CHEST PATH Flashcards
(98 cards)
1
Q
name CF manifestations in different systems
A
Sweat gland
- decreased absoprtion of NaCl from lumen, thus high in sweat (salty sweat)
Lung
- Infection (Pseudomonas)
- ABPA (10%)
- Bronchial artery hypertrophy -> Hemoptysis
Pancreass
- Pancreatic insufficiency
- Chronic pancreatitis
- Atrophy of exocrine pancreas (from mucous plugging)
Intestine
- Meconium ileus
- Steatorrhea
Salivary glands
- Ductal dilatation
Liver
- Secondary biliary cirrhosis (cannaliculi plugging)
Testes
- azoospermia and infertility
- Absent vas deferens
2
Q
Lung LCH pathophysio ?
A
- Immature dendritic cells
- S100 positive
- Birbeck granules
3
Q
How many % of lung LCH are smokers ?
A
95%
M = F
4
Q
Lung LCH associations
A
- ALL
- AML
5
Q
Radiology findings of LCH
A
- Upper zone Centrilobular cavitating nodules (early)
- Spares costophrenic and costomediastinal angles
- Normal lung parenchyma
- Bizarre shaped cysts, thin walled
- Fibrosis and honey combing (late)
6
Q
Lung LCH prognosis
A
- May regress if stop smoking
- End stage pulmonary fibrosis if continue smoking
7
Q
Lung LCH complications
A
- Pneumothorax (first presentation)
- Pneumomediastinum
- Interstitial fibrosis
- Pulmonary HTN and Cor pulmonale
8
Q
Lung LAM inheritance ?
A
- Spontaneous (most common)
- TSC1 and TSC2
9
Q
Lung LAM pathophysio ?
A
- Proliferation of smooth muscle cells* in lung
10
Q
Extra-thoracic findings of LAM
A
- renal AMLs (even if no TS)
- Chylous ascites
- Lymphangioleiomyomas
- Uterine leiomyomas
11
Q
Prognosis and treatment of LAM
A
- slow progressive over decades
- lung transplant only definitive treatment
12
Q
LAM complications
A
- Pneumothorax (recurrent, bilateral)
- AML bleed (often fatal)
- Hemoptysis
- Lung infections
- SEGA obstructing (death)
- Osteoporosis (in immobile pts)
13
Q
LIP epidemiology
A
- F (because Sjogren syndrome most common in F)
- middle age adults (most common)
- child (have AIDS)
14
Q
LIP associations
A
- Sjogren syndrome (most common - 25%)
- AIDS (children)
- Connective tissue diseases
- SLE
- Castleman
- RA
- Pulmonary amyloidosis
15
Q
Radiological features of LIP
A
- mid to lower zone
- small centrilobular nodules
- ground glass changes
- thin walled cysts
- mediastinal lymphadenopathy
16
Q
LIP complication
A
- Develop into Lymphoma* (5%)
- More than 30% of patients will develop end-stage disease and honeycombing despite treatment.
17
Q
UIP / IPF Risk factors
A
- Smoking (most important, increases several folds)
- Metal fumes
- Wood dust
- Farming, Hair dressing, Stone-polishing
- GORD*
18
Q
Microscopic of UIP
A
- **Spatial and temporal heterogeneity (fibrotic lesions at different stage)
- Patchy interstitial fibrosis is hallmark
- Fibroblastic foci (early stage)
19
Q
UIP complications
A
- Pulmonary HTN
- Cor pulmonale
- Lung cancer
- Pneumothorax/Pneumomediastinum
- Pneumonia
20
Q
UIP Aetiologies
A
Connective tissue disorders
- RA
- Scleroderma
- Polymyositis/Dermatomyositis (UIP, NSIP, COP)
- Asbestosis
- Hypersensitivity Pneumonitis
- Amiodarone
- ANCA vasculitis
- Radiation
21
Q
UIP epidemiology
A
- M > F (NSIP Female)
- Older patients (NSIP younger)
22
Q
NSIP epidemiology
A
- F > M (due to high association to collagen vascular disease)
- 40-50yo
23
Q
NSIP associations
A
NOT associated with smoking
Connective tissue disorders
- Scleroderma
- SLE
- Sjogrens
- Polymyositis
- Dermatomyositis
- IgG4
Autoimmune
- RA
- PBC
- Hashimoto thyroiditis
- Hypersensitivity pneumonitis
- Chemotherapy: Thalidomide
24
Q
Microscopic of NSIP
A
- Temporal and spatial homogeneity (c.f. UIP is Heterogeneity)
- NO acute lung injury
- NO hyaline membranes
- NO granulomas
- NO organisms or viral inclusions
- NO dominant airways disease or organising pneumonia, eosinophils and coarse fibrosis.
