CHEST PATH Flashcards
name CF manifestations in different systems
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
Lung LCH pathophysio ?
- Immature dendritic cells
- S100 positive
- Birbeck granules
How many % of lung LCH are smokers ?
95%
M = F
Lung LCH associations
- ALL
- AML
Radiology findings of LCH
- Upper zone Centrilobular cavitating nodules (early)
- Spares costophrenic and costomediastinal angles
- Normal lung parenchyma
- Bizarre shaped cysts, thin walled
- Fibrosis and honey combing (late)
Lung LCH prognosis
- May regress if stop smoking
- End stage pulmonary fibrosis if continue smoking
Lung LCH complications
- Pneumothorax (first presentation)
- Pneumomediastinum
- Interstitial fibrosis
- Pulmonary HTN and Cor pulmonale
Lung LAM inheritance ?
- Spontaneous (most common)
- TSC1 and TSC2
Lung LAM pathophysio ?
- Proliferation of smooth muscle cells* in lung
Extra-thoracic findings of LAM
- renal AMLs (even if no TS)
- Chylous ascites
- Lymphangioleiomyomas
- Uterine leiomyomas
Prognosis and treatment of LAM
- slow progressive over decades
- lung transplant only definitive treatment
LAM complications
- Pneumothorax (recurrent, bilateral)
- AML bleed (often fatal)
- Hemoptysis
- Lung infections
- SEGA obstructing (death)
- Osteoporosis (in immobile pts)
LIP epidemiology
- F (because Sjogren syndrome most common in F)
- middle age adults (most common)
- child (have AIDS)
LIP associations
- Sjogren syndrome (most common - 25%)
- AIDS (children)
- Connective tissue diseases
- SLE
- Castleman
- RA
- Pulmonary amyloidosis
Radiological features of LIP
- mid to lower zone
- small centrilobular nodules
- ground glass changes
- thin walled cysts
- mediastinal lymphadenopathy
LIP complication
- Develop into Lymphoma* (5%)
- More than 30% of patients will develop end-stage disease and honeycombing despite treatment.
UIP / IPF Risk factors
- Smoking (most important, increases several folds)
- Metal fumes
- Wood dust
- Farming, Hair dressing, Stone-polishing
- GORD*
Microscopic of UIP
- **Spatial and temporal heterogeneity (fibrotic lesions at different stage)
- Patchy interstitial fibrosis is hallmark
- Fibroblastic foci (early stage)
UIP complications
- Pulmonary HTN
- Cor pulmonale
- Lung cancer
- Pneumothorax/Pneumomediastinum
- Pneumonia
UIP Aetiologies
Connective tissue disorders
- RA
- Scleroderma
- Polymyositis/Dermatomyositis (UIP, NSIP, COP)
- Asbestosis
- Hypersensitivity Pneumonitis
- Amiodarone
- ANCA vasculitis
- Radiation
UIP epidemiology
- M > F (NSIP Female)
- Older patients (NSIP younger)
NSIP epidemiology
- F > M (due to high association to collagen vascular disease)
- 40-50yo
NSIP associations
NOT associated with smoking
Connective tissue disorders
- Scleroderma
- SLE
- Sjogrens
- Polymyositis
- Dermatomyositis
- IgG4
Autoimmune
- RA
- PBC
- Hashimoto thyroiditis
- Hypersensitivity pneumonitis
- Chemotherapy: Thalidomide
Microscopic of NSIP
- 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.
Treatment and Prognosis of NSIP
Prognosis
- Overall better prognosis 45% full recovery
Treatment
- Steroids effective*
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**
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.
ABPA microsscopic findings
- Charcot Leyden crystals (seen in asthma and eosinophilic diseases)
- Bronchocentric granulomatosis
- TH2 helper cell response
Asbestos 2 types ?
Serpentine Chrysotile - White: Least pathogenic
Amphiboles - Blue: Includes crocidolite (most pathogenic)
do lung carcinoid tend to metastasize ?
- no
how many % get carcinoid syndrome ?
10% get carcinoid syndrome
Survival rate for lung carcinoid
5 year survival 95%
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.
Cardiac myxoma
Carney complex
Dilated cardiomyopathy causes
- Idiopathic (most common)
- Genetic, alcohol, peripartum, post-viral, post-MI, haemochromatosis, sarcoidosis
Hypertrophic Cardiomyopathy causes
- 100% genetic
- sarcomeric proteins defect
Restrictive cardiomyopathy causes
o Idiopathic
o Amyloidosis
o Radiation
o Infiltration (amyloidosis, sarcoidosis, haemochromatosis, metastases), eosinophilic
endomyocardial disease (Loffler), endomyocardial fibrosis (tropical areas)
Cystic fibrosis mutation
- CF transmembrane conductance regulator
(CFTR) gene on chromosome 7. - The most common specific mutation is “delta F508 mutation”.
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)
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).
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]).
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**
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
How does Mucinous adenocarcinoma spread ?
spread aerogenously forming satellite tumours and can involve a whole lobe.
