Pathology Lectures (Galbraith) Flashcards
(158 cards)
1
Q
what are common viruses implicated in infectious rhinits?
A
adenovirus
rhinovirus
echovirus
2
Q
what type of Ab-mediated hypersensitivity rxn is allergic rhinits?
A
IgE
3
Q
nasal polyps are edematous protrusions of the nasal mucosa and have a prominence of this cell type in addition to lymphocytes, plasma cells, and neutrophils:
A
eosinophils
4
Q
microbes from what anatomic location are commonly involved in sinusitis?
A
oral cavity
may be allergic sinusitis
5
Q
severe chronic sinusitis may caused or complicated by fungi (aspergilus or mucormycosis), particularly in these types of pts:
A
diabetics and immunocompromised pts
6
Q
sinusitis should be medically tx due to concern of what?
A
spread within the cranial vault. Not very frequent but can happen
7
Q
pharyngitis and tonsillitis are most commonly caused by these viruses:
A
adenovirus
echovirus
rhinovirus
8
Q
bacteria may be the primary cause or may cause superinfx in pharyngitis and tonsillitis and these bacteria are most common:
A
usually B-hemolytic streptococci (strep throat)
occasionally S aureus
9
Q
necrotizing ulceration of the URT may be caused by:
A
acute fungal infix
granulomatosis with polyangiitis
extranodal NK/T cell lymphoma, nasal type –> associated with EBV, male, 40-50’s, Asian and Latin american, aggressive
10
Q
what is the epidemiology of necrotizing lesions of the URT, Extranodal NK/T cell lymphoma, nasal type?
A
Associated with EBV
Male, 40-50’s, Asian and Latin american
aggressive
11
Q
nasopharyngeal angiofribromas are vascular tumors seen almost always in this demographic:
A
Adolescent males, esp red-haired and fair-skinned (gingers, no souls)
12
Q
where do nasopharyngeal angiofibromas typically arise?
A
posterolateral roof of nasal cavity
classified as benign tumor but may be locally aggressive and extend intracranially
13
Q
___ is a “benign” tumor arising from the reps mucosa of the nasal cavity and paranasal sinuses that occurs in M > F, ages 30-60
A
sinonasal (schneiderian) papilloma
14
Q
exophytic and inverted subtypes of sinonasal (schneiderian) papilloma are associated with this virus:
A
HPV subtypes 6 and 11
15
Q
___ is a papillomatous growth of squamous cell-lined fronds downward from the mucosal surface into the underlying stroma.
A
inverted sinonasal papilloma
if not completely excised, it may recur
16
Q
what is a compilation of an inverted sinonasal papilloma if it is not completely excised?
A
extension into the orbit or cranial vault
10% malignant transformation
17
Q
what type of tumor is an olfactory neuroblastoma?
A
small, round blue cell tumor
arises from neuroectoderm in superior nasal cavity
18
Q
what is the age distribution for an olfactory neuoroblastoma?
A
bimodal –> peaks at 15 and 50
19
Q
nasopharyngeal carcinoma is an ___-related carcinoma that is rare in the US, but more common in parts of africa in __ and China in __ (think age ranges).
A
EBV
Africa: children
China: adults
20
Q
which type of nasopharyngeal carcinoma is least radiosensitive? which one is more radiosensitive?
A
keratinized carcinoma is LEAST radiosensitive
undifferentiated carcinoma is MORE radiosensitive
21
Q
what pathogens can be implicated in laryngitis?
A
RSV
H influenza
B-hemolytic streptococci
22
Q
__ are smooth round small protrusions on the true vocal cords that arise in the setting of repeated vocal cord strain (singers) or heavy smokers that often leads to hoarseness and is considered benign
A
reactive nodules
23
Q
Squamous papillomas are squamous-lined fronds with fibrovascular cores that may be single or multiple and may occur in children and in adults. It benign but may recur and is caused by this pathogen:
A
HPV 6 and 11
24
Q
what type of carcinoma is laryngeal carcinoma and what gender, age, and predisposing factor is most likely?
