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143 Cards in this Set
- Front
- Back
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where does wegener's granulomatosis target
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Nose, lung, kidney
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histologic fx of wegener's
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leukocytoclastic vasculitis with geographic necrosis, lymphocyte poor granulomatous reaction, palisaded histiocytes adn ulceration
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what is eosinophilici angiocentric fibrosis
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perivascular onion skin fibrosis and mixed inflammatory infiltrate with eos in mucosa of upper respiratory tract
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different types of sinonasal papillomas
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cylindrical cell, inverted,everted, oncocytic features, transititional, squamous and schneiderian (excluded from this group is the hyperkeratotic squamous papillomas of the nasal vestibule - more like skin lesions)
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can malignancy involve a sinonasal papilloma?
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yes - one of three ways (1. post excision; 2. focus of invasion at initial resection; 3. it really is more of a cancer to begin with but is just subtle)
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most common hpv in sinonasal papillomas
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hpv 6/11
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where do intranasal carcinomas most commonly arise
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vestibule and lateral wall (not usually septum)
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where do paranasal sinus tumors most often arise
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ethmoid sinuses
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what is a cylindrical cell carcinoma
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carcinoma related to squamous cell ca but has foci of intracellular mucin production (transitional; between mucin producing and squamous epithelium)
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most important prognostic indicator for sinonasal carcinoma
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tumor stage
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prognostic factors for nasopharyngeal ca
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patient age, stage, location of regional mets (upper necks/homolateral spread is better); worse prognosis for keratinizing (which for these, worse iflacck of lymphocytes, high microvessel count, c-erb B2 expression high density of S100 positive dendritic cells, others obvious)
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bone defects occur with which: encephalocele or glial heterotopia
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encephalocele
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ihc for neuroblastoma
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NSE, chromogranin, neurofilaments, Hu, keratin, patchy s100, scattered GFAP
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ihc for nk/t cell lymphoma, sinonasal type
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CD2, CD56+ (NCAM); CD3-; no clonal rearrangement of T cell receptor gene
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most common pathologic diagnosis behind lethal midline granuloma
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NK/T cell lymphoma
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alternate name for CD56
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NCAM
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hemangiopericytoma-like tumor ihc
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vimentin, focal reactivity for actin
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alternate name for masson tumor
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papillary endothelial hyperplasia
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pediatric nasopharyngeal malignancies
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nasopharyngeal carcinoma, lymphoma, rhabdomyosarcoma, embryonal type
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follicular dendritic cell tumor vs. nasopharyngeal ca in nasal cavity
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nucleoli are smaller in FDCT, and FDCT are CD21+, +/-CD35+ and negative for keratin
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causes of mycotic laryngitis in US
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blastomycosis and histoplasmosis
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granulomatous lesion of anterior larynx or having associated oral lesion is most likely caused by what?
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histoplasmosis
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hpv types associated with juvenile laryngeal papillomas
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HPV 6 and 11 (also true for adult type too)
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molecular features of laryngeal ca
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p53 overexpression in majority; absence of p16
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site of laryngeal ca with best prognosis
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glottic
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site of laryngeal ca with worst prognosis
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subglotti
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significance of aneuploidy in laryngeal ca?
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increased risk of recurrence
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where in larynx would a carcinoid tumor or even an atypical carcinoid or large cell neuroendocrine carcinoma be found
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supraglottic region
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Histochemical stains for mesothelioma
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Alcian blue or colloidal iron to show hyaluronic acid production; mucicarmine negative in cytoplasm
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IHC for meso vs. adeno ca
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In both: pankeratin, ema, basement membrane components, S11
in adeno ca (not meso): CEA*, CD15, B72.3*, Ber-Ep4, Bg8, MOC31*, TTF1 in meso (not adeno ca): Calretinin*, WT1*, keratin 5/6*, thrombomodulin, vimentin |
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variants of mesotheliomas
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spindle cell or sarcomatoid; desmoplastic, lymphohistiocytoid, deciduoid, malignant meso with squamous differentiations (SCCA), small cell (NSE+, Leu7+)
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prognostic factors in mesotheliomas
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stage, gender, type (spindle cell and desmoplastic are worse; deciduoid is better)
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which pleura is SFT usually attached to
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visceral
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ihc for SFT
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CD34, bcl2, vimentin, (+/- desmin)
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metastatic ca to pleura - what organs are involved and in order of frequency
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most common are lung, breast and stomach
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types of congenital cysts in lung
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pulmonary sequestration, congenital lobular emphysema, bronchogenic cyst, congenital bronchiectasis and cystic adenomatoid transformation
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syndrome in which cysts of the lung can be found
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Ehlers-Danlos syndrome
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histo fx of bronchiectasis
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dilated bronchial lumina with CI in bronchial wall
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tumorlets (nondular hyperplasia of Kulchitsky-type neuroendocrine cells) can be found in association with what?
