, and obstruction of lymph vessles

77
Q

Lymphangioleiomyomatosis is associated with loss of function of which tumor suppressor; this tumor suppressor is normally a negative regulator of what?

A

TSC2 –> neg. regulator of mTOR activity

79
Q

Diffuse involvement of both lungs with cystic, emphysem-like dilation of airspaces in Lymphangioleiomyomatosis, may present with what complication?

A

Pneumothorax

81
Q

What are 2 hematologic abnormalities that may be assocaited with lung tumors?

A
  • Leukemoid rxns
  • Trousseau syndrome (DVT and thromboembolism)
82
Q

What is a peculiar abnormality of connective tissue associated with lung tumors and what is seen with this?

A

Hypertrophic pulmonary osteoarthropathy –> clubbing of fingers

83
Q

Lambert-Eaton Syndrome is most often associated with what tumor of the lung?

A

Small cell carcinoma

84
Q

Trousseau sign (migratory thrombophlebitis) is most often associated with what type of lung tumor?

A

Adenocarcinoma

86
Q

Why is it important to differentiate DIPNECH from Carcinoid tumors?

A

Carcinoid tumors have ability to metastasize

90
Q

Immunohistochemical stains of carcinoid tumors may be positive for what 4 markers?

A
  • Serotonin
  • Neuron-specific enolase
  • Calcitonin
  • Bombesin
91
Q

What are clinical features of carcinoid tumors which may result from their intraluminal growth?

A
  • Persistent cough
  • Hemoptysis
  • Impairment of drainage of resp. pathways –> 2’ infections
  • Bronchiectasis
  • Emphysema
  • Atelectasis
92
Q

What are the sx’s of Carcinoid Syndrome?

A
  • Flushing
  • Diarrhea
  • Cyanosis
93
Q

What is the prognosis like for typical carcinoid vs. atypical carinoid tumors?

A
  • Typical have 5-year survival of 95%
  • Atypical have 5-year survival of 70%
98
Q

Lymphangioleiomyomatosis is a lung disorder occurring almost exclusively in whom?

A

Young women

99
Q

Which markers are expressed by Lymphangioleiomyomatosis?

A

Melanoma, like HMB-45 and smooth muscle cells

102
Q

What is the only definitive tx currently available for Lymphangioleiomyomatosis?

A

Lung transplant

104
Q

The strong tendency for Lymphangioleiomyomatosis to affect young women suggests what hormone contributes to proliferation of perivascular epithelioid cells?

A

Estrogen

105
Q

Inflammatory myofibroblastic tumors, though rare, is more common in whom; presents how?

A
  • Children
  • Fever + cough + chest pain + hemoptysis
  • May be asymptomatic
106
Q

Imaging of a child with suspected Inflammatory myofibroblastic tumor will show what?

A

Single (rarely multiple) round, well-defined, peripheral mass that often has calcium deposits

107
Q

Inflammatory myofibroblastic tumors are associated with activating rearrangements of which gene and on which chromosome?

A

ALK gene on chromosome 2p23

108
Q

Growth of contiguous tumors (aka direct continuity) into the lungs most often occurs with what 2 types of tumors?

A
  • Esophageal carcinomas
  • Mediastinal lymphomas
109
Q

Radiation used in therapy for tumors in the lung or mediastinum often causes what type of pleuritis?

A

Serofibrinous pleuritis

110
Q

A purulent pleural exudate (empyema) usually results from what?

A

Bacterial or mycotic seeding of the pleural space; by contiguous spread from intrapulmonary infections, but may also be from lymphatic or hematogenous dissemination

111
Q

Which inflammatory-type of pleural effusion is characterized by loculated, yellow-green, creamy pus composed of masses of neutrophils admixed with other leukocytes?

A

Empyema

112
Q

Empyema of the lungs may resolve, but more often the exudate organizes into what; causes what complications?

A
  • Organizes –> dense, tough fibrous adhesions
  • Frequently obliterate the pleural space or envelope the lungs
  • Restricting pulmonary expansion
115
Q

What are the 3 major mechanisms underlying transudative pleural effusions; examples?