A
squamous cell carcinoma
typically men in their 50s who smoke
25
what type of mass do you find with laryngeal carcinoma?
a bulky, fungating mass protruding from the laryngeal surface, often with ulceration
26
what is the prognosis of severe pulmonary hypoplasia?
fatal shortly after birth
27
this congenital anomaly is a segment of lung tissue without connection to the airway, and with systemic circulatory supply (not pulmonary)
pulmonary sequestration
28
__- refers to an area (or areas) of airless pulmonary parenchyma, d/t collapse or incomplete expansion
atelectasis
29
___ is a complete obstruction of an airway, air within the dependent lung is resorbed which leads to collapse, and the mediastinum shifts toward the affected lung
resorption
30
___ is fluid, tumor, or air accumulation within the pleural space, preventing normal expansion and the mediastinum shifts away from the affected lung
compression atelectasis
31
___ is pulmonary or pleural fibrosis preventing normal expansion and is not reversible
contraction atelectasis
32
This type of pulmonary edema is characterized by intra-alveolar fluid accumulation d/t increased hydrostatic pressure in the pulm circulation. Fluid accumulates basally at first, alveolar capillaries are congested, and an intra-alveolar transudate is seen which is pink and granular
hemodynamic pulm edema
33
what type of cells may be seen with chronic pulm edema?
hemosiderin-laden macrophages AKA heart failure cells
34
this type of pulmonary edema is caused by injury to and inflammation of alveolar vascular endothelium and/or resp epithelium. It can be due to infectious or toxic insults and may be localized or diffuse
edema secondary to microvascular (alveolar) injury
35
what are some predisposing conditions to acute lung injury (aka ARDS)?
infectious agents
physical injury
toxic substances
hemodynamic disturbances
sepsis, diffuse pulm infx, gastric aspiration, trauma account for > 50% of cases
36
list the steps in pathogenesis of acute lung injury
endothelial activation --> neutrophil accumulation and activation --> accumulation of intraalveolar fluid and hyaline membranes --> resolution of injury
37
what does acute lung injury look like grossly?
heavy, firm and wet lungs
38
what does acute lung injury look like microscopically?
congested, interstitial and intraalveolar edema, necrosis of type 1 and 2 pneumocytes, presence of hyaline membranes, collapse of some alveoli
39
What is the expected FEV1/FVC in obstructive lung diseases?
Decreased maximal flow rates during forced expiration --> FEV1/FVC < 0.7
40
What are some risk factors and at risk groups for COPD?
Women and AA's more susceptible
Strong association with smoking
41
This type of emphysema is the most common subtype, occurs predominantly in heavy smokers, often along with chronic bronchitis. The respiratory bronchioles are involved, sparing the alveoli.
Centriacinar emphysema
42
Where are lesions commonly found in centriacinar emphysema?
Upper lobes/apical segments
In the respiratory bronchioles, distal alveoli spared
43
What deficiency is panacinar emphysema associated with?
a1-antitrypsin deficiency
44
Where does panacinar frequently occur and which portions are involved?
Occurs more frequently in the lower and anterior aspects of the lung (bases are most severely involved)
Alveoli distal to respiratory bronchioles are involved
45
What sort of chemotactic factors do lung epithelial cells and macrophages release to recruit inflammatory cells from the circulation (emphysema pathogenesis) and what do inflammatory cells release that cause destruction?
IL-8
TNF
Inflamm cells release destructive proteases (elastase)
46
__ is a potent antiprotease encoded by the Pi locus on Chr 14
a1-antitrypsin
47
80% of Homozygotes for this allele will develop symptomatic panacinar emphysema, and will be accelerated and more severe if the pt smokes
PiZZ
Z allele, Pi locus Chr 14
48
When do emphysema pts typically experience symptoms? What are the initial symptoms? What about symptoms with severe emphysema?