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saccular bronchiectasis
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classic triad of wegener's
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necrotizing angiits, aseptic necrosis of upper respiratory tract and lung and focal glomerulitis
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first histo fx of DAD
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edema, intraalveolar hemorrhage and fibrin deposition
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middle stages of DAD - histo fx
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hyaline membrane formation (worst 3-7 days in); sparse interstitial inflammation,possibly fibrin thrombi and type II pneumocyte hyperplasia.
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histo fx of final stages of DAD
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interstitial and intraluminal fibroblastic proliferation, type II pneumocytic hyperplasia
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Acute interstitial pneumonia alternative name
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hamman rich syndrome - eventually looks like final stages of DAD
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histo fx of boop
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fibroblastic plugs; macrophages, scattered pmns, thickening of alveolar septae, PATCHY
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uip
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autoimmune (circulating immune complexes, aberrant wnt/beta-catenin pathway - temporal and geographic heterogeneity, 5 years progressive decline, not steriod responsive
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dip
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filling of alveolar spaces by large mononuclear cells with relatively little interstitial changes; radiographically - ground-glass bilaterally in the periphery
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LIP histo fx
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lymphocytes in interstitium
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LIP associated syndrome, good or bad steroid response?
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Sjogren's, poor steroid response
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microorganisms commonly associated with pneumonia
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H. influenza and S pneumo
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nocardia microscopic appearance
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branching filamentous bacilli,slightly beaded
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Langerhans' cell histiocytosis - demographics
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20-30s, smoking
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Langerhans' cell histiocytosis - ihc
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S100+, HLA-DR+, CD1a+ (remember birbeck granules)
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erdheim-Chester disease
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histiocytic disorder, affects lungs, bones and CNS; primarily septal pattern with touton giant cells, CD68+, negative for CD1a
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location of tumor resulting in pancoast syndrome and horner's syndrome (restate symptoms)
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apex of lung
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lung ca associated with hyperparathyroidism
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scca
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rough percent of lung adenoca that are TTF1 negative
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25%
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molecular changes in lung ca
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FHIT, p53, p16. recall p63 is usually present by ihc.
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micropapillary growth pattern in pulmonary adenoca confers a worse or better prognosis
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worse
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what ihc stain suggests glandular differentiation in lung cancers
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ck 7
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another name for pe10
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surfactant apoprotein
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cell types of origin for mucinous and nonmucinous BAC
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mucinous - bronchiolar goblet cells; nonmucinous - clara cells and type II pneumocytes
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By EM, how does one identify a type II pneumocyte?
