path respiratory - formatted Flashcards

Question

25. 13.02.27 What disease is characterised by non caseating granuloma? Rob p470 1. TB 2. Chronic Crohn’s 3. EG 4. Sarcoid

Answer 1

A: Sarcoid **but also see Crohns From robbins - TB: Caseating granulomas (Tuberculoma) - **Chronic Crohns: not characterized by however non-caseating granulomas is a hallmark of Crohn disease, found in 35% of cases. - Eosinophilic Granulomatosis with Polyangitis: Necrotising eosinophilic granulomas

Answer 2

2. Lung involvement characterized by necrotizing haemorrhagic pneumonitis true • A condition characterized by the simultaneous appearance of 1. Proliferative gomerulonephritis 2. Necrotizing haemorrhagic interstitial pneumonitis • Characteristic findings of focal proliferative glomerulonephritis in early cases, or rapid progressive/crescentic GN (RPGN) in rapidly progressive cases SCS: robbins. Additional facts - if only affecting Kidneys= antiglomerular basement membrane disease - the term goodpasture designates the 40-60% of pepz who develop pulmonary haemorrhage in addition to renal disease - male preponderance - majority are dirty smokers - most cases begin with lung symptoms ( pricipally haemoptysis and consolidation on CXR). Then manifestations of GN appear. - cause of death= uraemia - treat w plasmapheresis + immune suppression.

Answer 3

3. With haemorrhage underlying parenchyma is preserved • Smaller emboli can travel out into the more peripheral vessels, where they may or may not cause infarction. • In patients with adequate cardiovascular function, the • Only about 10% of emboli actually cause infarction. • Although the underlying pulmonary architecture may be obscured by the suffusion of blood, hemorrhages are distinguished by the preservation of the pulmonary alveolar architecture; in such cases, resorption of the blood permits reconstitution of the preexisting architecture • Pulmonary embolism usually causes infarction only when the circulation is already inadequate, as in patients with heart or lung disease. • For this reason, pulmonary infarcts tend to be uncommon in the young. • About ¾'s of all infarcts affect the lower lobes, and in more than ½ multiple lesions occur *SCS - taken from radiopaedia: Following an occlusive pulmonary embolus, the bronchial arteries are recruited as the primary source of perfusion for the pulmonary capillary network. The higher pressure of the bronchial arteries, in combination with locally increased vascular permeability and capillary endothelial injury, is thought to result in local pulmonary capillary hemorrhage.

Answer 4

A: Squamous and adeno 20-40%, small 20%, large 10%. From robbins - Adenocarcinoma 38% - SCC 20% - Small cell carcinoma 14% - Large cell carcinoma 3% - other 25%

Answer 5

A: Neuroendocrine granules and most likely to have paraneoplastic syndrome Other answers - "also known as oat cell": only found on radiopaedia. Not on statdx or robbins. - Treatment is radiation and chemotherapy only. - May arise in major bronchi or in the periphery of the lung. - It has the strongest association with smoking (only 1% of cases occur in non-smokers. 3. Treatment is with lobectomy and chemotherapy (Small cell is sensitive to radiotherapy & chemotherapy, with potential cure rates of 15 – 25 % for localized disease) 4. Ususally peripheral but has hilar nodes (Central, almost never cavitate) 5. Not associated with smoking ( Sqaumous and Small Cell show strongest association with Smoking; only 1% in non-smokers) * Carefull with the wording in the exam on this one, see below!! * This highly malignant tumor has a distinctive cell type. The epithelial cells are generally small, have little cytoplasm, and are round or oval and, occasionally, lymphocyte like (although they are about twice the size of a lymphocyte LW - smaller than neighbouring resting Lymphocytes). * This is the classic oat cell, which is a specific subtype. (not all Small cell are oat cell!!) true • Other small cell carcinomas have o spindle-shaped o polygonal cells o and may be thus classified (spindle or polygonal small cell carcinoma). The cells grow in clusters that exhibit neither glandular nor squamous organization. • Electron microscopic studies show dense-core neurosecretory granules in some of these tumor cells. The granules are similar to those found in the neuroendocrine argentaffin (Kulchitsky) cells present along the bronchial epithelium, particularly in the fetus and neonate. • 3-10% of lung cancers develop a paraneoplastic syndrome

Answer 6

1. Primary adenocarcinoma • Solitary Metastatic Lung Nodule o A solitary lung nodule represents a primary lung tumor in 62% in patients with known Hx of neoplasm o 5% of all solitary nodules are metastatic; most likely origin: colon carcinoma (30-40%), osteosarcoma, renal cell carcinoma, testicular tumor, breast carcinoma *LW: useful pathological marker is TTF-1: thyroid transcription facot, which is positive in primary lung adenocarcinoma and negtive in pulmonary metastatic disease from extra thoracic adenocarcinoma.

Answer 7

Answer unclear As Radiopaedia - Staging IIA: T2aN1M0 - 5 year survival 60%. However, as per robbins - In general, the 5-year survival rate is 52% for cases detected when the disease is still localized, 22% when there is regional metastases, and only 4% with distant metastases. - Untreated, the survival time for patients with small-cell carcinoma is 6-17 weeks. Even with treatment, the mean survival after diagnosis is only about 1 year.

Answer 8

A: Associated with alcohol, smoking and irradiation Other statments corrected: - Polyps and nodules VIRTUALLY NEVER GIVE RISE to cancer - Laryngeal cancer usually arises from the TRUE CORDS - Polyps and nodules usually involve the TRUE CORDS

Answer 9

1. asbestos bodies macrophage ingest asbestos fibre-> collage prolfieration-> stained with haemosiderin and iron **SCS: Radiopaedia. An asbestos body is a histological finding in interstitial LUNG disease following a parenchymal (NOT pleural ) biopsy. Macrophage ingestion of the asbestos fibres triggers a fibrogenic response via the release of growth factors that promote collagen deposition by fibroblasts. The fibres become encased in iron-rich material derived from proteins such as ferritin and haemosiderin.

Answer 10

A: associated with pulmonary effusion (NO PLEURAL EFFUSION) From robbins - may be small (1-2 cm) or may reach an enormous size - often attached to the pleural surface by a pedicle. - grossly consists of dense fibrous tissue with occasional cysts filled with viscid fluid. - no relationship to asbestos exposure.

Answer 11

6. GIST alpha-fetoprotein Liver cell cancer, nonseminomatous germ cell tumors of testis Less regularly in CA of colon, lung and pancreas Non-neoplastic : cirrhosis, toxic liver injury, hepatitis and pregnancy (especially when there is foetal distress or death), smoking

Answer 12

1. Hepatic cirrhosis

Answer 13

risk factors- smoking- alcohol- coffee- asbest

Answer 14

A: carcinoma above the true cord **note only the most correct from those statements (see below). From Robbins - Singer nodules located usually on the true vocal cords. - Carcinoma of the larynx usually develops on the vocal cords (true cords), but may also arise above or below the cords, on the epiglottis or aryepiglottic folds or in the pyriform sinuses. Additional facts from robbins: - 95% of laryngeal carcinomas are typical squamous cell tumors. - Carcinoma of the larynx is most commonly seen in men, 6th decard of life. - risk factors: smoking, alcohol, absestos, irradiation, HPV infection.

Answer 15

4. Parenchymal biopsy

Answer 16

1. lung cancer | carcinoma and tuberculosis are potential serious complications of silicosis.

