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35 Cards in this Set

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This lesion shows dense, intra-alveolar eosinophilic material with associated cholesterol clefts.

What are the causes? (4)

What is the treatment?
Lesion: Alveolar proteinosis

Causes: infections, acute silicosis, malignancies, idiopathic

Treatment: Some resolve spontaneously; whole lung lavage is very effective for those who need it.
What are the EM findings in mesothelioma vs. adenoarcinoma?

ICH Mesothelial cells vs. Glandular cells:
calretinin
WT1
CK5/6
B72.3
BerEP4
LeuM1 (CD15)
CEA
p63
EM: Mesothelioma features long slender microvilli while adenocarcinoma features short microvilli.

Mesothelial Cells:
calretinin +
WT1 +
CK5/6 +
B72.3 -
BerEP4 -
LeuM1 (CD15) -
CEA -
p63 -

Glandular Cells:
calretinin -
WT1 -
CK5/6 -
B72.3 +
BerEP4 +
LeuM1 (CD15) +
CEA +
p63 -
What condition is linked with mutations in BMPRII gene?
Primary pulmondary hypertension
Grossly, this lung lesion resembles cirrhotic liver on the surface due to scarring and retraction. Hstiologically, it is characterized by a peripheral lobular fibrosing process, resulting in dense fibrosis in the peripheral zones with loss of alveolar architecture. Pulmonary lobules show temporal heterogeneity.

What is meant by temporal heterogeneity?

What is the treatment?
Clinical idiopathic pulmonary fibrosis = pathological usual interstitial pneumonia

Temporal heterogeneity: uninvolved lung, fibroblastic/myofibroblastic proliferation, dense scarring all in the same biopsy.

Treatment: lung transplant. Steroids don't do anything.
Define centrilobular emphysema and name a risk factor. What is the mechanism of damage? What lobes are more typically affected?
Definition: Centrilobular emphysema = expansion of the respiratory bronchioles due to damage with sparing of the distal structures, alveoli.

Risk factor: Smoking

Mechanism: Smoking causes an imbalance between proteases (increased) that damage connective tissue (e.g. elastin) and antiproteases (decreased).

Location: upper lobes
Define panlobular emphysema and name the cause. What lobes are more typically affected?
Definition: Panlobular emphysema = expansion of the respiratory acinus (respiratory bronchioles + alveoli)

Cause: alpha-1-antitrypsin deficiency

Location: Lower lobes and anterior lung margins
Grossly, this lesion consists of marked dilation of the bronchi with dilation extending up to the pleura. The mucosa of the airway is discolored. There is significant loss of alveolar lung parenchyma with collapse.
Bronchiectasis
What generally causes bronchiectasis?
Generally, anything that leads to chronic obstruction of airways will result in conducting airway remodeling which causes dilation and down-stream structures to eventually collapse and fibrose:
Repeated infection
Allergic bronchopulmonary aspergillosis
Associated with autoimmune diseases (RA, IBD)
CF
Kartagener's syndrome
Exposure to this substance causes an allergic reaction which histologically resembles sarcoidosis.
Beryllium (berylliosis)

NOTE: Classically associated with beryllium mining or fluorescent light bulb manufacturing
This biphasic neoplasm is composed of papillary-like pojections lined by epithelioid cells with central proliferation of polygonal cells. Both populations are positive for EMA and TTF1. The epithelioid cells are positive for cytokeratin while the central polygonal cells are negative.

What population is more commonly affected?

What is the cell of origin?

What is the behavior?
Tumor: sclerosing hemangioma of lung

Population: females in their 40s

Cell of origin: type 2 pneumocyte

Behavior: benign, though this rarely metastasizes to lymph nodes, no one dies of disease
Coccidioides immitis:
Transmission/geography
Organism morphology
Histology
Coccidioides immitis:
Transmission/geography: dust/soil inhalation in Southwestern US
Organism morphology: Spherules (30-100 um) containing endospores (2-5 um)
Histology: chronic necrotizing granulomatous nodules with intact or fragmented spherules
Histoplasma capsulatum:
Transmission/geography
Organism morphology
Histology
Histoplasma capsulatum:
Transmission/geography: inhalation of bird poop, Ohio River Valley and lower Mississippi River Valley
Organism morphology: 2-4 um intracellular (macrophage) organisms with narrow based asymmetric buds
Histology: necrotizing granuloma
Cryptococcus neoformans:
Transmission/geography
Organism morphology
Histology
Cryptococcus neoformans:
Transmission/geography: inhalation of pigeon poop, found worldwide
Organism morphology: 4-6 um with prominent polysaccharide capsule (India ink), asymmetric narrow based budding
Histology: granulomatous or minimal reaction
Blastomyces dermatitidis:
Transmission/geography
Organism morphology
Histology
Blastomyces dermatitidis:
Transmission/geography: inhalation of dust/soil, Southeastern (Mississippi River Valley) and Midwestern US
Organism morphology: 8-15 um spheres with broad based budding
Histology: suppurative and granulomatous
What are the manifestations of Aspergillus? (5)
1. Aspergilloma
2. Minimal/chronic invasive
3. Invasive
4. Allergic bronchopulmonary aspergillosis
5. Necrotizing tracheobronchitis
This giant cell pneumonia contains distinctive giant cells cannibalizing histiocytes within its cytoplasm.
Hard metal (cobalt and tungsten) pneumoconiosis
TSC1 and TSC2
Alternative names
Chromosome
TSC1
Alternative names:
- tuberous sclerosis protein 1
- haartin
Chromosome: 9