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Cytoplasmic lamellar inclusion bodies/surfactant apolipoprotein
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how would one detect clara cells by ihc
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alpha 1 antitrypsin
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conventional adenoca of lung (CK7 vs. CK20) and an important exception
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CK7+CK20- (not mucinous type of BAC is usually CK20+)
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Pulmonary blastoma - which population is most often identified within
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adults
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another name for pulmonary endodermal tumor
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adenoca, fetal type
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6 ihc markers positive in small cell ca
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neurofilaments, leu7, chromogranin, synaptophysin, NSE, histidine decarboxylase
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four syndromes that small cell ca is associated with
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Eaton Lambert, SIADH, Cushing, carcinoid
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three common molecular alterations in lung ca
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del 3 (p14-p23); p53 mutations, inactivation of Rb
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lung carcinoma with best prognosis
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scca
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classic radiographic appearance of this lesion contains popcorn calcifications and is located peripherally
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hamartoma
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common molecular alteration in hamartomas
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czome 6p21 withint the HMG-I (Y) gene
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What is in Carney's triad
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hamartomas (aka pulmonary chondromas); GIST, functioning extra-adrenal paragangliomas
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alternative name for the minute meningothelioid nodule
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chemodectomass
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chromosomal changes in inflammatory myofibroblastic tumor
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2p23 with changes in alk-1 expression
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what other tumors are associated with lymphangiomyomatosis of the lung
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tuberous sclerosis, renal angiomyolipomas
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ihc in association with lymphangiomyomatosis
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SMA, HMB45 (like other PEComa like tumors), PR
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three clinical scenarios in which one might see lymphomatoid granulomatosis
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sjogren's, transplant recipients, HIV
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most common extrapulmonary sites of lymphomatoid granulomatosis
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skin, CNS, kidneys
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what is the theorized pathogenesis of lymphomatoid granulomatosis
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EBV infected B cells with associated reactive T cells and vasculitis
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what is pleuorpulmonary blastoma
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embryonal malignant pediatric neoplasm
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histo features of a pleuropulmonary blastoma
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primitive blastomatous and sarcomatous elements
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histologic features of alveolar proteinosis
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intra-alveolar eosinophilic material with cholesterol clefts
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Typical ihc profile for mesothelioma (3 positive stains; four negative stains)
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WT1, calretinin, ck5/6; negatives are: B72.3, BerEP4, CD15 (leuM1), CEA
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pleural plaques usually involve which pleura
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parietal
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histologic description of a plexiform lesion of the lung and the etiology
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abnormal vessel with neolumen formation and complex intimal proliferation - caused by PPH
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Mutation linked to PPH
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BMPRII gene
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organs/tissue that is commonly affected by sarcoidosis (6)
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Lung, mediastinal lymph nodes, cardiac, CNS, skin and eye
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two exposures that cuse non-necrotizing granulomas that can mimic sarcoidosis
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Berylliosis and aluminum
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histologic features of sclerosing hemangioma of lung
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bronchial epithelial cells with central proliferation of mesenchymal cells in a papillary projection (can see hemosiderin/blood)
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cell of origin for sclerosing hemangioma of lung
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two cell types (but clonal)- ck positive epithelioid cells along outside with EMA+ but Ck negative on mesenchymal appearing cells; probably from type II pneumocytes- both TTF1 positive
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size of histoplasma, shape, budding appearance (narrow vs. broad), surrounding histologic appearance of lung
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2-4 um; oval, narrow based budding; often granulomatous
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size of cryptococcus, shape, budding appearance (narrow vs. broad), surrounding histologic appearance of lung
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4-6 um, oval, narrow budding, granulomatous/histiocytic/minimal reaction
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good stains for cryptococcus (2)
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mucicarmine and fontana positive
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size of coccidioides, surrounding histologic appearance of lung
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spherules (30-100um) with endospores (2-5 um); granulomatous or suppurative
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good stain for coccidioides (1)
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gms+
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size of blastomyces, shape, budding appearance (narrow vs. broad), surrounding histologic appearance of lung
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8-15 um, spheres, broad based budding, granulomatos or suppurative
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aspergillus shape, branching pattern/angle, patterns in lung (5)
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septate hyphae with acute angle branching; patterns in lung 1. aspergilloma, 2. minimal/chronic; 3. invasive; 4. necrotizing tracheobronchitis; 5. allergic
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zygomycetes (rhizopus, mucor) shape, branching pattern/angle, 1 pattern
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broad (5-25 um), thin walled, branching variable; empty appearance, invasive
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candida in lung: shape, branching pattern/angle, patterns in lung (2)
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2-6 um yeasts and pseudohyphae; two lung patterns: disseminated pneumonia vs. suppurative/necrotic nodules
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histologic features of hard metal pneumoconiosis
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bronchiolocentric distribution, lymphoid hyperplasia
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cause of of hard metal pneumoconiosis