Answer 17

2. often adherent to vessels resulting in incomplete excision and recurrence of 10% (frequently recur after incomplete resection)

Answer 18

4. 10% atypical (of which will get carcinoid recurrence in 50%) * Bronchial carcinoids represent 1 to 5% of all lung tumors * Most patients with carcinoid tumors are younger than 40 years of age * incidence is equal for both sexes * The reported 5- to 10-year survival rates for typical carcinoids are 50 to 95%. * A minority (10%) of tumors show cytologic atypia, necrosis, and aggressive behavior (50% recurrence or metastasis after 2 years) and are designated atypical carcinoids. (note: Adelaide notes say 25%!!) * Histologically the tumor is composed of nests, cords, and masses of cells separated by a delicate fibrous stroma. In common with the lesions of the gastrointestinal tract, the individual cells are quite regular and have uniform round nuclei and infrequent mitoses - note central vs peripheral- typical and atypical

Answer 19

* after fractures of long bones, or rarely in the setting of soft tissue trauma and burns. * occurs in 90% of individuals with severe skeletal injuries, but less than 10% of such patients have any clinical findings. * syndrome typically begins 1 to 3 days after injury, with sudden onset of tachypnea, dyspnea, and tachycardia. * Besides pulmonary insufficiency, the syndrome is characterized by neurologic symptoms, including irritability and restlessness, which can progress to delirium or coma. * diffuse petechial rash in nondependent areas (occurring in the absence of thrombocytopenia) is seen in 20 to 50% of cases and is useful in establishing a diagnosis. * Patients may also present with thrombocytopenia, presumably caused by platelets adhering to the myriad fat globules and being removed from the circulation * anemia may result as a consequence of erythrocyte aggregation and hemolysis * fatal in up to 10% of cases. * The pathogenesis of fat emboli syndrome probably involves both mechanical obstruction and biochemical injury (free fatty acids) * platelet activation and recruitment of granulocytes * Because lipids are dissolved out of tissue preparations by the solvents routinely used in paraffin embedding, the microscopic demonstration of fat microglobules (i.e., in the absence of accompanying marrow) typically requires specialized techniques, including frozen sections and fat stains.

Answer 20

• Abnormal permanent enlargement of the airspaces distal to the terminal bronchiole with destruction of their walls, and without obvious fibrosis

Answer 21

5. Bird fanciers lung * The term pneumoconiosis was originally coined to describe the non-neoplastic lung reaction to inhalation of mineral dusts (e.g., coal dust anthracosis, silica silicosis, asbestos asbestosis, and beryllium berylliosis) commonly encountered in the workplace * Some have now broadened the term to include the organic dusts though

Answer 22

2. Von Meyenberg complexes Von Meyenberg complexes - multiple biliary harmatomas Alpha 1 antitrypsin deficiency: • alpha1 -Antitrypsin deficiency is an autosomal recessive disorder marked by abnormally low serum levels of this protease inhibitor (Pi) • However, homozygotes for the PiZZ protein have circulating alpha1 -antitrypsin levels that are only 10% of normal levels • Neonatal hepatitis with cholestatic jaundice appears in 10% to 20% of newborns with the deficiency. In adolescence, presenting symptoms may be related to hepatitis or cirrhosis. Attacks of hepatitis may subside with apparent complete recovery, or they may become chronic and lead progressively to cirrhosis. • Hepatocellular carcinoma develops in 2% to 3% of PiZZ adults, usually but not always in the setting of cirrhosis

Answer 23

1. Asbestos bodies • Asbestos bodies appear as golden brown, fusiform or beaded rods with a translucent center and consist of asbestos fibers coated with an iron-containing proteinaceous material • There are two distinct geometric forms of asbestos: serpentine (curly and flexible fibers) and amphibole (straight, stiff, and brittle fibers). • The serpentine chrysotile chemical form accounts for most of the asbestos used in industry. • Amphiboles include crocidolite, amosite, tremolite, anthophyllite, and actinolyte. • amphiboles, even though less prevalent, are more pathogenic particularly with respect to induction of malignant pleural tumors (mesotheliomas). • Some studies of mesotheliomas have shown the link is almost always to amphibole exposure. • The relatively few cases of mesotheliomas arising in chrysotile workers are in all likelihood due to contamination of chrysotile with the amphibole tremolite. • The greater pathogenicity of straight and stiff amphiboles is apparently related to their aerodynamic properties and solubility. • Chrysotiles, with their more flexible, curled structure, are likely to become impacted in the upper respiratory passages and removed by the mucociliary elevator. Furthermore, once trapped in the lungs, chrysotiles are gradually leached from the tissues because they are more soluble than amphiboles. • In contrast, the straight, stiff amphiboles may align themselves in the airstream and thus be delivered deeper into the lungs, where they can penetrate epithelial cells and reach the interstitium. • The length of amphibole fibers also plays a role in pathogenicity, those longer than 8 mm and thinner than 0.5 mm being more injurious than shorter, thicker ones. • Nevertheless, both amphiboles and serpentines are fibrogenic, and increasing doses are associated with a higher incidence of all asbestos-related disease except that only amphibole exposure correlates with mesothelioma. *WJI - caution, need to see what other options are as this could easily be a trick around asbestos exposure (asbestos bodies, fibrosis could both be seen) and the syndrome of asbestosis which requires symptoms.

Answer 24

non correct • None of these are correct, correct option absent • Adenocarcinoma 40% o Most common form in M & F, and in nonsmokers • Squamous cell 30% • Small cell 20% • Large cell 10-15% • Scar cancers are usually adenocarcinomas

Answer 25

4. bronchioloalveolar cell carcinoma • Neoplasms of neuroendocrine cells in the lung include benign tumorlets, small, inconsequential hyperplastic neuroendocrine cells seen in areas of scarring or chronic inflammation; carcinoids; and the (already discussed) highly aggressive small cell carcinoma of the lung.

Answer 26

• Microscopically, they have a central zone of fibrinoid necrosis surrounded by a prominent rim of epithelioid histiocytes and numerous lymphocytes and plasma cells

Answer 27

1. Caseous necrosis • Acid fastness is not specific for Mycobacterium tuberculosis, as all species of mycobacteria possess this characteristic (i.e. acid fastness should not be equated with tuberculosis).

Answer 28

63. PATH2004 Lung mets commonest cause 3. Breast wji: breast>renal>head and neck>CRC most likely to have lung mets: renal=osteosarc=choriocarcinoma>thyroid>melanoma>breast

Answer 29

2. Delayed hypersensitivity reaction SCS: this principle underlies the mantoux test. T cell mediated killing

Answer 30

. Ghon complex F – ghon complex is the initial focus of primary infection consisting of a parenchymal subpleural lesion close to interlobar fissure and hilar lymph nodes

Answer 31

• curled & flexible (serpentine) = chrysotile (90%) – most common type used in industry • brittle, straight amphiboles = crocidolite / amosite (10%) – less prevalent, but more pathogenic * Amphiboles fracture → smaller (longer than 8 mm and thinner than 0.5 mm) fragments which reach the airways, and penetrate epithelium to reach interstitium * Chrysotiles → deposit in the upper tracheobronchial tree & are cleared, because more flexible & more soluble * N.B. all types are fibrogenic but crocidolite is the most carcinogenic (particularly w.r.t. mesothelioma) • Asbestos bodies = fibres coated with iron-containing proteinaceous material, which arise when macrophages attempt to phagocytose fibres – iron derived from phagocyte ferritin o Golden brown, fusiform or beaded rods with translucent centre • Ferruginous bodies = similar iron protein coated inorganic particles, with no asbestos core • Focal pleural plaques and mesothelioma do not have asbestos bodies

Answer 32

5. haemangioblastoma

Answer 33

1. dilation of airspace distal to terminal bronchioles with destruction of walls • Abnormal permanent enlargement of the airspaces distal to the terminal bronchiole with destruction of their walls, and without obvious fibrosis

Answer 34

2. 75% are in the lower lobes * SCS Robbins - ischemic necrosis occurs on only 10% of PE - dual supply. Infarction occurs in those with compromised CVS status (CHF)or in areas of pulmonary disease. - The more peripheral, more risk of infarct - pulmonary haemorrhage doesn’t equal infarct *SCS - taken from radiopaedia: Following an occlusive pulmonary embolus, the bronchial arteries are recruited as the primary source of perfusion for the pulmonary capillary network. The higher pressure of the bronchial arteries, in combination with locally increased vascular permeability and capillary endothelial injury, is thought to result in local pulmonary capillary hemorrhage

Answer 35

1. Exposure to chrysotile fibres ``` serpentine = chrosytile - shallow infiltration - less phathogenic amphibole = crocidolite - deep respiratory infiltration - more pathogenic ``` **LJS - agree that serpentine is lower risk. Though could also be testing the fact that only 20% mesothelioma pt have concurrent pulmonary asbestosis