TSC2
Alternative names:
- tuberous sclerosis protein 2
- tuberin
Chromosome: 16
Cytopathic change:
Adenovirus
Smudge cell
Cytopathic change:
CMV
Enlarged cell with nuclear and cytoplasmic inclusions. Nuclear inclusion with halo.
Cytopathic change:
HSV
Multinucleation with nuclear inclusions (steel gray nuclei) with nuclear molding
Cytopathic change:
Measles
Large cells with multinucleation with nuclear and cytoplasmic inclusions (Warthin-Finkeldey cells)
Are ferruginous bodies specific for asbestos?
No, though ferruginous bodies indicate asbestos, not all ferruginous bodies are due to asbestos.
What is a chemodectoma?

What population is at risk?

What is the mechanism of development and is it a neoplasm?
Chemodectoma: minute pulmonary meningothelial-like nodule

Population: women

Mechanism: associated with states of chronic tissue hypoxia; is a neoplasm (clonality)
This lesion is characterized by alveolar spaces packed by pigmented macrophages. There may also be mild smooth muscle thickening. What is this lesion due to?
Respiratory bronchiolitis due to smoking

NOTE: symptomatic people have the clinical diagnosis of respiratory bronchiolitis-interstitial lung disease
This lesion characterized by grooved histiocytes is strongly associated with smoking and pneumothorax due to traction emphysema.
Langerhan's cell histiocytosis
What is another name for idiopathic bronchiolitis obliterans organizing pneumonia (BOOP)? What are some of the causes of BOOP? What is the treatment? Do people develop ILD?
BOOP = cryptogenic organizing pneumonia (COP)

Causes: adjacent lung mass, infection, rheumatologic, idiopathic
Treatment: steroids, responds well

ILD: not usually (no permanent scarring)
This medium vessel autoimmune vasculitis is characterized by eosinophilic granulomatous inflammation. Involved areas include lung, GI tract, and peripheral nerves. Patient's are P-ANCA positive.
Churg-Strauss
This systemic vasculitis, typically affecting skin, is characterized by deposition of immune complexes containing IgA. The disease is usually self-limited though 1% of people develop irreversible renal failure. One third of patients have relapse.
Henoch-Schonlein purpura
Wegener's granulomatosis, microscopic polyangiits, SLE, mixed cryoglobulinemia, Henoch-Schonlein purpura, antiphospholipid antibody syndrome, and Goodpasture's syndrome can all present with this in the lungs, histologically characterized by: alveolar walls with dense infiltrate of neutrophils associated with neutrophilic debris, prominent fibrin, capillaritis.
Diffuse pulmonary hemorrhage syndrome
This lesion is characterized by interstitial lymphocytic inflammation involving alveolar walls. The process is seen more commonly in children with AIDS, but may also be seen in adults with AIDS. It can be seen in autoimmune disease such as Sjogren's syndrome as well as medication reaction.
Lymphocytic interstitial pneumonia (LIP)
This lesion is characterized by airways whose lumens are completely obliterated and replaced by fibrous tissue. Causes include infections (e.g. adenovirus), autoimmune disease (e.g. RA), and medications especially penicillamine. This may occur in lung transplant patients over time and sometimes occurs with fume exposures. This process is sometimes idiopathic.
Constrictive bronchiolitis (AKA obliterative bronchiolitis)
This lesion, more commonly found in the bladdr, is grossly characterized by ulcer, plaque, or papule and microscopically contains Michaelis-Gutmann bodies admixed with histiocytes forming large areas of consolidation. This can be seen in immunosuppression and is asociated with Rhodococcus equi infection.
Malakoplakia
What special stain can be used to visualize Legionella pneumophilia?
Dieterle stain
Define:
Carcinoid tumorlet
Typical carcinoid
Atypical carcinoid
Large cell neuroendocrine carcinoma
Define:
Carcinoid tumorlet: less than 0.5 cm

Typical carcinoid: less than 2 mites per 10 HPF, no necrosis

Atypical carcinoid: 2-10 mites per 10 HPF, necrosis present

Large cell neuroendocrine carcinoma:
- NE differentiation by morphology, IHC, or EM
- Greater than 10 mites per 10 HPF
- Necrosis
- Non-small cell morphology
What is rounded atelectasis? What is it associated with?
Atelectasis formed from adhesion between a parietal pleural plaque and the visceral pleura resulting in progressive fibrosis and retraction entrapping (and folding up) lung parenchyma (rounded atelectasis).

Association: asbestos exposure