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cobalt
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differential diagnosis of cystic lungs
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Langerhans' cell histiocytosis (aka eosinophilic granuloma), emphysema, end-stage lung fibrosis and LAM
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Other lesions associated with LAM (4)
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tuberous sclerosis (Lam is found in 25% of TS patients), micronodular pneumocyte hyperplasia, clear cell tumor and angiomyolipoma
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Other lesions associated with tuberous sclerosis (4)
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LAM, micronodular pneumocyte hyperplasia, cler cell tumor and angiomyolipoma
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Molecular alterations found in LAM
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loss of heterozygosity in TSC2 and less frequently TSC1
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histological pattern of lung with adenoviral infection
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DAD, bronchiolitis
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histological pattern of lung with cytomegaloviral infection
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Necrosis, DAD/diffuse
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histological pattern of lung with HSV infection
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bronchitis or pneumonia
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histological pattern of lung with varicella infection
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pneumonia/necrosis, giant cell pneumonia, fibrotic nodules
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histological pattern of lung with measles infection
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giant cell pneumonia
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histological pattern of lung with parainfluenza infection
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pneumonia, giant cell pneumonia, bronchiolitis, similar to RSV
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histological pattern of lung with RSV infection
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pediatric URI, pneumonia, bronchiolitis, giant cell pneumonia
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are carcinoid tumors more commonly peripheral or endobronchial
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endobronchial
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clinical associations/causes of chronic eosinophilic pneumonia
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idiopathic, infections, meds, Churg-Strauss
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alternative name for Churg-Strauss
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allergic granulomatous angiitis
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Name three vascular syndromes that affect small and medium sized vessels and affect cANCA levels
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Churg-Strauss, Wegener's, microscopic polyangiitis
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Symptoms of Churg Strauss
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asthma, granulomatous inflammation, necrotizing glomerulonephritis, systemic vasculitis
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what is a minute meninothelial-like nodule
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aka chemodectoma, small 1-3 mm, can be multiple; spindled cells that may be whorled and thicken the alveolar walls
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name two ihc and two important negative ihc for minute meningothelial-like nodules
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positive for EMA and vimentin; negative for CD34, negative for neuroendocrine markers
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what are birbeck granules associated with and what do they look like on EM
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Langerhans' cell histiocytosis; tennis rackets
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alternative name for BOOP
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cryptogenic organizing pneumonia
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is boop diffuse or patchy
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patchy
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is boop steroid responsive
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yes
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3 histologic features of extrinsic allergic alveolitis
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lymphocytic bronchiolitis, mild intersitial lymphocytic inflammation and scattered, poorly formed granulomas
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name 7 diffuse pulmonary hemorrhage syndromes
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(aka associated with capillaritis): Wegener's, microscopic polyangiitis, lupus, mixed cryoglobulinemia, H-S purpura, antiphospholipid antibody syndrome, goodpasture's syndrome
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what does staphylococcal pneumonia look like
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microabscesses
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what does PCP look like
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intra-alveolar foamy eosinophilic exudates with GMS-positive cup shaped organisms; or granulomatous
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what does pseudomonal pneumonia look like
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can be necrotizing and hemorrhagic
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what does pseudomonas look like on a gram stain
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gram-negative rod
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name three categories of disease or diseases in which one could see LIP
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Sjogren's and other autoimmune disease, AIDS and meds
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Name five disease categories or specific examples in which constrictive/obliterative bronchiolitis coulc be found
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infections (ex. adenovirus); connective tissue diseases (ex. RA), meds (ex. penicillamine), lung transplant patients; fume exposures
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five infections that can lead to giant cell pneumonia
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RSV, parainfluenza, varicella, herpes simplex, measles
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pulmonary disorders that can spread through lymphatics
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sarcoid, lymphoma, kaposi's sarcoma, lymphangitic carcinoma and histiocytic disease (like Langerhans')
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mitotic counts for the category of atypical carcinoid in the lung
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2-10 per 10 hpf
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what is an alveolar adenoma
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a multicystic nodular proliferation with epithelial lining of the cysts adn mesnchymal cell proliferation in between cysts
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what is hermansky-pudlak syndrome
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autosomal recessive disease with albinism, coagulation defects, ceroid filled histiocytes and pulmonary fibrosis
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what is pulmonary branching ossifications
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branching intra-alveolar bone
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most common pulmonary complication of MTX use
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cellular interstitial pneumonia that resembles NSIP
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is chromogranin and synaptophysin staining in small cell carcinoma of the lung
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only weakly or absent
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name three pulmonary diseases that can result in pneumothorax
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Langerhans, LAM and emphysema
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what is demonstrated using a Dieterle stain
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legionella, short rod, treated with macrolides
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