Answer 36

1. Inflammation- histiocytes /lymphocytes confined to alveolar walls T Psittacosis is an infection caused by the obligatory intracellular bacterium Chlamydia psittaci; assocd with birds & poultry. Predominant manifestation is respiratory tract infection with constitiutional symptoms. May also have CNS, skin, spleen, renal, haematologic, joint involvement... The most common imaging finding is unilateral, LL dense infiltrate/consolidation. May present in a bilateral, nodular, miliary, or interstitial pattern. Histo findings may include tracheobronchitis and interstitial pneumonitis with air-space involvement and predominant mononuclear cell infiltration. Findings may also include macrophages that contain cytoplasmic inclusion bodies (ie, Levinthal-Coles-Lillie [LCL] bodies), focal necrosis of hepatocytes along with Kupffer cell hyperplasia in the liver, and hepatic noncaseating granulomata. [eMedicine]

Answer 37

4. Cryptococcus T - preferentially infects immunosuppressed individuals, occurs in up to 155 of AIDS patients. Pulmonary infection is usually associated with brain / meningeal disease, but this scenario implies that the patient is immunocompetent. CXR: patchy infiltrates/pneumonitis (affecting any part of lung; bilateral/unilateral, mulitlobar/lobar). May manifest as granulomas 2-7mm, or miliary disease (similar to TB). [Cryptococcosis, eMedicine] *EDWH - I think the answer is cryptococcus - radiopaedia says “Cryptococcosis predominantly occurs in immunocompromised patients but can also be seen in immunocompetent hosts” **LJS - all these occur in immunosuppressed. ?Kaposi in least suppressed? Typically seen at CD4 < 200 but nowadays seen with higher CD4 (since AART) RP says cryptococcus causes asymptomatic disease in lung only in immunocompetent, so maybe this is true? 2. Patient with HIV with normal CD4 count, patchy pulmonary opacities on CXR, which is most likely: (GC) 1. CMV F - occurs in those with CD4 counts <100. CXR: bilateral hazy infiltrates, focal nodules, masses, bronchiectasais. 2. Toxoplasmosis F - cerebral toxoplasmosis occurs in those with CD4 counts <150. 3. Kaposi’s F - usually develops in the setting of a low CD4 count (<150-200 according to RG, Dahnert gives <400). CXR: mid-lower zone pulmonary opacities and parenchymal nodules (peribronchovascular distribution). 4. Cryptococcus T - preferentially infects immunosuppressed individuals, occurs in up to 155 of AIDS patients. Pulmonary infection is usually associated with brain / meningeal disease, but this scenario implies that the patient is immunocompetent. CXR: patchy infiltrates/pneumonitis (affecting any part of lung; bilateral/unilateral, mulitlobar/lobar). May manifest as granulomas 2-7mm, or miliary disease (similar to TB). [Cryptococcosis, eMedicine] 5. PCP F - occurs in those with CD4 counts <200. CXR: bilateral perihilar interstitial infiltrates, diffuse alveolar infiltrates, often associated with pneumatocoeles, apical predominance in pts on prophylactic aerosolised pentamidine). [Dahnert] Despite the emphasis on opportunisitic infections, remember that 'typical' bacterial lower respiratory tract infections are one of the most serious disorders in IV infection (S.pneumoniae, S.aureus, H.influenzae, GNB). [Robbins]

Answer 38

Answer: Empyema (atypical finding) ROBBINS C. albicans most common type. Most commonly, candidiasis takes on superficial infection (thrust). Other forms include: esophagitis, vaginitis, cutaneous Invasive forms - renal abscess - myocardial abscess and endocarditis - brain micro abscesses and meningitis - endophthalmitis - hepatic and splenic abscess Candida endocarditis is the most common fungal endocarditis, usually occurring in the setting of prosthetic heart valves or in IVDU. In the latter group, the tricuspid valve is involved. RADIOPAEDIA Osteomyelitis - all types of organisms including viruses, parasites, fungi, and bacteria can produce osteomyelitis. - common fungi: candida, aspergillum - C. albicans: vertebral osteomyelitis most common location in adults, femoral/huemral bones are most common in kids. ADDITIONAL PAPERS Only a few case reports of candida empyema (eg. Indian J Pathol Microbiol 2008). A Taiwanese study (Chest 2000) looked at 67 patients with fungal empyema (of which 18 were immunocompromised); found that Candida was the most commonly encountered species (64%).

Answer 39

Answer: increased risk of GIT, pancreas, biliary and lymphoma ROBBINS Cystic fibrosis is an inherited disorder of ion transport that affects fluid secretion in exocrine glands and in the epithelial lining of the respiratory, gastrointestinal, and reproductive tracts. - most common lethal genetic disease that affects caucasian populations. - Autosomal recessive inheritance BUT heterozygote carriers have a higher incidence of respiratory and pancreatic diseases. FROM RADIOPAEDIA Both translated and non-transplanted patients are at increased risk of some malignancies - GIT: in particular oesophageal, gastric, small bowel, and colorectal - Gallbladder and extra hepatic biliary tree - Lymphoma/leukaemia - testicular.

Answer 40

3. Patient A has type A and patient B has type B T ``` Stanford type A (60%) - involves at least ascending aorta (DeBakey types I and II), surgical management. Stanford B (40%) - limited to descending aorta (De Bakey III), medical treatment. ``` De Bakey pnemonic - BAD (both, ascending, descending - I, II, III)

Answer 41

2. Southeast Asian T - almost unknown among Native Indians, Aussie aboriginals, or southern Asians (eg Koreans). [eMedicine] 6. Sarcoid, which is most atypical: (GC) 1. Afro-American F - 10 times more prevalent in US Afro-Americans than in US cauasians. [Robbins] 2. Southeast Asian T - almost unknown among Native Indians, Aussie aboriginals, or southern Asians (eg Koreans). [eMedicine] 3. Ashkenazi Jew - Jews have incidence rates of around 2 in 100,000. [Respirology 2006] 4. Living in tropics - incidence in Africa, SE Asia, India, central & south America is <10 per 100,000. [eMedicine] 5. Male - females are more often affected than males, but only by 3:1. [Dahnert]

Answer 42

*LW quoting Robbins: Organising stage, type 2 pnuemocytes undergo proliferation to try and regenerate the alveolar lining damaged. Resolution is unusual, more commonly there is organisation of the fibrin exudate with resultant intra alveolar septa fibrosis, and marked thickening of alveolar septa, due to proliferation of interstitial cells and collagen deposition. Fatal cases often have superimposed broncho-pneumonia. Initial injury is to capillary endothelium most commonly, progressing to both endothelium and alveolar epithelium. Acute consequences of damage to alveolar capillary membrane include increased vascular permeability, and alveolar flooding, loss of difusion capacity, surfactant abnormalities due to type 2 cell injury, Exudate and diffuse tissue destruction cannot be easily resolved, resulting in organisation with scarring --> chronic disease. Thus: 1. Usually associated with bacterial superinfection: not always (tend to be fatal cases), so less correct 2. Always goes on to fibrosis: commonly goes onto fibrosis, so partially correct, but term 'always' makes me dubious. 3. Abnormality is due to endothelaial damage and increased capillary permeability: TRUE. 4. Takes a long time for Type II pneumocytes to recover: semi true. Previous notes: controversy: some answer had 2 and some had 3 3. Abnormality is due to endothelial damage, as well as increased capillary permeability T - integrity of the alveolar-capillary membrane is compromised by either endothelial or epithelial injury, or usually both. * Exudative stage: - endothelial - increased vascular permeability (oedema, inflammation, haemorrhage), leads to fibrin exudation and formation of hyaline membranes. - epithelial - damage to type II pneumocytes, resulting in widespread reduction in surfactant production (with subsequent decreased lung compliance and alveolar collapse). * Proliferative (organising) stage: type II pneumocytes attempt to regenerate the alveolar lining, organisation of fibrin exudate. * Fibrotic stage: variable scarring and cyst formation. 7. ARDS takes a long time to resolve because: (GC) 1. Usually associated with bacterial superinfection F - sepsis is the most common risk factor for ARDS. But as far as having ARDS, with secondary bacterial superinfection as the cause of delayed resolution... *** can't find much on this. *** 2. Always goes on to fibrosis F - the acute (exudative) phase usually resolves completely. Less commonly, residual pulmonary fibrosis occurs, in which the alveolar spaces are filled with mesenchymal cells and new blood vessels. This process seems to be facilitated by IL-1. [eMedicine] 3. Abnormality is due to endothelial damage, as well as increased capillary permeability T - integrity of the alveolar-capillary membrane is compromised by either endothelial or epithelial injury, or usually both. * Exudative stage: - endothelial - increased vascular permeability (oedema, inflammation, haemorrhage), leads to fibrin exudation and formation of hyaline membranes. - epithelial - damage to type II pneumocytes, resulting in widespread reduction in surfactant production (with subsequent decreased lung compliance and alveolar collapse). * Proliferative (organising) stage: type II pneumocytes attempt to regenerate the alveolar lining, organisation of fibrin exudate. * Fibrotic stage: variable scarring and cyst formation. 4. Takes a long time for type II pneumocytes to recover F - type I pneumocytes are more vulnerable than type II. Hyperplasia of type II pneumocytes occurs around 5-7 days. [Dahnert, Robbins]