25
Treatment and Prognosis of NSIP
Prognosis
- Overall better prognosis 45% full recovery
Treatment
- Steroids effective*
26
Patho appearance of COP
- Alveolar and bronchiole inflammation
- Polypoid plugs of loose organising connective tissue (Masson bodies)**
- The connective tissue is of the same age**
- Underlying lung architecture is normal. No interstitial fibrosis or honeycomb lung**
27
COP Complications and Prognosis and Treatment
Prognosis: Waxing and waning course, often treated for months for “recurrent” pneumonia.
Treatment: Some recover spontaneously, most need oral steroids for 6 months + for full recovery.
28
ABPA microsscopic findings
- Charcot Leyden crystals (seen in asthma and eosinophilic diseases)
- Bronchocentric granulomatosis
- TH2 helper cell response
29
Asbestos 2 types ?
Serpentine Chrysotile - White: Least pathogenic
Amphiboles - Blue: Includes crocidolite (most pathogenic)
30
do lung carcinoid tend to metastasize ?
- no
31
how many % get carcinoid syndrome ?
10% get carcinoid syndrome
32
Survival rate for lung carcinoid
5 year survival 95%
33
Cardiac myxoma site?
- Most common cardiac tumor
- 90% of myxomas arise in the atria, with a L to R ratio of 4:1
- Usually single
- Region of the fossa ovalis in the atrial septum is the favoured site of origin.
34
Cardiac myxoma
Carney complex
35
Dilated cardiomyopathy causes
- Idiopathic (most common)
- Genetic, alcohol, peripartum, post-viral, post-MI, haemochromatosis, sarcoidosis
36
Hypertrophic Cardiomyopathy causes
- 100% genetic
- sarcomeric proteins defect
37
Restrictive cardiomyopathy causes
o Idiopathic
o Amyloidosis
o Radiation
o Infiltration (amyloidosis, sarcoidosis, haemochromatosis, metastases), eosinophilic
endomyocardial disease (Loffler), endomyocardial fibrosis (tropical areas)
38
Cystic fibrosis mutation
- CF transmembrane conductance regulator
(CFTR) gene on chromosome 7.
- The most common specific mutation is “delta F508 mutation”.
39
What renders a lung cancer unresectable ?
T4: Invasion of mediastinal structures, vertebral body, tumor nodule(s) in separate ipsilateral lung lobe
N3: Metastases to scalene, supraclavicular, contralateral mediastinal, or contralateral nodes
M1: Metastatic disease (malignant pleural effusion)
40
What's the key observation in aberrant right subclavian artery ?
The aneurysmal dilatation of the proximal portion of the aberrant subclavian artery is called a diverticulum of Kommerell. It may compress the esophagus and cause dysphagia (dysphagia lusoria).
41
Sinus venosus atrial septal defects (ASDs) are almost always associated with ?
Sinus venosus atrial septal defects (ASDs) are almost always associated with partial anomalous pulmonary venous return (usually of a right upper lobe pulmonary vein into the superior vena cava [SVC]).
42
Lung Squamous cell carcinoma associations
- Highly associated with smoking.
- Has diverse genetic abnormalities many of which involve loss of tumour suppressor genes such as
TP53 gene** (loss leads to uncontrolled cell division) and RB tumour suppressor**
43
Lung adenocarcinoma
- micro and macro
- spread pattern ? invasion ?
Macro: Found more often in the periphery of the lung and tend to be smaller. Rarely cavitate
Micro: glandular differentiation and mucin production.
Lepidic pattern of spread at periphery of tumour - neoplastic cells lining the alveoli with no architectural disruption and no invasion ie. Spreading along existing structures
44
How does Mucinous adenocarcinoma spread ?
spread aerogenously forming satellite tumours and can involve a whole lobe.
