Rosai Chapter 10 - Lung Flashcards
Main proliferating component after alveolar injury
cuboidal type II (granular) pneumocytes
Main types of bronchial and bronchiolar epithelial cells (6)
- Basal cells
- Neuroendocrine cells
- Ciliated cells
- Serous cells
- Clara cells
- Goblet cells
Which of the main types of bronchial and bronchiolar epithelial cells decrease in number as one approaches the terminal bronchioles? (2)
- Ciliated cells
- Goblet cells
Which of the main types of bronchial and bronchiolar epithelial cells increases proportionally in number as one approaches the terminal bronchioles?
-Clara cells
Represent the main progenitor cells after bronchiolar injury and has a secretory function
-Clara cells
Submucosal glands in larger bronchi is composed of:
-both Serous and Mucous cells
Structures sometimes seen in alveolar spaces that are of no diagnostic significance (4)
“CARB”
- Corpora amylacea (common in elderly)
- Alveolar macrophages (pigmented in smokers)
- fresh RBCs
- Blue bodies (Calcium carbonate)
Pulmonary ARTERY or VEIN:
Has internal and external elastic membrane
Pulmonary Artery
Pulmonary ARTERY or VEIN:
Has single (outer) elastic layer
Pulmonary Vein
Basic facts needed to know before diagnosing lung biopsy (6)
- patient Age
- whether or not the patient is immunocompromised
- onset and tempo of disease progression (Acute or Chronic)
- localized vs. diffuse radiological distribution of abnormalities
- presence and degree of functional impairment
- occupational or travel history
The type of lung biopsy that has the highest sensitivity, specificity, and accuracy for all non-neoplastic lung diseases
Wedge biopsy
Histologic findings that complicate interpretation of transbronchial biopsy (3)
- “Holes” or “Bubbles” artifact that mimics the appearance of exogenous lipid pneumonia
- procedure-related hemorrhage which is common
- mesothelial cells when the biopsy unintentionally captures pleural tissue
Most common types of congenital cystic disease encountered in surgical pathology (4)
- Congenital Lobar Overinflation (Congenital Lobar Emphysema)
- CCAM / CPAM
- Bronchogenic cyst
- Pulmonary sequestration
Characterized by the presence of variously sized intercommunicating cysts lined by cuboidal-to-ciliated pseudostratified columnar (“adenomatoid”) epithelium
-CCAM / CPAM
Stocker types of CCAM / CPAM is based on __ and __
- Size
- Number of cysts
Stocker type:
-Large cyst (>2 cm)
Stocker type 1
Stocker type:
-Small cyst (<2 cm)
Stocker type 2
Most common form of acquired cystic disease
-Emphysema
Defined as an increase in size of airspaces distal to the terminal bronchiole associated with destruction of their walls
Emphysema
Most important morphologic substrate of COPD
Emphysema
Large (>/= 1 cm) cystic spaces covered by a thin, stretched pleura
Bullae
Formed by the rupture of an alveolus directly beneath the pleura and the escape of air into the areolar layer of the pleura which results in interstitial pneumonia
Blebs
Size of Blebs
< 1 cm in diameter
Characterized by:
- Severe emphysema
- Bronchiectasis
- Bronchiolitis obliterans
Swyer-James (McLeod) syndrome
Severe emphysema in Swyer-James (McLeod) syndrome is occasionally accompanied by:
Placental transmogrification
Rare genodermatosis associated with an increased risk of renal and colonic neoplasia
Birt-Hogg-Dube syndrome
Pulmonary component of Birt-Hogg-Dube syndrome is composed of:
Pleuropulmonary blebs and cysts (usually basilar)
Rare form of localized cystic lung malformation in which connective tissue septa contain cartilaginous islands
Chondroid cystic malformation
Chondroid cystic malformation is due to:
trisomy 8 mosaicism
Characterized by partial or complete separation of a portion of a lobe of the lung with a systemic arterial supply often affiliated with no connection to the functional components of the tracheobronchial tree (bronchial atresia)
Bronchopulmonary sequestration
Key to diagnosis of intralobar variety of Bronchopulmonary sequestration
surgeon’s identification of a large elastic artery representing systemic vascular connection