Answer 43

3. With haemorrhage underlying parenchyma is preserved 8. In pulmonary emboli, which is true: (GC) 1. Haemorrhage implies infarction F - infarction resulting from pulmonary haemorrhage is the exception rather than the rule, occurring as few as 10% of cases. It occurs only if there is compromise in cardiac function or bronchial circulation, or if the region of the lung at risk is underventilated as a result of underlying pulmonary disease. 2. Always infarct as vessels are end arteries F - see above. 3. With haemorrhage underlying parenchyma is preserved 4. Breast carcinoma is the most common cause of tumour embolism F - gastric Ca most common. Other solid malignancies that may form emboli in the IVC: breast, prostate, lung, HCC, ovarian, osteosarcoma, lymphoma, choriocarcinoma.

Answer 44

3. Any tumour of thymic epithelial cells = the term "thymoma" is restricted to tumours in which epithelial cells constitute the neoplastic element [Robbins]. Divided into "invasive" and "non invasive" (cf. benign and malignant, as in Robbins) because invasive thymoma may lack the histologic features of malignancy [RG].

Answer 45

1. Primary pulmonary hypertensionT - plexogenic pulmonary arteriopathy (see Robbins) is seen in PPH or congenital heart disease with left to right shunts - tuft of capillary formations that spans the lumens of dilated thin-walled arteries

Answer 46

1. 2-5 x risk of lung cancer independent of smoking F - once smoking-related risk is taken into account, there is no increased frequency of bronchogenic carcinoma (distinguishes from silicosis and asbestosis). * LW: Robbins states no increased incidence of TB, unlike silicosis, thus also false. 13. Coal workers pneumoconiosis, which is not true: (GC) 1. 2-5 x risk of lung cancer independent of smoking F - once smoking-related risk is taken into account, there is no increased frequency of bronchogenic carcinoma (distinguishes from silicosis and asbestosis). 2. <10% go on to Progressive massive fibrosis T - fewer than 10% of cases of simple CWP progress to PMF, a generic term that applies to a confluent fibrosing reaction in the lung (complication of any of the fibrogenic pneumoconioses - CWP, silicosis, asbestosis). Generally takes many years to develop. PMF in CWP is a fibrotic mass of haphazardly arranged collagen with numerous pigment-laden macrophages and with abundant free pigment, +/- necrosis, cholesterol clefts, chronic inflam infiltrate. Hx differs from silicosis of PMF: central hyalinised collagen surrounded by a rim of macrophages, focal necrosis common +/- granulomatous inflammation. 3. Characterised by coal macules and coal nodules T - macules are dust laden macropahges, 1-2mm. Nodules are slightly larger (still <5mm), and have additional delicate network of collagen fibres. Lesions are scattered throughout, but predominantly in upper lobes and upper zones of lower lobes. Radiographically indistinguishable from silicosis. 4. PMF tends to progress even in the absence of further exposure T 5. Increased incidence of TB T - as in silicosis. [Robbins; Pneumoconiosis RG 2006] Added options 4 and 5.

Answer 47

5. Golden brown nodes with iron containing proteinaceous material - no asbestos bodies

Answer 48

1. Tuberous sclerosis F - 1-4% have lung involvement - LAM. CRITTSCOPRAInfectionTBTransplantSewyer james/sarcoid

Answer 49

5. Involvement of liver lugano modified ann arbor stage 1 - one nodal group stage 2 - two nodal group stage 3 - on both side of diaphragm. Or one nodal group above hemidiaphragm and spleen involvement stage III(1): involvement of the spleen or splenic, hilar, coeliac, or portal nodes stage III(2): involvement of the para-aortic, iliac, inguinal, or mesenteric nodes stage 4 - extra nodal met-> cannot be irradiated in same radiation field a - no systemic symptoms b - systemic symptom involvement e - extra nodal contiguous extension that can be encompassed by radiation field

Answer 50

3. Lung involvement characterised by necrotizing haemorrhagic pneumonitis T - acute focal necrosis of alveoli, intra-alveolar haemorrhage, fibrous thickening of septa etc = necrotising haemorrhagic interstitial pneumonitis 20. In Goodpasture’s syndrome (JS) 1. Lung involvement precedes renal involvement T (??) - glomerulonephritis and pneumonitis occur simultaneously but it begins clinically with respiratory symptoms (haemoptysis) and focal pulmonary consolidation 2. Lung involvement occurs later than renal F 3. Lung involvement characterised by necrotizing haemorrhagic pneumonitis T - acute focal necrosis of alveoli, intra-alveolar haemorrhage, fibrous thickening of septa etc = necrotising haemorrhagic interstitial pneumonitis 4. Usually mild F - used to have a dismal prognosis but plasma exchange has improved outcomes Goodpasture syndrome, also referred as antiglomerular basement membrane (anti-GBM) antibody disease, is an autoimmune disease characterised by damage to the alveolar and renal glomerular basement membranes by a cytotoxic antibody (type 2 hypersensitivity). It is a type of pulmonary-renal syndrome.Goodpasture syndrome is defined by:- pulmonary haemorrhage- glomerulonephritis- circulating antiglomerular basement membrane antibodiesM> FPulmonary : non-specific opacityCT : crazy paving, consolidation, LN. Resolve in 2 weeksPrognosis : poor. may need plasmophoresis and immunosuppressant

Answer 51

4. Lymphocyte depleted - rare and somewhat controversial. Most cases prove to be large cell lymphoma. Older male patients with disseminated disease, HIV patients, developing countries. 21. What is most controversial subgroup in Hodgkin’s lymphoma classification: (GC) 1. Nodular sclerosis - most common, young adults M=F. Hx: lacunar variant of RS cell, collagen bands with formation of nodules, CD30/CD15+. Excellent prognosis. 2. Lymphocytic predominant - young adult males. Hx: L+H (popcorn) variant of RS cell, panB-cell markers +. Excellent prognosis; 3-5% transform into DLBCL. 3. Lymphocytic rich - rare. 4. Lymphocyte depleted - rare and somewhat controversial. Most cases prove to be large cell lymphoma. Older male patients with disseminated disease, HIV patients, developing countries. 5. Mixed cellularity - older men. Hx: lots of RS cells and heterogeneous infiltrate. Usually disseminated disease + B symptoms. Changed option 3 from "lymphocyte proliferative". [Robbins]

Answer 52

2. Complete recovery in 60-80% T - 70% recover with no or minimal residual disease 22. Sarcoid, which is true: (GC) 1. Relapsing/ remitting course F - majority recover, 10% develop progressive pulmonary fibrosis and cor pulmonale. 2. Complete recovery in 60-80% T - 70% recover with no or minimal residual disease 3. Residual pulmonary/ visual impairment in 30% F - 20% 4. Parenchymal involvement only has a better prognosis than nodes + lung changes F - stage 3 versus stage 2 disease (Silzbach classification on CXR: Stage 0 - normal, 1 - adenopathy, 2 - LN + lung, 3 - lung only, 4 - pulmonary fibrosis).