No symptoms until 1/3 of lung tissue is affected
Initial: dyspnea, cough, wheezing
Severe: weight loss, barrel chest (overdistention), prolonged expiration, pink puffer d/t overventilating
49
What can emphysema progress to and what do these pts usually die from?
May progress to pulm HTN and RHF
Death usually d/t: CAD, resp failure, RHF, pneumothorax --> lung collapse
50
What is a characteristic pathogenic finding in chronic bronchitis?
Mucus hypersecretion
51
Describe some morphologic changes seen in chronic bronchitis:
Edema and swelling of resp mucosa, often w/ squamous metaplasia
Hyperplasia of submucosal mucous glands of tranches and larger bronchi (thickness of mucus gland layer increases) --> Reid index increases
Increased goblet cells in small bronchi and bronchioles, and extensive small airway mucous plugging
52
What is the typical clinical course and symptoms of a pt with chronic bronchitis?
Persistent productive cough
Dyspnea on exertion
Classically: Hypercapnia, hypoxia, mild cyanosis ("blue bloater")
53
Asthma is a chronic disorder of the __ airways
Conducting
54
What type of hypersensitivity rxn is atopic asthma? When is typical onset? What may be found in the pts serum?
Type I, IgE-mediated
Usually childhood
Pts may have high serum IgE
55
What helper T cell type is involved in atopic asthma? Which cytokines stimulate production of IgE? Which cytokine recruits eosinophils? Which cytokine stimulates mucus production?
Th2
IL-4, IL-13 --> stimulate production of IgE
IL-5 --> recruit eosinophils
IL-13 --> stimulates mucus production
56
What is the immediate phase (minutes) of atopic asthma characterized by?
Bronchoconstriction
Mucus secretion
Increased vascular permeability
57
What is the late phase (hours) of atopic asthma characterized by?
Recruitment of more inflamm cells (neutrophils, eosinophils, lymphocytes)
Results in damage to mucosal tissue
58
What are some causes of non-atopic asthma?
Respiratory viruses
Inhalation of irritants
Cold air
Exercise
59
Describe morphologic changes seen in asthma:
Airway remodeling which includes:
- bronchial wall smooth m. hypertrophy and hyperplasia
- subepithelial fibrosis
- submucosal gland hyperplasia; increased goblet cells
- increased airway vascularity
- increased thickness of the airway wall
60
What are morphologic changes seen in severe cases of asthma?
Bronchi and bronchioles become occluded by thick mucus plugs, which may be expelled in sputum or BAL specimens (Curschmann spirals)
Sputum and BAL specimens may also contain numerous eosinophils and Charcot-Leyden crystals (esp in atopic cases)
61
__ is chronic, recurrent necrotizing infx eventually destroy smooth m. and elastic tissue, leading to permanent dilation of bronchi and bronchioles
Bronchiectasis
62
What are some predisposing conditions to bronchiectasis?
Conditions affecting mucus clearing --> primary ciliary dyskinesia, cystic fibrosis; Immunodeficiency conditions
63
In bronchiectasis, repeated attempts to resolve the inflamm process may result in ___
Peribronchial fibrosis --> may be extensive enough to obliterate nearby bronchioles (Bronchiolitis obliterans)
64
What is the abnormal gene and which chromosome is it on for cystic fibrosis?
CFTR gene on Chr 7
65
CFTR normally inhibits __, found on epithelial cell surfaces (except sweat glands)
ENaC
A lack of functioning CFTR results in over-active ENaC, resulting in epithelia taking up Na ions and water passively following
66
What type of mucus would you expect in a CF patient?
Dehydrated but very thick mucous
67
The inability to clear mucus in CF can eventually lead to:
Chronic infx --> eventually bronchiectasis
68
What are some common bacterial species that can cause infx of the lungs in CF?