Define Adenocarcinoma in-situ features
- <3cm
- Contains dysplastic cells only
- PURE lepidic growth
- Non-mucinous (usually)
Atypical adenomatous hyperplasia (AAH) features
- Pre-invasive
- GGN less than or equal to 5 mm
- 10% grow, 1% develop into MIA or invasive adenocarcinoma
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
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*
Lung cancer paraneoplastic syndromes
- SIADH
- Cushing syndrome
- Carcinoid syndrome
- Gynaecomastia
- Adrenal insufficiency
- Hyperparathyroidism
- Hypocalcaemia
- Limbic encephalitis
- HPOA
- Polymyositis
How many with Mesothelioma have asbestosis ?
- 10-20% have concurrent asbestosis.
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)
% of cases of Mesothelioma related to asbestos exposure ?
90% of cases related to asbestos exposure
Which type of Mesothelioma most common and resembles..?
Epitheliod (most common)
- resembles adenocarcinoma
Sarcomatoid type
- resembles fibrosarcoma
Howw does Mesothelioma spread ?
- Direct invasion (early)
- Hematogeneous and Lymphatic spread (late)
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
What’s the most frequent type of Acute pericarditis ?
- Fibrinous Pericarditis (DRESSLERS - post infarction)
What are the types of Chronic pericarditis
- Adhesive
- Constrictive
What causes Caseous pericarditis ?
- TB
- Fungal (less common)
Types of Pneumonias
- Community acquired pneumonia
e.g. Strep pneumoniae, H influensae, Staph aureus, Legionella, Mycoplasma - Heath Care-Associated
e.g. MRSA, MSSA, Pseudomonas, Strep - Hospital-Acquired
e.g. Gram neg rods like Klebsiella, E coli; Staph aureus - Aspiration pneumonia
e.g. Anaerobic oral flora like Bacteroides - Chronic pneumonia
e.g. Nocardia, Actinomyces, Granulomatous like TB and MAC - Necrotising pneumonia and lung abscess
e.g. Staph, Klebsiella, - Pneumonia in immunocompromised host e.g. CMV, PJP, MAC, invasive aspergillosis
4 stages of inflammatory response in Pneumonia
- Congestion
- Red hepatisation (12h)
- Gray hepatisation (10days)
- Resolution
Lobar pneumonia pathogens
Strep pneumoniae and klebsiella most commonly
Bronchopneumonia pathogens
Usually staph, gram negative, anaerobes and legionella
Pulmonary abscess pathogens
- Klebsiella
- Staph
- Strep
Rheumatic fever cause
Group A pharyngitis
Exaggerated immune response to the strep M proteins
Rheumatic fever epi
Children 5-15 years
Country where people dont have antibiotics
Major criterias of Rheumatic fever
- Migratory polyarthritis
- Pancarditis
- Chorea
- Erythema marginatum
- Subcutaneous nodules
Rheumatic fever microscpic
- Aschoff bodies
Rheumatic fever prognossis ?
- Death is rare*
- Myocarditis
- Mitral stenosis
Sarcoidosis epidemiology
- younger than 40
- FEMALE*
- African americans
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*
2 forms of silica, which form more fibrogenic ?
- Crystalline form more fibrogenic*
- Amorphous form
(Dose dependent!)
Lung cancer risk in Silicosis ?
- DOUBLED
Complications / Associations of Silicosis ?
- TB
- PAP
- Lung cancer
Thymic neoplasm Associations
- Myaesthenia gravis
- Red cell aplasia
- Aplastic anaemia
- Hypogammaglobulinaemia
- SLE
- RA
Thymic carcinoma is mostly what ?
- SCC
- 5% of thymomas
Rheumatic heart disease involves what valve ?
- Mitral valve – virtually the only cause of mitral stenosis
Rheumatic heart disease wart appearance, size
small warty vegetations
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.
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).
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
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.
Lambl’s excresence
mimic – aggregation of fibrin covered by endothelium.
Acute rheumatic carditis
Common manifestation of active RF -> may progress over time to chronic rheumatic heart disease
mainly manifesting as valvular abnormalities.
Aschoff bodies seen in ?
- ACUTE Rheumatic fever
- found in all 3 layers: pancarditis*
Anitschkow cells seen in ?
- Pathogneumonic for: Rheumatic fever (more common in acute)
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.
MacCallum plaques seen in ?
- Acute RF
Chronic RF mitral valve appearance ?
“fish mouth” stenosis - mitral valve stenosis
leaflet thickening, commissural fusion and shortening/thickening/fusion of the tendinous cords.
Lung adenocarcinoma What mutations ?
Gain-of-function mutations
- Receptor tyrosine kinases such as EGFR.
- New therapies such as tyrosine kinase inhibitors prolong survival.
Most common cardiac valve disease
Aortic valve stenosis
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
Pre-existing aortic lesion leading to Dissection ?
- Cystic medial degeneration is the pre-existing lesion
- However, many with CMD do not get aortic dissection*
Do all aortic dissections see tears ?
- 5-10% have no obvious intimal tear
- Can extend retrograde or anterograde
Least involved location of sarcoid ?
Gastrointestinal tract
Most common sites involved by Sarcoid ?
- The lungs and hilar lymph nodes are most commonly involved, followed by eye and skin.
Squamous cell carcinoma of lung associated mutations ?
- TP53 gene
- RB tumour suppressor
What are 4 causes of fibrosing mediastinitis?
- IgG4
- TB
- Histoplasma
- Sarcoidosis
- Intrathoracic malignancy
- Lymphoma
- Radiation
- Drugs (methysergide)