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
aberrantly located pulmonary mesenchyme as an extrapulmonary island of lung tissue enveloped by its own pleural covering
Extralobar
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
any level from the thoracic inlet to the diagphragm or even within the abdominal cavity
Extralobar
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
Left side
BOTH
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
Associated with:
- diaphragmatic hernias
- polyhydramnios
- edema
Extralobar
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
more heterogeneous
Intralobar
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
Bronchial atresia with systemic vascular connection
Intralobar
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
Symptomatic
Intralobar
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
within the Lower lobe
Intralobar
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
Single cyst, multicystic area, or solid mass
Intralobar
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
Artery has an elastic pulmonary type
Intralobar
EXTRALOBAR / INTRALOBAR
Bronchopulmonary sequestration:
Microscopically - Nonspecific, Chronic inflammation, and Fibrosis
Intralobar
a condition characterized by fixed radiological abnormalities limited to the right middle lobe and/or lingula
Middle lobe syndrome
A syndrome characterized by:
- Chronic sinonasal infection
- Frequent bronchiectasis
- complete sinus inversus
- infertility
Kartagener or Immotile cilia syndrome (primary ciliary dyskinesia)
A syndrome characterized by:
- Chronic sinonasal infection
- Frequent bronchiectasis
- Infertility caused by azoospermia but lacking ultrastructural ciliary abnormalities
Young syndrome
LOCALIZE / DIFFUSE
Bronchiectasis:
partial or total obliteration of the bronchial lumen by a neoplasm, foreign body, localized inflammatory process, inspissated mucus, or external compression
Localize
LOCALIZE / DIFFUSE
Bronchiectasis:
Any area of Lung
Localize
LOCALIZE / DIFFUSE
Bronchiectasis:
If source of compression is relieved at an early stage, there is regression
Localize
LOCALIZE / DIFFUSE
Bronchiectasis:
Irreversible if microscopically has secondary inflammation and fibrotic changes
Localize
LOCALIZE / DIFFUSE
Bronchiectasis:
associated with Middle lobe syndrome
Localize
LOCALIZE / DIFFUSE
Bronchiectasis:
consequence of inflammation and post-inflammatory destruction of airway walls that is usually the result of repeated episodes of infection
Diffuse
LOCALIZE / DIFFUSE
Bronchiectasis:
Associated with
- Cystic fibrosis
- Kartagener syndrome
- Young syndrome
Diffuse
Most common locations of lung abscesses in surgical series (3)
- Right lower lobe
- Right upper lobe (particularly SUBAPICAL segment)
- Left lower lobe
*in decreasing order
Most common causes of lung abscesses in children (3)
- Staphylococcus aureus
- Streptococcus sp.
- Klebsiella pneumoniae
Second only to HIV as a cause for fatal infection in the developing world and remains a major global public health challenge
Tuberculosis
Solitary lung nodules seen in adults due to reinfection by tuberculosis
Tuberculomas
Infection due to MAC in an elderly woman that may resemble the middle lobe syndrome
Lady Windermere syndrome
A syndrome resembling hypersensitivity pneumonia resulting from exposure to hot tubs contaminated with nontuberculous mycobacteria
Hot tub lung
Presentations of Sarcoidosis in the thoracic cavity (5)
- moderate to marked perihilar lymph node involvement without pulmonary disease
- diffuse pulmonary disease without radiographic evidence of node involvement
- a combination of lymph node enlargement and diffuse pulmonary disease
- Pulmonary interstitial fibrosis
- Localized bronchostenosis with distal bronchiectasis and atelectasis
Hallmark of Sarcoidosis
compact non-caseating granuloma composed of:
- Epithelioid cells
- Langhans giant cells
- Lymphocytes
Pulmonary disease characterized by extensive vascular granulomas that infiltrate and occlude pulmonary artery and vein and are accompanied by widespread necrosis of lung tissue