S aureus
H influenzae
B cepacia
P aeruginosa
69
This bacterial species can produce a mucoid capsule (Latinate) which allows it form a protective biofilm seen in CF pts:
Pseudomonas aeruginosa
70
In restrictive lung disease, interstitial fibrosis leads to:
Decrease in:
- diffusing capacity
- lung volume
- lung compliance
71
List the Fibrosing Restrictive diseases:
```
Idiopathic pulmonary fibrosis
Nonspecific interstitial pneumonia
Cryptogenic Organizing pneumonia
Pneumoconioses --> Coal workers, silicosis, asbestos-related, etc.
Drug rxns
Radiation pneumonitis
```
72
List the following in Idiopathic Pulmonary Fibrosis:
- Cause: ___
- who is affected: ___
- Sources of injury: ___
Cause is unknown but thought to occur secondary to repeated injury to alveolar epithelial cells with a pro-fibrotic response in genetically-susceptible
Occurs in older pts > 50
Sources include tobacco smoke, occupational irritants, viruses, persistent gastric reflux
73
what are the telomerase mutations seen in Idiopathic Pulmonary Fibrosis?
TERT and TERC
74
Describe the morphology in Idiopathic Pulmonary Fibrosis:
Forms a pattern known as Usual Interstitial Pneumonia (UIP)
Subpleural and interlobular septal fibrosis
Microscopically, fibrosis is patchy, variable in intensity and age --> early lesions show cellular fibroblastic foci
Honeycomb fibrosis with spaces surrounded by dense collagen, and lined with hyper plastic type II pneumocytes and bronchial epithelium
75
How does Idiopathic Pulmonary fibrosis initially present and how does it progress? Prognosis?
initially, gradually worsening dyspnea with exertion, with development of progressive hypoxemia, cyanosis and clubbing occurring late
76
What do you find histologically in Crptogenic Organizing pneumonia?
tufts, balls or plugs of immature fibroblastic tissue (MASSON BODIES) found within terminal bronchioles
no iterstitial fibrosis or honeycombing
77
how to pts with cryptogenic organizing pneumonia present and what is their prognosis?
present w/ cough and dyspnea
may resolve spontaneously or with course of steroids
78
in anthracosis, inhaled carbon is taken up by alveolar and interstitial macrophages which then accumulate in ___
lymphatics and lymphoid tissues
79
complicated coal workers pneumoconiosis (progressive massive fibrosis) is characterized by multiple anthracotic scars, which if extensive, may lead to:
- resp failure
- pulm HTN
- cor pulmonale
80
where can the nodular scars of silicosis occur and what do they look like on chest x ray?
scars may occur in lungs or hilar LNS
calcified LN's may sometimes be identified by xray
81
what do the nodules of silicosis look like microscopically, where do they tend to occur first in the lung?
nodules are formed of whorled balls of dense collagen fibers, surrounded by dust-containing macrophages. Silica particles are birefringent with plane-polarized light
Initially more prominent apically (start upper lung)
82
Silicosis leads to increased susceptibility to ___
TB
Silicosis pts have 2x risk of lung cancer compared to gen pop
83
which type of asbestos fiber is more pathogenic?
amphibole
84
what type of fibrosis is seen in asbestos-related lung disease? Which part of the lungs is affected first? What can be seen within macrophages?
interstitial fibrosis, pattern similar to UIP and may result in honeycomb lung
Lung bases affected 1st, progresses upwards
Asbestos (Ferruginous) bodies within macrophages
85
What is the clinical course of plaques in asbestos-related lung disease? What are some clinical signs and symptoms? What is the prognosis of asbestosis complicated by pulm or pleural malignancy?
Pleural plaques typically asymptomatic
Dyspnea is usually 1st manifeztation often accompanied by productive cough; lag of 10-20 yrs post-exposure
Grim prognosis
86
What are the Restrictive Granulomatous diseases?
