Robbins and rosai Flashcards
Histologic manifestation of Acute lung injury(ALI)/acute respiratory distress syndrome(ARDS)
Diffuse alveolar damage
Appearance of acute lung injury in the absence of known triggers and follows a rapid progressive clinical cause
Acute interstitial pneumonia
4 events in the pathogenesis of diffuse alveolar damage(dad)
- Endothelial activation
- adhesion and extravasation of neutrophils
- accumulation of intra-alveolar fluid and formation of hyaline membranes
- resolution of injury
Characteristically lines the alveolar walls in DAD
Hyaline membranes
congenital lesion characterized by the presence of variously sized intercommunicating cysts lined by cuboidal-to-ciliated pseudostratified columnar (‘adenomatoid’) epithelium
Congenital cystic adenomatoid malformation (CCAM)
refers to the permanent dilation of bronchi usually associated with destruction of some elements of the bronchial wall and inflammatory changes that extend into the surrounding or distal lung parenchyma.
Bronchiectasis
a condition characterized by fixed radiological abnormalities limited to the right middle lobe and/or lingula
Middle lobe syndrome
autosomal recessive disease causing mutations result in ciliary dysfunction due to defects in ciliary motor proteins preventing mucociliary clearance, setting the stage for recurrent infections that lead to bronchiectasis
Primary ciliary dyskinesia
hallmark of the disease is a compact non- caseating granuloma composed of epithelioid cells, Langhans giant cells, and lymphocytes
Sarcoidosis
most common of the idiopathic interstitial pneumonias and has marked regional variation in the nature and degree of the fibrosis, with a distinct patchwork distribution and architectural derangement in the form of scarring and honeycomb change
Usual interstitial pneumonia (UIP)
A smoking related diffuse lung disease characterized by an accumulation of lightly pigmented alveolar macrophages within respiratory bronchioles spilling into neighboring alveoli
respiratory bronchiolitis- interstitial lung disease (RBILD)
a smoking related, mild, paucicellular fibrosis in the form of lamellar eosinophilic collagenous thickening of alveolar septa in a patchy, mainly subpleural distribution
smoking- related interstitial fibrosis (SRIF)
second most common finding in patients with interstitial pneumonias of unknown cause. findings may be patchy or diffuse but retain a degree of qualitative uniformity in areas of abnormality
nonspecific interstitial pneumonia (NSIP)
a rapidly progressive form of idiopathic interstitial pneumonia and is synonymous with the Hamman–Rich syndrome
Acute interstitial pneumonia (AIP)
represented by fibroblastic plugs (‘Masson bodies’) filling air spaces
organizing pneumonia
acute lung injury secondary to nitrogen dioxide inhalation and is characterized by the presence of DAD without granulomatous inflammation
Silo-filler’s disease
T/F Langerhans Cell Histiocytosis occurs almost exclusively in cigarette smokers
True
Immunohistochemically Langerhans cells can be highlighted by (give 3)
S-100, CD1a, or langerin
mutation associated with LCH
BRAFV600E mutations
histiocytic disorder which can affect the lung, in conjunction with bones, soft tissues, and the central nervous system characterized by an infiltrate of foamy histiocytes, lymphocytes, and Touton giant cells
Erdheim–Chester disease
characterized by silica associated micronodular scars with a characteristic pattern of central lamellar fibrosis distributed in a bronchiolocentric pattern with a cellular periphery in which dust-laden macrophages predominate
Silicosis
non-neoplastic reaction of the lungs to inhaled mineral or organic dust
Pneumoconiosis
the changes are mainly represented by BOOP, usually in combination with multinucleated giant cells, acute bronchopneumonia and/or bronchiolitis, and suppurative granulomas. The key finding is the presence of foreign material
Aspiration pneumonia
characterized by accumulation of an amorphous eosinophilic (but sometimes basophilic) PAS- positive material of predominantly phospholipid nature in the alveolar lumina, associated with a minimal infiltrate of lymphocytes, macrophages, and desquamated pneumocytes.