45
Define Adenocarcinoma in-situ features
- <3cm
- Contains dysplastic cells only
- PURE lepidic growth
- Non-mucinous (usually)
46
Atypical adenomatous hyperplasia (AAH) features
- Pre-invasive
- GGN less than or equal to 5 mm
- 10% grow, 1% develop into MIA or invasive adenocarcinoma
47
Micro-invasive Adenocarcinoma (MIA) features
- Less than or equal to 3 cm
- Invasion of less than or equal to 5 mm in any 1 focus
- Absence of stromal, vascular or pleurale invasion.
- Usually nonmucinous
48
Small cell (14%) lung cancer features
- most aggressive
- originate from Neuro-endorcrine cells
- Small cells with scant cytoplasm
- Necrosis common
- All small cell cancer are high grade*
49
Lung cancer paraneoplastic syndromes
- SIADH
- Cushing syndrome
- Carcinoid syndrome
- Gynaecomastia
- Adrenal insufficiency
- Hyperparathyroidism
- Hypocalcaemia
- Limbic encephalitis
- HPOA
- Polymyositis
50
How many with Mesothelioma have asbestosis ?
- 10-20% have concurrent asbestosis.
51
What's the risk of Mesothelioma in asbestos workers who smoke ?
- No increased risk of mesothelioma in asbestos workers who smoke.
- Interestingly this is in contrast to the risk of asbestos-related lung cancer which is markedly magnified by smoking (50x, just 5x asbestos non smoker)
52
% of cases of Mesothelioma related to asbestos exposure ?
90% of cases related to asbestos exposure
53
Which type of Mesothelioma most common and resembles..?
Epitheliod (most common)
- resembles adenocarcinoma
Sarcomatoid type
- resembles fibrosarcoma
54
Howw does Mesothelioma spread ?
- Direct invasion (early)
- Hematogeneous and Lymphatic spread (late)
55
Early vs Late complications of MI
early
- Arrythmias, Infarct, Fibrinous Pericarditis*
- Myocardial rupture/Haemopericardium, Mural thrombus
late
- Autoimmune percarditis
- Ventricular aneurysm
- Papillary muscle rupture
- Congestive heart failure
56
What's the most frequent type of Acute pericarditis ?
- Fibrinous Pericarditis (DRESSLERS - post infarction)
57
What are the types of Chronic pericarditis
1. Adhesive
2. Constrictive
58
What causes Caseous pericarditis ?
- TB
- Fungal (less common)
59
Types of Pneumonias
1. Community acquired pneumonia
e.g. Strep pneumoniae, H influensae, Staph aureus, Legionella, Mycoplasma
2. Heath Care-Associated
e.g. MRSA, MSSA, Pseudomonas, Strep
3. Hospital-Acquired
e.g. Gram neg rods like Klebsiella, E coli; Staph aureus
4. Aspiration pneumonia
e.g. Anaerobic oral flora like Bacteroides
5. Chronic pneumonia
e.g. Nocardia, Actinomyces, Granulomatous like TB and MAC
6. Necrotising pneumonia and lung abscess
e.g. Staph, Klebsiella,
7. Pneumonia in immunocompromised host e.g. CMV, PJP, MAC, invasive aspergillosis
60
4 stages of inflammatory response in Pneumonia
1. Congestion
2. Red hepatisation (12h)
3. Gray hepatisation (10days)
4. Resolution
61
Lobar pneumonia pathogens
Strep pneumoniae and klebsiella most commonly
62
Bronchopneumonia pathogens
Usually staph, gram negative, anaerobes and legionella
63
Pulmonary abscess pathogens
- Klebsiella
- Staph
- Strep
64
Rheumatic fever cause
Group A pharyngitis
Exaggerated immune response to the strep M proteins
65
Rheumatic fever epi
Children 5-15 years
Country where people dont have antibiotics
66
Major criterias of Rheumatic fever
- Migratory polyarthritis
- Pancarditis
- Chorea
- Erythema marginatum
- Subcutaneous nodules
67
Rheumatic fever microscpic
- Aschoff bodies
68
Rheumatic fever prognossis ?
- Death is rare*
- Myocarditis
- Mitral stenosis
69
Sarcoidosis epidemiology
- younger than 40
- FEMALE*
- African americans
70
Findings in Sarcoidosis ?