Sarcoidosis
| Hypersensitivity pneumonitis
87
What is the epidemiology of sarcoidosis? which parts of the lung are often involved and what type of granulomas are found?
Younger adults, More common in females and 10x higher in AAs
Lung and or hilar LN involvement
Noncaseating granulomas
88
What HLA subtypes are associated with sarcoidosis?
HLA-A1 and HLA-B8
89
Describe the morphology of sarcoidosis:
- small, noncaseating granulomas, often with multinucleated giant cells (can see Schaumann bodies and Asteroid bodies)
- Granulomas distributred along pulmonary lympthatics
90
How can a sarcoidosis pt present? What is the prognosis? How do you dx?
Variable presentation
Constitutional --> weight loss, fever, night sweats, fatigue
Pulm--> dyspnea, hemoptysis, cough, pain
Most experience resolution, with or w/o steroids. Some can be slowly progressive, remitting, and relapsing
Dx established by biopsy demonstrating noncaseating granulomas
91
This restrictive disease is an immunologic rxn to inhaled organic ags, causing interstitial pneumonitis, noncaseating granulomas, and interstitial fibrosis
Hypersensitivity pneumonitis
92
Farmers lung, Pigeon breeders lung, and air conditioner lung are part of this restrictive disease:
hypersensitivity pneumonitis
93
What are the smoking-related interstitial diseases?
Desquamative interstitial pneumonia
| Respiratory bronchiolitis-associated interstitial lung disease
94
What is the morphology of desquamative interstitial pneumonia? Who is generally effected and what do they present with? Prognosis?
abundant intra-alveolar dusty brown (smokers) macrophages with mild interstitial inflammation and minimal fibrosis
M=F, 30's-40's, smokers, dry cough, dyspnea, clubbing
Usualyl respond to steroids
95
patchy bronchiolar accumulations of smokers macrophages with peribronchiolar inflammation and mild fibrosis is associated with this restrictive disease
Respiratory bronchiolitis-associated interstitial lung disease
96
What do pts with Respiratory bronchiolitis-associated interstitial lung disease present with and what is the tx?
cough and dyspnea in long-term smokers
tx with smoking cessation
97
Pulmonary langerhans cell histiocytosis is a rare disease characterized by focal collection of langerhans cells that cause progressive fibrosis. Who is most likely effected? What improves the condition? What acquired activating mutation can lead to disease progression?
> 95% of affected pts are young adult smokers
Smoking cessation leads to improvement most cases
serine-threonine kinase BRAF
98
What is the most common pathogenic case of Pulmonary Alveolar Proteinosis (PAP)?
Autoimmune --> 90% of cases. AutoAbs to GM-CSF
99
How do pts present with PAP? What does it look like histologically? What is the pathogenesis? How to tx?
Present with cough, producign sputum and gelatinous material
Alveoli are filled with granular pink precipitate, PAS+, lipid-laden protein exudates filling alveolar spaces
Surfactant accumulates within the lungs, making them enlarged and heavy
Whole lung lavage
100
What biomarkers can be elevated in bacterial pneumonia?
Hepatic acute phase reactants --> CRP and Procalcitonin
101
List some predisposing conditions to community-acquired bacterial pneumonia:
```
Age: young or old
Chronic disease (COPD, diabetes, CHF)
Absent splenic function (predisposes toward encapsulated bacterial infx)
```
102
This gram + diplococcus is the most common cause of community-acquired bacterial pneumonia
S. pneumoniae
103
Which encapsulated type of H influenza is most virulent? What is it an important cause of in pediatric age group? Chronic disease?
type b most virulent
pediatric bacterial pneumonia, meningitis, otitis media
most common cause of bacterial acute exacerbation of COPD
104
What can Moraxella catarrhalis cause in the elderly? Pediatrics?
elderly: bacterial pneumonia, exacerbation of COPD
peds : otitis media
105
This gram + org often complicates viral illnesses and has a high risk of abscess formation and empyema.