Necrotizing sarcoid granulomatosis
Main characteristics of Granulomatosis with Polyangiitis (GPA) / Wegener granulomatosis (4)
- necrotizing granulomatous inflammation
- necrotizing vasculitis
- c-ANCA
- small-to-medium size vessels
Main morphologic change in the lungs of patients with GPA
necrotizing granulomatous inflammation grossly resembling other localized necrotizing processes
CLASSIC / LIMITED (LOCALIZED)
GPA:
Triad of:
- necrotizing angiitis
- Aseptic necrosis (involving both URT and the lungs)
- focal glomerulitis
Classic
CLASSIC / LIMITED (LOCALIZED)
GPA:
other vessels may be involved
Classic
CLASSIC / LIMITED (LOCALIZED)
GPA:
responsive to cytotoxic drugs (i.e. Cyclophosphamide)
Classic
CLASSIC / LIMITED (LOCALIZED)
GPA:
confined to the Lungs
Limited (Localized)
CLASSIC / LIMITED (LOCALIZED)
GPA:
No renal involvement
Limited (Localized)
CLASSIC / LIMITED (LOCALIZED)
GPA:
more protracted clinical course
Limited (Localized)
CLASSIC / LIMITED (LOCALIZED)
GPA:
Steroids and Cytotoxic drugs are both highly effective
Limited (Localized)
Main characteristics of Eosinophilic Granulomatosis with Polyangiitis (EGPA) / Churg-Strauss (3)
- combination of necrotizing granulomatous inflammation, eosinophilia, and necrotizing vasculitis
- small-to-medium size vessels
- associated with p-ANCA and MPO
A disease occurring almost exclusively in asthmatics and patients with cystic fibrosis in whom the bronchocentric granulomatosis is usually accompanied by eosinophilic pneumonia and mucoid impaction of bronchi
Allergic bronchopulmonary aspergillosis
This is a complex group of non-neoplastic pulmonary diseases that often require correlation of morphologic, clinical, and radiological findings
Diffuse Interstitial Lung disease
Comprise the largest category of DILD and account for over 80% of patients with idiopathic interstitial pneumonia who undergo surgical lung biopsy
Chronic Fibrosing Interstitial Pneumonias
Most common of the idiopathic interstitial pneumonias, accounting for nearly 2/3 of patients who undergo biopsy for diffuse lung disease of unknown cause
Usual Interstitial Pneumonia (UIP)
a syndrome that classically has an insidious onset characterized by a combination of breathlessness and cough with a relentlessly progressive evolution, many of the patients dying of respiratory failure after 3-4 years
Idiopathic Pulmonary Fibrosis (IPF)
Common final pathway in patients with underlying UIP and is characterized by sudden worsening of symptoms and more rapid progression of respiratory failure
Acute exacerbation
Single most important feature that distinguishes UIP from the other interstitial pneumonias
Marked regional variation in the nature and degree of the fibrosis with a distinct patchwork distribution and evidence of architectural derangement as evidenced by:
- scarring
- Honeycomb change (in distal collapsed fibrotic lung)
Characteristic of UIP but are not specific
Fibroblast foci
2nd most common finding in patients with interstitial pneumonia of unknown cause
Non-specific Interstitial Pneumonia (NSIP)
Main morphologic difference of UIP vs. NSIP
Based on pattern of lung involvement:
- UIP (heterogeneous)
- NSIP (lacks heterogeneity)
FIBROTIC / CELLULAR
NSIP:
Alveolar septa in NSIP is expanded by an inflammatory infiltrate WITH collagen fibrosis
Fibrotic NSIP
FIBROTIC / CELLULAR
NSIP:
Alveolar septa in NSIP in expanded by an inflammatory infiltrate WITHOUT collagen fibrosis
Cellular NSIP
Characterized by an accumulation of lightly pigmented alveolar macrophages within respiratory bronchioles spilling into neighboring alveoli
Respiratory bronchiolitis
Symptomatic RB
Respiratory bronchiolitis-interstitial lung disease (RBILD)
RB/RBILD vs. Desquamative interstitial pneumonia (DIP):
Histiocytic accumulation is Diffuse
RB/RBILD
RB/RBILD vs. Desquamative interstitial pneumonia (DIP):
with an associated interstitial pneumonia
RB/RBILD
RB/RBILD vs. Desquamative interstitial pneumonia (DIP):
Histiocytic accumulation is Centriacinar
DIP