A
  • ↑ hydrostatic pressure; “overflow” from the lung interstitium = CHF
  • ↓ plasma oncotic pressure = nephrotic syndrome
  • Movement of transudative ascitic fluid thru the diaphargam = cirrhosis
116
Q

What are the 2 major mechanisms underlying exudative pleural effusions; some examples?

A
  • Inflammation –> infection (TB, pneumonia), PE (infarction), CT disease (SLE, RA), adjacent to subdiaphragmatic disease (pancreatitis, subphrenic abscess)
  • Malignancy
117
Q

What is the arrow indicating on this CT scan?

A

Pleural effusion

118
Q

How will a pleural effusion associated with mediastinal carcinomatosis appear grossly due to decreased lymph drainage?

A

Milky chylous effusion = Chylothorax

119
Q

Noninflammatory collections of serous fluid within pleural cavities are known as what; appear how grossly?

A

Hydrothorax; clear fluid that is straw colored

120
Q

What is the most common cause of hydrothorax; other causes?

A
  • Most common = Cardiac failure
  • Also, renal failure and cirrhosis
121
Q

Which type of pleuritis is manifested by sanguineous inflammatory exudates?

A

Hemorrhagic pleuritis

122
Q

Hemorrhagic pleuritis is found in what 3 settings?

A
  • Hemorrhagic diatheses
  • Rickettsial diseases
  • Neoplastic involvement
123
Q

Spontaneous idiopathic penumothorax most often occurs in which age group and is due to what?

A
  • Young patients
  • Rupture of small, peripheral, usually apical subpleural blebs
125
Q

When injury to chest wall results in a one-way valve allowing air into the pleural space, but not out, effectively acting as a pump and ↑ pressure, this is known as?

A

Tension pneumothorax

126
Q

A tension pneumothorax is due to expansion of which structure?

A

Chest wall (not lungs per se)

127
Q

What is one of the more devastating consequences of Empyema that makes it hard to treat and hard to clear out?

A

Creating loculations –> web-like traps for fluid

128
Q

What type of pneumothorax is this?

A

Primary pneumothorax

129
Q

What type of pneumothorax is this?

A

Tension pneumothorax

130
Q

Which type of pneumothorax is associated with pleural cavity pressure is < atmospheric pressure?

Pleural cavity pressure > atmospheric pressure?

A
  • Pleural cavity < atmospheric = primary pneumothorax
  • Pleural cavity > atmospheric = tension pneumothorax
131
Q

In spontaneous idiopathic pneumothorax which direction will the trachea shift?

A

TOWARD the side of collapse

135
Q

Which soft-tissue tumor has a propensity to occur in the pleura is often attached to the surface by a pedicle?

A

Solitary fibrous tumor

137
Q

What do solitary fibrous tumors show microscopically?

A

Whorls of reticulin and collagen fibers w/ interspersed spindle cells resembling fibroblasts

139
Q

Hemothorax is a usually fatal complication associated with what?

A

Ruptured aortic aneurysm or vascular trauma

140
Q

How does malignant mesothelioma appear grossly?

A

Affected lung is ensheathed by a thick layer of soft, gelatinous, grayish-pink tumor tissue

141
Q

What are the 3 variants of growth that can be seen microscopically with mesothelioma?

A
  • Epithelioid
  • Sarcomatoid
  • Mixed
142
Q

Epithelioid type of mesothelioma resembles adenocarcinoma; how can it be differentiated via immunohistochemical stains for which 5 markers?

A
  • (+) Keratin
  • (+) Calretinin
  • (+) Wilms tumor 1 (WT-1)
  • (+) Cytokeratin 5/6
  • (+) D2-40
143
Q

Pneumothorax is most commonly associated with what 3 conditions?

A
  • Emphysema
  • Asthma
  • Tuberculosis
145
Q

Typical course of spontaneous idiopathic penumothorax; and likelihood or recurrence?

A
  • Typically subsides spontaneously as air is resorbed
  • Reccurent attacks = common

2020 CPR II Pulm Exam 2 (28 decks)

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