Answer 53

*AJL - Robbins says: Chronic bronchitis is defined clinically as persistent cough with sputum production for at least 3 months in at least 2 consecutive years in the absence of any other identifiable cause. I would therefore favour 1. to be the best answer in this question. 2. Reid index >0.5 T - Normally 0.4. Ratio of submucosal gland layer (b-c) to that of the bronchial wall (a-d). Increased in chronic bronchitis. But this is an "old" method (still quoted in Robbins); the other method is to measure the mucus gland area in a given area of mucosa (eMedicine). 1. Definition of chronic bronchitis: (GC) 1. Clinical + micro/ macro F - diagnosis is made on clinical grounds. Some people with symptoms of chronic bronchitis may have no demonstrable histologic abnormality in their airways. However, there is a strong tendency for the presence of mucus gland hyperplasia in bronchial walls. This accounts for the increased sputum production (bronchial glands produce ~98% of total airway mucus production). 2. Reid index >0.5 T - Normally 0.4. Ratio of submucosal gland layer (b-c) to that of the bronchial wall (a-d). Increased in chronic bronchitis. But this is an "old" method (still quoted in Robbins); the other method is to measure the mucus gland area in a given area of mucosa (eMedicine). 3. Histo defined F - clinical definition: perisistent cough productive of sputum for >= 3 months in >= 2 consecutive years. 4. Histo which was bronchiectasis F - Hx: 1. enlargement of mucus-secreting glands (hallmark) 2. increased goblet cells (mainly involves bronchioles) 3. bronchiolar obstruction (mucus plugging, inflam, fibroisis) 4. bronchiolitis. Bronchiolitis obliterans in severe cases. [Adelaide notes]

Answer 54

3. Hyponatraemia T - secondary to SIADH; main features of the syndrome consist of hyponatremia and hypotonicity (< 280mOsm/kg), absence of fluid volume depletion, inappropriate urinary osmolality (> 100 mOsm/kg), increased urinary sodium excretion (>40mmol/L) while on normal salt and water intake, and absence of thyroid, adrenal, pituitary or renal dysfunction. SIADH results from 3 factors: 1. inappropriate stimulation from pulmonary pathology (bacterial pneumonia, TB, lung abscess) or drugs (eg. cytotoxics); 2. uncontrolled secretions from virtually any CNS disorder (including trauma, surgery); 3. ectopic ADH elaboration by tumours, particularly small cell (oat cell) lung carcinoma, duodenum and pancreatic cancers, olfactory neuroblastoma and lymphomas. [Hyponatremia and SIADH, CAMJ 2002]. 3. Man does not want surgery, hilar mass, oncologist wants to know if small cell, does CT – most likely bit of hx that would help: (GC) 1. CXR 1 year ago, hilar mass, lost to follow up F - 5YS with therapy is 25% for limited stage disease (disease confined to one hemithorax, including mediastinal/supraclavicular nodes); extensive stage (beyond definition of limited stage, or malig. pleural effusion) is rapidly fatal. Most have spread at presentation. 2. Normal CXR 9 months ago F - non discriminatory. 3. Hyponatraemia T - secondary to SIADH; main features of the syndrome consist of hyponatremia and hypotonicity (< 280mOsm/kg), absence of fluid volume depletion, inappropriate urinary osmolality (> 100 mOsm/kg), increased urinary sodium excretion (>40mmol/L) while on normal salt and water intake, and absence of thyroid, adrenal, pituitary or renal dysfunction. SIADH results from 3 factors: 1. inappropriate stimulation from pulmonary pathology (bacterial pneumonia, TB, lung abscess) or drugs (eg. cytotoxics); 2. uncontrolled secretions from virtually any CNS disorder (including trauma, surgery); 3. ectopic ADH elaboration by tumours, particularly small cell (oat cell) lung carcinoma, duodenum and pancreatic cancers, olfactory neuroblastoma and lymphomas. [Hyponatremia and SIADH, CAMJ 2002]. 4. Peripheral location F - usually perihilar surrounding large bronchi, but peripheral location is non discriminatory. 5. Mediastinal widening F - non discriminatory.

Answer 55

Answer: EM (Epithelioid mesothelioma) difficult o differentiate from adenocarcinoma FROM ROBBINS - Can arise from either visceral or parietal pleura - The lifetime risk of developing mesothelioma in heavily exposed individuals is as high as 7% to 10%. - long latency period of 25-45 years - no increased risk of mesothelioma is asbestos workers who smoke. - microscopically, malignant mesotheliomas may be epithelioid (60%), sarcomatous (20%), or mixed (20%). - Epithelioid type of mesothelioma consists of cuboidal, columnar, or flattened cells forming tubular or papillary structures resembling adenocarcinoma. - Immunohistochemical stains are very helpful in differentiating it from pulmonary adenocarcinoma.

Answer 56

Answer: Pulmonary alveolar proteinosis. **see below All answers are ASSOCIATED with silicosis However in the given clinical context of a RETIRED miner, pulmonary alveolar proteinosis is least likely as it is a RARE and ACUTE manifestation of silicosis occurring in week or months after intense exposure to fine dust high in silica. FROM ROBBINS - quartz type is most commonly implicated and very fibrogenic. - CXR typically show fine modularity in the upper zones - Silicosis is associated with an increased susceptibility to tuberculosis. - onset of silicosis is most commonly slow (10-30 years after exposure). - the disease may continue to worsen even if the patient is no longer exposed. - Patients with silicosis have double the risk for developing lung cancer. - Acute silicosis (rare) can cause secondary pulmonary alveolar proteinosis i.e. alveolar silicoproteinosis

Answer 57

4. Bronchial ulceration means bacterial superimposed infection F - causes include HSV, radiation, drugs, TB, malignancy, Wegener's, post transplant lymphoproliferative disease. 7. Community acquired pneumonia – least correct: (GC) 1. Viral T - community acquired atypical pneumonias include viral infections (RSV, parainfluenza (kids); influenza A & B (adults); adenovirus (military reccruits). 2. Mycoplasma + Chlamydia T 3. Px with symptoms of dyspnoea, haemoptysis, effusion T - onset is usually abrupt, with high fever, shaking chills, pleuritic chest pain, productive mucopurulent cough; occasionally haemoptysis. Parapneumonic effusion. 4. Bronchial ulceration means bacterial superimposed infection F - causes include HSV, radiation, drugs, TB, malignancy, Wegener's, post transplant lymphoproliferative disease.

Answer 58

Answer: Difficult to make diagnosis on FNA (True) FROM ROBBINS/STATDX/RADIOPAEDIA Neoplasms of neuroendocrine cells in the lung include (all separate entities). - benign tumorlets - carcinoids - small cell lung carcinoma - large cell neuroendocrine carcinoma of the lung. Carcinoid tumor represent 1-5% of all lung tumors affects <40 years of age, m=f. 20-40% of non-smokers. Carcinoid tumors of the lungs can be divided into 2 groups dependent of location, and 2 groups dependent on histology - Location: Bronchial (75%) vs peripheral (40%) - histology: typical (90%) vs atypical (10%) Most are confined to mainstream bronchi. Others penetrate the bronchial wall to fan out in the peribronchial tissue, producing "collar-button lesions".

Answer 59

1. Organ involved can be CNS or kidney in isolation This is true -SG11. TB, which is most correct: (GC) 1. Organ involved can be CNS or kidney in isolation This is true -SG 2. Type 1 hypersensitivity reaction F - type IV immune reaction. 3. Ghon focus is parenchymal + LN F - GF is the parenchymal scar (may calcify); Gohn complex includes the hilar node. 4. Biopsy caseous region verses non necrotic region for dx of bacilli for treatment

Answer 60

1. Aspiration from mouth organism T - bacteroides is one of the anaerobic oral flora that is implicated in aspiration pneumonia (others: prevotella, fusobacterium, peptostreptococcus), admixed with aerobic bacteria (S.pneumonia, S.aureus, H.influenza, P.aeruginosa). Resultant pneumonia is partly chemical (irritant gastric acid), and partly bacterial; often necrotizing and may lead to abscess formation in survivors. Abscess would contribute to cachexia - symptoms include clubbing, anaemia, weight loss. Secondary amyloidosis may develop. [Robbins]