- "Well-formed" non-necrotising granulomas made of clustered macrophages and giant cells
- Asteroid bodies, Schuermann bodies (60% found in granulomas, Characteristic but NOT specific)
- High serum ACE*
71
2 forms of silica, which form more fibrogenic ?
- Crystalline form more fibrogenic*
- Amorphous form
(Dose dependent!)
72
Lung cancer risk in Silicosis ?
- DOUBLED
73
Complications / Associations of Silicosis ?
- TB
- PAP
- Lung cancer
74
Thymic neoplasm Associations
- Myaesthenia gravis
- Red cell aplasia
- Aplastic anaemia
- Hypogammaglobulinaemia
- SLE
- RA
75
Thymic carcinoma is mostly what ?
- SCC
- 5% of thymomas
76
Rheumatic heart disease involves what valve ?
- Mitral valve – virtually the only cause of mitral stenosis
77
Rheumatic heart disease wart appearance, size
small warty vegetations
78
Infective endocarditis wart size and location
- Large crumbling masses on the valve cusps or mural endocardium that can extend onto the chordae, single or multiple.
- The vegetations of subacute (versus acute) are associated with less valvular destruction, although the
distinction can be subtle.
79
Non-bacterial thrombotic endocarditis (NBTE) – aka Marantic Small sterile vegetations
- do they incite inflammation ?
- appearance and location ?
- caused by what ?
- Vegetations are non invasive and do not incite an inflammatory response
- Usually attached to the line of closure.
- Loosely attached to the underlying valve.
- Associated with underlying malignancy/hypercoaguability (eg. sepsis and cancer especially mucinous adenocarcinoma).
80
Libman-sacks endocarditis (endocarditis of SLE)
- appearance and location ?
- Small verrucous vegetations on either or both sides of the valve leaflets (mitral/tricuspid).
- Often intense valvulitis
81
Carcinoid heart disease
- appearance and location ?
- association ?
- Plaque-like deposits of fibrous tissue on valves and elsewhere. Predilection for R heart valves. Glistening white.
- Typically occur only when there is a large volume of hepatic disease.
82
Lambl’s excresence
mimic – aggregation of fibrin covered by endothelium.
83
Acute rheumatic carditis
Common manifestation of active RF -> may progress over time to chronic rheumatic heart disease
mainly manifesting as valvular abnormalities.
84
Aschoff bodies seen in ?
- ACUTE Rheumatic fever
- found in all 3 layers: pancarditis*
85
Anitschkow cells seen in ?
- Pathogneumonic for: Rheumatic fever (more common in acute)
86
Inflammation of the valves in RF results in what type of necrosis ?
fibrinoid necrosis
- Small 1-2 mm vegetations overlying the necrotic foci along the lines closure are called verrucae.
87
MacCallum plaques seen in ?
- Acute RF
88
Chronic RF mitral valve appearance ?
“fish mouth” stenosis - mitral valve stenosis
leaflet thickening, commissural fusion and shortening/thickening/fusion of the tendinous cords.
89
Lung adenocarcinoma What mutations ?
Gain-of-function mutations
- Receptor tyrosine kinases such as EGFR.
- New therapies such as tyrosine kinase inhibitors prolong survival.
90
Most common cardiac valve disease
Aortic valve stenosis
91
Aortic dissection tear into inner or outer Media ?
- Intimal tear into MID to OUTER 1/3 of media
- Occasionally the dissecting haematoma re-enters the true lumen via a second distal tear creating a double
barrelled aorta
92
Pre-existing aortic lesion leading to Dissection ?
- Cystic medial degeneration is the pre-existing lesion
- However, many with CMD do not get aortic dissection*
93
Do all aortic dissections see tears ?
- 5-10% have no obvious intimal tear
- Can extend retrograde or anterograde
94
Least involved location of sarcoid ?
Gastrointestinal tract
95
Most common sites involved by Sarcoid ?
- The lungs and hilar lymph nodes are most commonly involved, followed by eye and skin.
96
Squamous cell carcinoma of lung associated mutations ?
- TP53 gene
- RB tumour suppressor
97
What are 4 causes of fibrosing mediastinitis?
- IgG4
- TB
- Histoplasma
- Sarcoidosis
- Intrathoracic malignancy
- Lymphoma
- Radiation
- Drugs (methysergide)
97
