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Organ Pathology Seminar / FAQ Respiratory Tract Diseases Jaroslava Dušková Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague Respiratory tract nose & paranasal cavities nasopharynx larynx trachea bronchi LUNG Disease Nosologic Unit Definition Incidence , age/sex prevalence (if any) Etiology Possible clinical manifestation Pathogenesis – – – – macroscopy microscopy ultrastructure or other dg. tools other (nonmorphological) dg. tools Complications !!! Healing & prognosis !!!! Diseases of the nose and paranasal cavities Classification? Most frequent/important ones ? Non-neoplastic – inflammation acute/chronic specific/non-specific superficial/intersticial – pseudotumours Neoplastic Pseudotumours of the nasal & paranasal cavities ? Tumours of the nose & paranasal cavities? Tumours of the nose & paranasal cavities benign (papilloma, adenoma, hemangioma) malignant - carcinoma – adenoca –squamous cell Nasopharyngeal tumours? Nasopharyngeal tumours? angiofibroma nasopharyngeal carcinoma (Schmincke´ lymphoepithelioma ) EBV Larynx -diseases Classification? Most frequent/important ones ? Non-neoplastic – inflammation acute/chronic specific/non-specific superficial/intersticial – pseudotumours Neoplastic Inflammation - Classification: Type of exsudate: serous nonpurulent – lymphoplasmocellular purulent fibrinous gangrenous Laryngeal Pseudotumours ? Laryngeal Neoplasms ? NEOPLASIA – classification HISTOGENETIC mesenchymal epithelial neuroectodermal mixed germ cell, teratoma choriocarcinoma mesotelioma Epithelial Tumours surface epithelium papillomas carcinomas / papillocarcinomas glandular epithelium adenomas adenocarcinomas double diff. mucoepidermoid Laryngeal Neoplasms papilloma carcinoma Bronchi &Trachea -diseases Classification? Most frequent/important ones ? Non-neoplastic – inflammation acute chronic – pseudotumours Neoplastic Ca bronchogenes Definition Incidence , age/sex prevalence (if any) Etiology /risk factors Possible clinical manifestation Pathogenesis – macroscopy !!!!! – microscopy – ultrastructure or other dg. tools – other (nonmorphological) dg. tools Complications Healing & prognosis Macroscopy (x-ray) forms of lung ca central peripheral Pancoast pulmopleural pulmomediastinal multifocal lobar early symptoms surgery possible Horner´ triad x meta x meta x meta x non neopl. dis. Classification of Lung Cancer (Clinical) (biology behaviour) small cell non small cell Small Cell Ca chemotherapy sensitive symptomatic period short distant meta at the time of dg. common expression of the myc oncogen Non Small Cell Ca chemotherapy insensitive surgery (if possible = 20-40%) mutation of K-ras oncogen Histopathology Classification of Lung Cancer small cell – highly malignant , with/without neuroendocrine diff. spinocellular (epidermoid) adenocarcinoma (subtype bronchioloalveolar ca) large cell (undifferentiated) Risk factors for pleural & lung (!) neoplasms ? Lung Cancer - course agressive cough, weight loss, pain, dyspnea 5 yr survival remains in non small cell ca 10% Other Bronchial & Lung Tumours ? LUNG -diseases Classification? Most frequent/important ones ? Non-neoplastic – inflammation acute/chronic specific/non-specific superficial/intersticial – pseudotumours Neoplastic Non-neoplastic – childhood – atelectasis, bronchopulmonary dysplasia,newborn RDS, SIDS – vascular - acute and chronic venostasis – inflammation acute/chronic (obstructive & restrictive lung dis.) specific/non-specific superficial/intersticial – pseudotumours Neoplastic Atelectasis - Collapse Atelectasis – imperfect expansion at birth Collapse – return to airless state Inflammation - Classification: Type of exsudate: serous nonpurulent – lymphoplasmocellular purulent fibrinous gangrenous Interstitial pneumonitis and fibrosis Idiopathic int. Pneumonitis –fibrosing alveolitis Fibrosis without recognized cause, immune complex deposition Progressive dyspnea, resp. failure, cor pulmonale Usual IP (UIP)-acute phase is followed by proliferation of fibroblasts producing Collagen-fibrosis, derangement of alv. walls Desquamative IP (DIP)-macrophages in alveoli,good prognosis Lymphocytic IP (LIP) - extensive inf. of the interstitium with lymphocytes and plasma cells Giant cell IP- after inhalation of fumes of hard metals alloys Hypersensitivity pneumonitisextrinsic allergic alveolitis After inhalation of antigens Farmer´s dis actinomycete in moldy hay Mushroom´s worker´s dis – Bird-fancier´s lung- bird dropping Maple bark stripper´s dis – maple bark Malt worker´s lung – barely malt Suberosis – mouldy cork dust Pituitary snuff takers lung- pituitary snuff Pathology -acute interst. pneumonitis Granulomas with giant cells bronchiolitis fibrosis Hypersensitivity pneumonitis extrinsic allergic alveolitis After inhalation of antigens Farmer´s dis actinomycete in moldy hay Mushroom´s worker´s dis – Bird-fancier´s lung- bird dropping Maple bark stripper´s dis – maple bark Malt worker´s lung – barely malt Suberosis – mouldy cork dust Pituitary snuff takers lung- pitutitary snuff Pathology -acute interst. pneumonitis granulomas with giant cells bronchiolitis fibrosis Emphysema Definition – permanent overdistension of the air passages distal to the terminal bronchioles. It is ass. with destruction of the walls of airspaces within the acini Etiology – cigarette smoking, atmospheric pollution,infection, genetic defect Classification – proximal acinar e.(centriacinar) panacinar em. distal acinar e. irregular e. Symptoms – dyspnea, chronic coughing, chest „barrel shaped“ Ribs almost horizontal, prominent sternoclavicular muscles Pulmonary hypertension, prolonged expiration, respir. acidosis, hypoxia Pathology – lungs are voluminous, pale, edges of lung are rounded, bullae like bubbles at the periphery, the heart is obscured during autopsy Histology – thining and destruction of alv. walls, alveoli are confluent, Large airspaces, capillaries are diminished in number Interstitial emphysema Entrance of air into the connective tissue of the lung, mediastinum and soft tissue Spontaneously – incr. intraalveolar pressure, cough In patiens on respiratore, lung trauma – fractured ribs Symptoms – swelling of the neck and head Crackling crepitation Acute interstitial pneumonia Etiology – viruses- measles v., adenoviruses, cytomegalovirus Chlamydia psittaci Rickettsiae- C. Burneti Mycoplasma pneumoniae Pathology- alveolar septa expanded-hyperemia, lympho-plasmocytic inf. hyaline mebranes viral inclusion bodies multinucleated giant cells- measles, RSV Complication – interstitial fibrosis Asbestosis pleural plaques, diffuse pleural thickening interstitial fibrosis-asbestos bodies mesotheliomas carcinoma of the lung asbestos body – fibre coated with layers of iron containing proteins fine septal scarring, changes in resp. bronchioles macrophages release the cytokines and growth factors proliferation of fibroblasts Silicosis inhalation of silica or silicon dioxide • toxic effect of the crystalline silica on the lysosomal membranes • lysosomal rupture, release of enzymes Pathology: diff. reticular fibrosis small nodules having a whorled pattern fusion of nodules- massive fibrosis Coal miner´s pneumoconiosis Simple form-small black macule containing dust- laden macrophages Macule progress to become nodules containing collagen Complicated form – progressive masive fibrosis Nodules exceeding 1cm in diameter Tuberculosis ? Disease Nosologic Unit Definition Incidence , age/sex prevalence (if any) Etiology Possible clinical manifestation Pathogenesis – macroscopy – microscopy – ultrastructure or other dg. tools – other (nonmorphological) dg. tools Complications Healing & prognosis TUBERCULOSIS Mycobacterium tuberculosis (Koch 1882) Mycobacterium bovis acidoresistance M. avium,intracellulare, Kansasii atypical mycobacterioses Vasculitis & necrotizing granulomas Alergic granulomatosis (Churg-Strauss) Wegener´s granulomatosis TUBERCULOSIS Type of infection childhood (primary, preimmune) adult (postprimary, immune) TUBERCULOSIS Morphological features primary infect (Ghon´s focus) & primary complex caseification isolated organ metastasis tubercle, exsudate, cavity early and late generalisation – milliary spread TUBERCULOSIS Terms –Forms– Locations: phtisis gallopans scrofulosis meningitis basillaris lupus vulgaris mallum Potti, cold absces