S aureus --> Also, IV drug abusers at high risk for Staph pneumonia in association with endocarditis
106
This org is the most common cause of gram negative bacterial pneumonia. Afflicts debilitated individuals, especially chronic alcoholics and malnourished
Klebsiella pneumoniae
107
This org is a common cause of nosocomial infix with propensity to invade blood vessels and spread systemically; Also common in CF and neutropenic pts
Pseudomonas aeruginosa
108
This org flourishes in artificial aquatic environments and spreads through aerosolization. Infx causes severe pneumonia in immunocompromised pt
Legionella pneumophila
109
Infx with this org is common in children and young adults; they occur sporadically as local epidemics (Schools, military camps, prison) and creates a walking pneumonia
Mycoplasma pneumonia
110
What are the 2 types of consolidation patterns seen in bacterial pneumonia and what is the morphologic change seen?
Bronchopneumonia and Lobar pneumonia
Invasion of bacteria leads to alveolar filling with inflame cells and exudate --> leads to consolidation of the lung tissue
111
Where does bronchopneumonia tend to occur in the lungs? How many lobes involved? One or both lungs?
basal
often multilobar
frequently bilateral
112
List the stage of inflammatory response in Lobar pneumonia:
___: vascular engorgement, cell-poor intra-alveolar fluid with bacteria
___: robust exudate with neutrophils, erythrocytes, fibrin
___: fibrinosuppurative material, erythrocyte disintegration, early organization
___: organizing fibrosis admixed with macrophages
congestion
red hepatization
grey hepatization
resolution
113
How can pts present with community-acquired bacterial pneumonia? What does the CXR show for lobar pneumonia? CXR for bronchopneumonia? What are complications?
abrupt fever, shaking chills, productive cough (rust-colored sputum)
Lobar: Opaque lobe
Bronchopneumonia: focal opacities
Complications: abscess, empyema, bacteremia
114
What are common viral orgs involved in community acquired viral pneumonia? Predisposing factors?
Influenza A, RSV, Human Metapneumovirus, adenovirus, rhinovirus
very young and elderly, malnutrition/alcoholism, chronic disease
115
Which viral protein from influenza virus binds to reap epithelial cells, allowing cell infix? Which viral protein allows release of newly-created visions?
Hemagluttinin (H1-H3)
Neuraminidase (N1-N2)
116
Antigenic drift is associated with ___
Antigenic shift is associated with ___
epidemics --> Drift is d/t acquired mutations in hemagluttinin or neuraminidase proteins
pandemics --> Shift is when proteins are replaced altogether by recombo of RNA segments of those animal viruses
117
Influenza virus generally involves which part of the reps tract? What are prominent findings? Clearing an established infix required __ cells and __ immune responses.
upper respiratory tract
vascular congestion, inflammation in alveolar wall interstitial tissue (edema, lymphocytes and macrophages)
Cytotoxic T cells and Innate immune responses
118
What type of virus is the Human metapneumovirus? Who gets effected?
paramyxovirus
causes bronchiolitis and pneumonia in very young, very old, and immunocompromised
119
What type of virus is involved in Severe Acute Respiratory Syndrome (SARS)? What can it infect?
coronavirus
has an ability to infect the lower resp tree and spread systemically
120
What are the most common orgs in Health care associated pneumonia?