Answer 61

3. Symptoms are usually established within 6 hours of trauma / fracture F - syndrome typically begins 1 to 3 days after injury, with sudden onset of tachypnea, dyspnea, and tachycardia. 13. Concerning fat embolism, which of the following is least correct: (GC) 1. Over 80% of fat emboli are asymptomatic T - occurs in 90% of individuals with severe skeletal injuries, but less than 10% of such patients have any clinical findings. Fatal in up to 10% of cases. 2. Features of fat embolism include hypoxia, thrombocytopenia and CNS symptoms T - thrombocytopenia is presumably caused by platelets adhering to the myriad fat globules and being removed from the circulation. Anemia may result as a consequence of erythrocyte aggregation and hemolysis. 3. Symptoms are usually established within 6 hours of trauma / fracture F - syndrome typically begins 1 to 3 days after injury, with sudden onset of tachypnea, dyspnea, and tachycardia. 4. Neurological manifestations include irritability, restlessness, confusion, and coma T - besides pulmonary insufficiency, the syndrome is characterized by neurologic symptoms, including irritability and restlessness, which can progress to delirium or coma. 5. 20-50% of cases are associated with a purpuric rash T - diffuse petechial rash in nondependent areas (occurring in the absence of thrombocytopenia) is seen in 20 to 50% of cases and is useful in establishing a diagnosis. [see Eric's answer 04.48]

Answer 62

3. Hypersensitivity pneumonitis - immunologically mediated response to an extrinsic antigen that involves both immune-complex (type III) and delayed-type (IV) hypersensitivity reactions. Clinically: acute reaction (large loadfever, cough, SOB 4-8hrs post exposure), subacute (acute episodes, background of progressive respiratory deterioration - chronic cough and SOB), or chronic disease. Non caseating granulomas in >2/3 of cases. 15. 35yo outpatient HRCT request ‘Late onset asthma’ worsening dyspnoea, initially episodic and now chronic. Biopsy shows granuloma. Which of the following is most likely with this history: (GC) 1. Silicosis - occupational exposure. Hx: silicotic nodule of whorled collagen fibres, silica particles are weakly birefringent. 2. TB - reactivation (secondary) TB either from reactivation (due to weakened host resistance) or exogenous reinfection (in areas of high contagion). <5% of those with primary disease develop secondary TB. May be asymptomatic if localised; or insidious onset of low-grade fever, night sweats, sputum production, haemoptysis, pleuritic pain; extrapulmonary manifestations. 3. Hypersensitivity pneumonitis - immunologically mediated response to an extrinsic antigen that involves both immune-complex (type III) and delayed-type (IV) hypersensitivity reactions. Clinically: acute reaction (large loadfever, cough, SOB 4-8hrs post exposure), subacute (acute episodes, background of progressive respiratory deterioration - chronic cough and SOB), or chronic disease. Non caseating granulomas in >2/3 of cases. 4. Sarcoid - multisystemic disease of unknown aetiology; non caseating granulomas. 50% may be asymptomatic; 2/3 of symptomatic cases present with gradual onset of SOB, dry cough, vague substernal discomfort, or constitutional symptoms (fever, wt loss, anorexia, fatigue, night sweats). 5. Histoplasmosis - H.capsulatum, endemic in Ohio and central Mississippi region. 3 clinical forms: acute (primary), chronic (cavitary), disseminated miliary (in immunocompromised, infants). Symptoms resemble a flu-like syndrome, most often self-limited. Cavitary disease occurs in vulnerable host (upper lobe, similar to secondary TB) - symptoms of cough, haemoptysis, SOB and chest pain may occur. Hx: granulomas, strikingly similar to TB, differentiation requires identification of yeast forms (PAS or silver stains).

Answer 63

b Proximal part of acini is enlarged , relative or complete sparing of distal acini. 16. Concerning centrilobular emphysema, which of the following is most correct: (JS) a The acini are uniformly enlarged from the level of terminal bronchioles to terminal alveoli. F - Panacinar/panlobular b Proximal part of acini is enlarged , relative or complete sparing of distal acini. T c Proximal part of the acinus normal or near normal with dominant involvement of distal portion. F - Paraseptal/distal acinar d Whole acini destroyed leaving irregular spaces > 1cm in diameter. F - can occur with both panacinar and centrilobular - if severe e Acini uniformly involved but disease effects central zone of secondary pulmonary lobule. F

Answer 64

3. Adenocarcinoma is more likely to be peripheral and affect women, rather than SCC T - 75% are peripheral, most common tumours to affect women and non smokers (but also seen in smokers). SCCs tend to be central, more common in male smokers. 17. Pulmonary adenocarcinoma vs squamous cell carcinoma. Which of the following is most correct: (GC) 1. The 2 are macroscopically similar F - adenocarcinoma usually peripheral, may be associated with scarring, 80% contain mucin. SCC appears as grey-white infiltrative tumour, usually associated with larger, more central bronchi, cavitation in 30%. 2. Adenocarcinomas are more likely to show cavitation F - cavitating tumours = 4/5 SCC, followed by BAC. [Dahnert] 3. Adenocarcinoma is more likely to be peripheral and affect women, rather than SCC T - 75% are peripheral, most common tumours to affect women and non smokers (but also seen in smokers). SCCs tend to be central, more common in male smokers. 4. Adenocarcinoma have a stronger association with emphysema, chronic bronchitis F - implies a heavy smoking history, thus small cell and SCC would have a stronger association. 5. Adenocarcinoma is more likely to show massive lymphadenopathy and extrathoracic disease compared with SCC ?? T - SCC typically a large central mass that spreads to local hilar nodes, but disseminates outside thorax later than other types. Adeno's metastasise widely at an early stage.

Answer 65

1. Only approximately 10-20% with patients with mesothelioma will have associated lower lobe fibrosis T - 90% of malig. mesotheliomas are related to asbestos but only 10% have asbestosis. As opposed to peritoneal mesothelioma, where 50% have asbestosis. [Robbins]18. Patient with markedly thickened pleura encasing one lung. Which of the following statements is most correct? (GC) 1. Only approximately 10-20% with patients with mesothelioma will have associated lower lobe fibrosis T - 90% of malig. mesotheliomas are related to asbestos but only 10% have asbestosis. As opposed to peritoneal mesothelioma, where 50% have asbestosis. [Robbins] 2. Mesothelioma and metastatic adenocarcinoma have markedly different appearances on light microscopy F - differentiated by antigenic keratin positivity, CEA stain negative, presence of long microvilli on EM. 3. Presence of asbestos bodies on pleural biopsy suggest change is more likely reactive / fibrosis F - diffuse pleural thickening is rare, involves visceral pleura (cf. pleural plaques - parietal). Neither of theses entities (or mesothelioma) contain asbestos bodies on histology. 4. Granuloma in pleural tissue suggests chronic irritation rather than neoplastic infective etiology F 5. Apical lung lesion suggests it is most likely infective in nature F

Answer 66

3. 8cm cystic mass F - carcinoid tumours are usually 2-5cm; typical (classic) <2.5cm and atypical >2.5cm. 19. A chest CT request states ‘pulmonary carcinoid’. Which of the following findings would be least expected: (GC) 1. Bronchiectasis T - post obstructive. 2. 1cm endobronchial mass confined to a mainstem bronchus T - typical (classic) carcinoids usually central and manifest as an endobronchial nodule or hilar/perihilar mass close to the bronchus. 3. 8cm cystic mass F - carcinoid tumours are usually 2-5cm; typical (classic) <2.5cm and atypical >2.5cm. 4. Pneumonia T - post obstructive. 5. Hilar lymphadenopathy T - mets to regional lymph nodes in 25%. [Dahnert; Bronchial carcinoid RG 2002]

Answer 67

4. In the presence of a predisposing cause, secondary episodes may occur in up to 30% - T - 30% develop a second embolus 20. Concerning PE, which of the following is most correct: (TW) 1. Approximately 40% lead to infarction - F - 10% (path notes) 2. Emboli affect upper, mid and lower zones equally but not all lobes equally - F - 75% affect lower lobes (path notes). Dahnert says (about) 25% each LL, about 15% for ULs and ML. 3. In the young, PE more frequently leads to infarction - F - May potentially be seen more in young cf old as usually due to abnormal bronchial supply, usually assoc with underlying heart-lung disease, but still as a whole only accounts for 10% of cases. 4. In the presence of a predisposing cause, secondary episodes may occur in up to 30% - T - 30% develop a second embolus 5. Pulmonary haemorrhage in PE implies reperfusion of an infarct - F - collateral circulation of lung - dual pulmonary blood supply and only pulmonary arteries involved in PE. Haemorrhage results without lung infarction. Inadequate bronchial artery supply usually results in lung infarct