Pulmonary alveolar proteinosis
The most reliable stain for detecting the organism P. jirovecii in lung specimens
GMS
most common cause of cancer mortality worldwide
Lung cancer
most common histologic subtype of lung cancer in never-smokers
Adenocarcinoma
gain-of-function mutations in receptor tyrosine kinases genes in lung adenocarcinoma (5)
EGFR, ALK, ROS1, MET, RET
gain-of-function mutations in serine/threonine kinases genes in lung adenocarcinoma
BRAF, PI3K
Frequently mutated in Lung squamous cell carcinoma
CDKN2A (p16) and TP53 (p53)
is virtually always smoking related and has the highest mutational burden among lung cancers
Small cell carcinoma
almost universal inactivation of both genes in small cell lung carcinoma
Tp53 and RB
Lung cancers in nonsmokers are more likely to have this mutation
EGFR
Four types of morphologic precursor epithelial lesions for lung cancer
- atypical adenomatous hyperplasia
- adenocarcinoma in situ
- squamous dysplasia and carcinoma in situ
- diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
Common location in the lung of the adenocarcinoma subtype of lung cancer
peripheral lung
Common location in the lung of the squamous cell carcinoma subtype of lung cancer
central/hilar
a small precursor lesion (≤5 mm) characterized by dysplastic pneumocytes lining alveolar walls that are mildly fibrotic. It can be single or multiple and can be in the lung adjacent to invasive tumor or away from it.
Atypical adenomatous hyperplasia
Lung lesion that is less than 3 cm in size and is composed entirely of dysplastic cells growing along pre-existing alveolar septa. The cells have more dysplasia than atypical adenomatous hyperplasia and may or may not have intracellular mucin
Adenocarcinoma in situ
lung adenocarcinoma tumors (≤3 cm) with a small invasive component (≤5 mm) associated with scarring and a peripheral lepidic growth pattern are called
Minimaly invasive lung adenocarcinoma
Basophilic staining of vascular walls due to encrustation by DNA from necrotic tumor cells is frequently present in what lung cancer subtype and what is this effect called
Small cell cancer and Azzopardi effect
syndrome that causes venous congestion and edema of the head and arm and, ultimately, circulatory compromise
superior vena cava syndrome
T Staging System for Lung Cancer T1m, T1a T1b T1c, T2a, T2b, T3, T4
T1m - minimally invasive (</=3cm, invasive comp </=5mm)
T1a - <1cm
T1b - 1-2cm
T1c - 2-3cm / main stem bronchus
T2a - 3-4
T2b - 4-5cm / main stem bronchus
T3 >5-7cm / parietal pleura, chest wall
T4 - >7cm / mediastinum, heart, recurrent laryngeal nerve
small cell carcinomas usually produce what hormones?
ADH and ACTH
squamous cell carcinomas usually produce what hormone and thus causes what?
calcitonin - hypocalcemia
tumors grow as finger-like or spherical polypoid masses that commonly project into the lumen of the bronchus and are usually covered by an intact mucosa composed of organoid, trabecular, palisading, ribbon, or rosette-like arrangements of cells separated by a delicate fibrovascular stroma
Carcinoid tumor
Typical carcinoids have fewer than ___ mitoses per 10 high-power fields and lack necrosis, while atypical carcinoids have between _____ mitoses per 10 high-power fields and/or foci of necrosis
2, 2-10
fusion gene that appears to be virtually unique to solitary fibrous tumor
NAB2-STAT6 fusion
this mutation occurs at a significantly higher frequency in adenocarcinomas from East Asians than from non-Asians, from women than from men, and from never-smokers than from ever-smokers
EGFR
____translocated pulmonary adenocarcinomas show the following characteristics: occurrence in never-smokers or light smokers, younger patient age, and lack of mutations in the EGFR, KRAS, and TP53 genes. These tumors tend to show a solid growth pattern, often with signet ring cell morphology.
ALK
most common mutations in lunc scca
TP53 and CDKN2A
most common of the neuroendocrine lung tumors
Typical carcinoid tumor
most common primary malignant lung neoplasm in children
Typical carcinoid tumor
driver mutation in carcinoid tumors
MEN1/PSIP1
adenosquamous carcinoma is used for lung tumors in which distinct areas of squamous and glandular differentiation are found in the same neoplasm, each accounting for at least __% of the sampled tumor
10%
characterized by the presence of a well-differentiated adenocarcinomatous component in a cellular stroma composed of undifferentiated small (‘blastematous’) spindle cells
Pulmonary blastoma
gene fusion implicated in Adenoid cystic carcinoma
MYB oncogene and NFIB
gene fusion implicated in Mucoepidermoid carcinomas
MECT1–MAML2 fusion