Methicillin-resistant S aurues and P aeruginosa
121
Hospital-acquired pneumonia commonly involves these orgs:
Gram + cocci --> S aureus and S pneumoniae
Gram - rods --> Enterobacteriacea and Pseudomonas species
122
Aspiration pneumonia occurs in these types of pts: __. What type of pneumonia is it associated with and what is a common complication?
markedly debilitated or unconscious pts
pneumonia is often necrotizing and pursues a fulminant course
lung abscess a common complicaiton
123
What is the clinical course of a lung abscess?
complications include extension into the pleural cavity, hemorrhage, septic embolization, and secondary amyloidosis
124
This org can cause chronic pneumonia, is from inhaled soil particles contaminated by bird/bat droppings, in the US is endemic along the Mississippi and Ohio rivers (midwest). This org targets macrophages
Histoplasmosis (H. capsulatum)
Can cause: 1) Self-limited pulm infx; 2) Chronic progressive lung infx (apical, night sweats, fever, coughing); 3) extrapulm involvement, particularly the liver, adrenals, mediastinum, meninges; 4) wide dissemination
125
What type of granulomas are often found in histoplasmosis? How do you identify the org?
caseating
silver stain
126
This org can cause chronic pneumonia, is a soil-dwelling dimorphic fungus found in central and southern US. Symptoms can include productive cough, chest pain, headache, anorexia/weight loss, fever/chill, night sweats
Blastomycosis (B. dermatiditis)
127
List morphologic features of blastomycosis and what type of granuloma it forms:
Double wall and visible nucleus
Broad-based budding
Suppurative granulomas
128
This org can cause chronic pneumonia, is endemic in the southwestern US. It can cause lesions varying from pyogenic to granulomatous, and lung granulomas harbor giant cells containing large spherules filled with endospores
Coccidiomycosis (C. immitis)
129
This org causes San Joaquin valley fever (fever, cough, detectable lung granulomas, pleuritic pain, skin lesions such erythema nodusum and multiform)
Coccidiomycosis
130
List most common bacterial agents for pneumonia in immunocomproised:
Viral:
Fungal:
bacterial --> Pseudomonas, mycobacteria, legionella, listeria
viral --> CMV and herpesvirus
fungal --> Pneumocystis, Candida, Aspergillus
131
Who are candidates for lung transplants?
emphysema
idiopathic pulm fibrosis
CF
primary pulm HTN
132
This complication is seen in lung transplant pts in 50% of recipients by 3-5 years posttransplant
chronic rejection
133
what are survival rates at 1, 5, and 10 years post lung transplant?
1-79%
5-53%
10-30%
134
What are the tumor suppressor loci deletions in squamous cell carcinoma? What are strong associations with this malignancy and what is frequently mutated? Where does it most often arise?
3p, 9p --> site of CDKN2A gene
17p --> site of TP53 gene
Strong associations with tobacco smoke and high frequency of p53 mutations and over expression
most often arise in central lung/hilar region
135
This carcinoma of the lung has the strongest association with smoking, shows loss-of-function mutations in TP53 (75-90% of tumors), Rb (almost 100%), and MYC family gene amplification
Small cell carcinoma
136
Describe the malignancy of small cell carcinoma, where it tends to occur in the lung. What is found ultra structurally and IH stains?
the most malignant of lung cancers
presents as a central or hilar tumor
ultra structurally the cancer cells exhibit neurosecretory granules and IH stains usually demonstrate neurosecretory neurodendocrine markers
Most often produce paraneoplastic syndromes
137
Does adenocarcinoma occur in smokers or non smokers? Where do the lesions tend to be in the lung and what is a characteristic of the lesion? What type of mutations?
can occur in either
peripherally with characteristic gland formation, usually producing mucin
Gain of function involving GF receptor pathways --> EGFR, ALK, ROS, MET, RET
138
lung cancers in never smokers most commonly occur in what gender and are what type of carcinoma? What type of mutations?
most commonly in women and more commonly adenocarcinomas
more likely to have EGFR mutations almost never have KRAS mutations
139
What can you immunostain for small cell carcinomas?
chromogranin, synaptophysin, CD57+
140
Describe the morphology of small cell carcinoma
tumor cells are "small" with little cytoplasm, closely arranged nuclei with "molding" and absent nucleoli
cells grow in clusters, no architectural pattern
necrosis may be marked
141
what are favored metastatic sites of lung carcinoma?
adrenal, brain, liver, and bone
any type of lung cancer may spread to the pleural space, may spread hematogenously or within lymphatics
142
what type of mutation in lung carcinoma is associated with a poor prognosis?