Answer 68

3. Hx of chlamydial infection F - C.psittaci (psittacosis: inhaled dust particles of infected bird excreta; most commonly a single lower lobe consolidation). C.pneumoniae (10-20% of adult CAP; usually a single subsegmental lower lobe infiltrate, ARDS has been reported). Can't see that either causes fibrosis. NB. C.trachomatis infection is seen in infants (vertical transmission from infected mum). 21. 57yo male has non specific pulmonary fibrosis. Which of the following is least likely to be related: (GC) 1. Past employment as a stone mason - chronic silicosis may occur in these patients 20-40yrs after exposure (inhalation of crystalline silica dusts). HRCT findings include nodules, interstitial thickening, subpleural curvilinear lines, GGO, parenchymal fibrous bands, traction bronchiectasis, honeycombing. 2. Hx of erosive arthropathy - Rheumatoid lung occurs in up to 50% of patients with RA, lower lobe predominance. 3. Hx of chlamydial infection F - C.psittaci (psittacosis: inhaled dust particles of infected bird excreta; most commonly a single lower lobe consolidation). C.pneumoniae (10-20% of adult CAP; usually a single subsegmental lower lobe infiltrate, ARDS has been reported). Can't see that either causes fibrosis. NB. C.trachomatis infection is seen in infants (vertical transmission from infected mum). 4. Hx of previous chemotherapy - chemotherapeutic agents such as methotrexate, bleomycin, cylcophosphamide and carmustine may cause diffuse lung disease and fibrosis. 5. Muscle biopsy – non caseating granuloma - sarcoidosis may cause myopathy in 25%; fibrosis occurs in 20% of those with pulmonary involvement. End stage sarcoidosis may appear as upper zone fibrosis.

Answer 69

4. SCC - T - production of keratin and intercellular bridges in well-differentiated forms. Pathoutlines: Pink cytoplasm with distinct cell borders and intercellular bridges characteristic for squamous cell carcinoma. 21. FNA of a 52 yo hilar lung mass shown abdundant cells with eosinophilic cystoplasm and intercellular bridges, together with minor necrotic tissue, most characteristic of: (TW) 1. Bronchial carcinoid - F - nests, cords, and massess of cells separated by a delicate fibrous stroma. Regular cells, uniform round nuclei. 2. Nodal involvement with Sarcoidosis - F - noncaseating granulomas, each composed of an aggregate of tightly clustered epithelioid cells, often with Langerhans or foreign body-type giant cells. Laminated concretions (Schaumann bodies), stellate inclusions (asteroid bodies). 3. Bronchogenic cysts - F - lined by bronchial type epitleium and are usually filled with mucinous secretions or with air. 4. SCC - T - production of keratin and intercellular bridges in well-differentiated forms. Pathoutlines: Pink cytoplasm with distinct cell borders and intercellular bridges characteristic for squamous cell carcinoma. 5. Small cell carcinoma of the lung - F - small epithelial cells, little cytoplasm, round or oval and, occasionally, lymphocyte like (but about 2x size of lymphocyte) - "classic oat cell". KCC type 3 (Kulchitsky cell carcinoma, where type 1 is "classic carcinoid", type 2 is "atypical carcinoid).

Answer 70

Answer: Progressive massive fibrosis ROBBINS & RADIOPAEDIA Asbestos-related diseases include - localised fibrous plaques (often calcified), or rarely, diffuse pleural fibrosis - pleural effusions, recurrent - Parenchymal interstitial fibrosis - lung carcinoma (may produced lobar collapse) - Mesothlioma - Larygenal, ovarian, and other extra pulmonary neoplasms Asbestosis - fibrosis begins around respirate bronchioles and alveolar ducts, extends to involve adjacent alveolar sacs and alveoli - fibrous tissue distorts the architecture, creating honeycombing - the pattern of fibrosis I similar to that seen in UIP. - begins in lower lobes and subpleurally. - mediastinal lymphadenopathy can sometimes be present PROGRESSIVE MASSIVE FIBROSIS - refers to formation of large mass-like conglomerates, predominantly in the upper pulmonary lobes, associated with radiating strands - these classically develop in context of certain pneumoconioses (coal workers, silicosis) -CXR: large symmetric bilateral opacities with irregular markings in the upper lobes.

Answer 71

4. PAN - F - PAN typically affects small to medium system arteries, resulting in a necrotizing vasculitis. Involvement of bronchial arteries has been described in PAN, however, the presence of capillaritis or other lung parenchymal involvement by vasculitis strongly suggests another disease process (eg microscopic polyangiitis, Wegener’s, or Churg-Strauss). 24. Least likely to have associated pulmonary findings on HRCT: (TW) 1. SLE - T - a variety of pulmonary abnormalities may be seen in SLE. Pneumonia, lupus pneumonitis, diffuse pulmonary haemorrhage, COP/OP, pulmonary fibrosis (uncommon in SLE compared with other CVDs). 2. Progressive systemic sclerosis - T - Only 1% present with pulmonary symptoms, but up to 75% have evidnce of pulmonary disease at some point in their disease. HRCT findings of interstitial fibrosis in PSS are similar to those of IPF, including: GGO, consolidation, fine reticular opacities, coarse or irregular reticulation, traction bronchietasis, and honeycombing. 3. RA - T - Commonly associated with thoracic abnormalities, including interstitial pneumonia and fibrosis. Histologically NSIP or UIP. HRCT - range of possible findings from GGO to fine reticulation and coarse reticulation with honeycombing. COP / OP may also occur in patients 4. PAN - F - PAN typically affects small to medium system arteries, resulting in a necrotizing vasculitis. Involvement of bronchial arteries has been described in PAN, however, the presence of capillaritis or other lung parenchymal involvement by vasculitis strongly suggests another disease process (eg microscopic polyangiitis, Wegener’s, or Churg-Strauss). 5. Dermatomyosistis – polymyositis - T - PM-DM is less commonly associated with pulmonary involvement than other connective tissue diseases. Reported incidence of pulmonary function abnormalities is 30%, and 5% show chest radiographic abnormalities. Pattern of involvment is typically that of COP / NISP / or UIP. HRCT findings of PM-DM include GGO, fine reticular opacities indicating fibrosis, consolidation, honeycombing (TI and UTD)

Answer 72

Answer: Sides of hilarious and mediastinal nodes involved. Right hilarious mass invading mediastinum ==> T4 Associated hilar, mediastinal lymphadenopathy ==> ?N No other metastases (assuming no other mets means no pleural effusion ==> M0 As per pathoma, N stage is important if M is M0. Tx, T0, Tis T1: <3 cm T2: 3-5 cm OR tumor which (involves main bronchus, invades visceral pleural, associated atelectasis, obstructive pneumonitis, involving all or part of lung. T3: 5-7 cm OR separate tumor nodules in same lobe as primary tumor OR directly invades chest wall, phrenic nerve, parietal pericardium T4: >7 cm OR separate tumor nodule in different ipsilateral lobe OR invades diaphragm, mediastinum, heart, great vessels, trachea, RLN, oesophagus, vertebral body, carina. N0: no regional lymph node N1: ipsilateral peribronchial or hilar or intrapulmoanry nodes N2: ipsilateral mediastinal or subcarinal N3: contralateral mediastinal, hilar OR any scalene/supraclavicular node M0: no metas M1a: separate tumor nodule in contralateral lobe, tumor with pleural or pericardial nodules, malignant pleural or pericardial effusions M1b: sings extra thoracic mets (single organ/non-regional node) M1c: multiple extra thoracic mets.

Answer 73

3. New retroviral treatment T - AIDS-associated (epidemic) KS was originally found in 1/3 of AIDS patients, particularly male homosexuals. However, with current regimens of intensive antiretrovrial therapy, the incidence is now <1% (although still the most prevalent malignancy in AIDS pts in the US). Four forms of KS: 1. Classic KS - typically older men of Eastern European or Mediterranean descent, red-purple skin plaques/nodules esp. on the distal extremities, tumours tend to remain localised to skin and subcutaneous tissue. 2. Endemic KS (aka lymphadenopathic or African) - children of subsaharan Africa (same geographic distribution as Burkitt's), present with lymphadenopathy, occ. involves viscera, extremely aggressive. 3. Transplant- assocd KS - solid organ transplant recipients on long-term immunosuppression, aggressive with nodal, mucosal, and visceral involvement. 4. AIDS-assocd (epidemic) KS [Robbins]

Answer 74

Answer: Progressive primary disease most commonly causes middle or lower lobe consolidation. ROBBINS - Clinically significant disease develops in about 5% of newly infected people (therefore 95% asymptomatic) - Progressive primary tuberculosis more often resembles an acute bacterial pneumonia, with consolidation of the lobe hilarity adenopathy, and pleural effusion.

Answer 75

Answer: Resolved. ROBBINS Cryptogenic organising pneumonia is synonymous with the popular term bronchiolitis obliterates orgnising pneumonia (COP now preferred). It is a clfinicopathologic syndrome of unknown ethology. Sx: cough, dyspnoea + patchy sub pleural or peribronchial areas of airspace consolidation radiographically. Histology: Masson bodies. Underlying lung architecture is normal. No interstitial fibrosis or honeycomb lung. Prognosis: some patients recover spontaneously, but most need treatment with oral steroids for 6 months or longer for complete recovery. DAHNERT - 30% persistent disease, 10% mortality due to preogressive / recurrent disease. 84% improve with Rx.

Answer 76

b. TB bronchopneumonia characterized by 3-5 mm yellow nodules - F - in the highly susceptible, highly sensitized individual, the TB infection may spread rapidly throughout large areas of lung parenchyma "galloping consumption". Sometimes, with such overwhelming disease, well-developed tubercles do not form. (remember centrilobular nodules and tree in bud appearance)

Answer 77

Answer: 6-18 weeks ROBBINS Untreated, the survival time for patients with small cell cancer is 6 to 17 weeks. Even thought SCLC is particularly sensitive to radiation and chemoTx with the potentiazl for cure, most patients hav edistant mets at Dx. At presentation 60-70% of SCLC have extensive stage, 30-40% have limited stage disease. (limited staged get Rx combined modality therapy; extensive stage get chemotherapy alone). From time of Dx - median survival ranges: - limited disease 15-20months - extensive 8-13 months. 2 20-40% limited stage and less than 5% of extensive stage survive two years. 10-13% limited stage and 1-2% extensive stage survive 5y.

Answer 78

Answer: Treatable, but may progress to UIP (true) ROBBINS Desquamative interstitial pneumonia is characterised by large collections of macrophages in the airspaces in a current of former smoker. (Desquamative is a misnomer, as no desquamative pneumocytes) - Histology: smoker's macrophages (dusty brown pigment with finely granular iron). - Interstitial fibrosis when present, is mild. Emphysema is often present. - Presents in the 4th-5th decades. Men = women. All cigarette smokers. - Prognosis: excellent response to steroid therapy and smoking cessation, but occasional patients may progress to interstitial fibrosis. Hamman-Rich syndrome - rapidly progressive non-infectious interstitial lung disease of unknown aetiology - AKA Acute interstitial pneumonitis.

Answer 79

Answer: dDilation of airway distal to terminal bronchioles + distension of airspaces ROBBINS Emphysema is characterised by irreversible enlargement of the airspaces distal to the terminal bronchiole, accompanied by destruction of their walls without obvious fibrosis. 4 major types - Centriacinar (Centrilobular) - Panacinar (pan lobular) - Distal acini (paraseptal) - Airspace enlargement with fibrosis (irregular)

Answer 80

Answer: Bronchoalveolar carcinoma (Adenocarcinoma in-situ) Robbins - Small cell: STRONGEST association with smoking with only 1% of cases occurring in non-smokers. - Squamous cell: highly associated with smoking - Adenocarcinoma: associated with smoking. - Cancers in non-smokers are more common in women, and most are adenocarcinomas.

Answer 81

Answer: Thymic neoplasm Question distinguishes between MG and LEMS (see below) ROBBINS MYASTHENIA GRAVIS Autoimmune disease associated with autoantibodies directed against EACh receptors at the NMJ. Strong association between pathogenic anti-ACh receptor autoantibodies and thyme abnormalities. - 10% of patients with MG have a thymoma - 30% have thyme hyperplasia (B-cell follicles in the thymus) LAMBERT-EATON MYASTHENIC SYNDROME Autoimmune disorder caused by antibodies that block ACh release by inhibiting a presynaptic calcium channel. - In about half the cases, there is an underlying malignancy, most often neuroendocrine carcinoma of the lung. - symptoms may precede the diagnosis of cancer by years.

Answer 82

Carcinoid occur in alveolar cells and deviates rather than invading adjacent bronchi - F - arise from K cells of bronchial mucosa and penetrate the bronchial wall to fan out producing the collar-button lesions 18. Neuroendocrine neoplasm of the lungs – FALSE (JS) a. Small foci of neuro endocrine cells in a scar are inconsequential - T - can have benign tumorlets in areas of scarring or chronic inflammation b. Carcinoid occur in alveolar cells and deviates rather than invading adjacent bronchi - F - arise from K cells of bronchial mucosa and penetrate the bronchial wall to fan out producing the collar-button lesions c. Mitotic rate, lymph node invasion and differentiation important to differentiate between typical and atypical carcinoid but this is difficult with a FNA - T - I presume but can't find anything about FNA diagnosis... d. Small cell is neuroendocrine tumour - T - contain neurosecretory granules similar to those found in Kulchitsky cells

Answer 83

Answer: Proliferative glomerulonephritis ROBBINS Sarcoidosis is a systemic disease of unknown cause that my involve many different tissues and organs. Bilateral hilar lymphadenopathy or lung involvement is most common, occurring in 90% of cases. histology: Non-necrotizing granulomas. Schaumann bodies and asteroid bodies found within 60% of giant cells. Extrathoracic manifestations - Spleen: 3/4 cases but enlarged in only 1/5. - Liver: slightly less affected than spleen. - Ocular: 1/4 cases. Iritis or iridocyclitis - Skin: 1/4 cases. Subcutaneous nodules. Erythema nodosum (tender erythematous plaques. - Bone marrow: 1/5 cases. phalanges of hands and feet - Other: Dilated cardiomyopathy. Lofgren syndrome: Specific acute presentation of systemic sarcoidosis that usually manifests with lymphadenopathy, fevers, erythema nodosum and polyarticualr arthritis. Renal manifestations of sarcoidosis are commonly related to nephrocalcinosis, which results from hypercalcemia. On occasion, the granulomatous process can directly involve the kidney, producing interstitial nephritis and glomerulonephritis. The major histological findings of renal sarcoid that have been described are: FSGS, membranous GN, mesangial proiferative GN, IgA nephropathy, and cresenteric GN. Most patients have proteinuria, clinical nephritic syndrome, and/or HTN. [RG 2004; Atlas of sarcoidosis 2005; Dahnert] OTHER Loffler Syndrome is a simple pulmonary eosinphilia. Erythema marginatum is associated with rheumatic fever (major jones criteria)

Answer 84

Answer: Allergic rhinitis and Gastroenteritis ROBBINS Churg-Strauss syndrome aka Eosinophilic granulomatosis with polyangitiis. - small-vessel necrotising vasculitis - classically associated with asthma, allergic rhinitis, lung infiltrates, peripheral hypereosinophilia, and extravascular necrotising granulomata. ANCAs (mostly MPO-ANCAs i.e. p-ANCAs) are present in less than half the cases. Sx - palpable purpura - GIT bleeding - Renal disease, primarily as focal and segment glomerulosclerosis. - myocardial involvement may give rise to cardiomyopathy. - cardiac involvement is seen in 60% of cases, and accounts for almost half of the deaths in the syndrome. FROM STATDX Clinical issues - Allergic phase: Asthma, allergic rhinitis, sinusitis - Eosinophilic phase: infiltration of lungs, heart, and GI system - Vasculitis phase: Purpura or neuropathy. Constitutional symptom

Answer 85

Answer: Small cell Old question. Oat cell is no longer used, not found in Robbins. STATDX states it is an old term.