KRAS
143
what is mean survival of untreated small cell carcinoma?
6-17 weeks --> still sensitive to radiation and chemo
144
a carcinoid tumor is part of a spectrum of tumors arising from this type of proliferation:
List the epidemiology and grade of malignant neoplasm
Neuroendocrine proliferation --> neuroendocrine tumorlets, carcinoid tumors, small cell carcinoma
1-5% of all lung tumors, M=F, < 40
low grade malignant neoplasm--> can have typical and atypical (more aggressive) forms
145
Where are carcinoid tumors likely to be located?
may be central or peripheral
central carcinoids often protrude into the bronchial lumen
146
what are atypical carcinoids more likely to invade? What are 5 yr survival rates for typical and atypical?
lymphatic vessels
typical=95%
atypical=70%
147
Carcinoid syndrome includes flushing, diarrhea, cyanosis and is caused by tumor cells secreting ___
vasoactive amines (serotonin)
148
what is the most common growth pattern in tumors metastatic to the lung and what are the common primary sites prior to metastasis to the lung?
most common growth pattern is multiple, scattered nodules
breast, colon, kidney, prostate, urinary bladder
149
what chromosomal aberrations are associated with hamatomas?
6p21 or 12q14-q15
150
This tumor is a pulm disorder that primarily affects young women of childbearing age. It is characterized by a proliferation of perivascular epithelioid cells that express markers of both melanocytes and smooth muscle cells. Lesional epithelioid cells frequently harbor loss of function mutations in the TSC2. Pts most commonly present with dyspnea or spontaneous pneumothorax
Lymphangioleiomyomatosis
151
This tumor is rare and more common in children with an equal M:F ratio. Pts present with fever, cough, chest pain, and hemoptysis but may also be asymptomatic. Imaging shows single round, well-defined, usually peripheral mass with Ca deposits in 1/4 cases. Some have activating rearrangements of ALK gene on Chr 2p23
Inflammatory myofibroblastic tumor
152
Name the type of inflammatory pleural effusion:
__: reflects pulm inflamm (TB, pneumonia, infarcts, abscesses) or systemic diseases (RA, uremia).
__: usually reflects pleural space infx leading to pus accumulation
__: occurs with bleeding disorders, neoplastic involvement, and certain rickettsial diseases
serofibrinous pleuritis
suppurative pleuritis (empyema)
hemorrhagic pleuritis
153
What are some noninflammatory pleural effusions?
hydrothorax
chylothorax
hemothorax
154
What is a potentially fatal complication of pneumothorax?
Progressively increasing pleural pressure compresses the contralateral lung and mediastinal structures
155
This noninvasive, fibrosing, rarely malignant tumor is composed of spindle cells resembling fibroblasts .They are associated with Chr 12 inversion creating a NAB2-STAT6 fusion gene. Resection usually curative
Solitary fibrous tumor (pleural tumor)
156
What is the most common mutational abnormality in malignant mesothelioma? What is its relationship to smoking?
Homozygous deletion of p16 (CDK2NA/INK4a), seen in 80%
Increased incidence with asbestos exposure, NOT compounded by smoking
157
What are the microscopic patterns of malignant mesothelioma?
Epithelioid (60%): resembling adenocarcinomas; Antigenic (Calretinin, WT-1, and CK5/6+) and ultra structural (Long, slender microvilli) allow distinction from adenocarcinoma
Sarcomatoid (20%): malignant, spindle-shaped cells resembling fibrosarcoma
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how do pts present with malignant mesothelioma? What is the survive rate?
chest pain, dyspnea, recurrent pleural effusion
Highly malignant and can invade lung and metastasize widely
1 yr survival ~50%, most don't survive 2 years
