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 v acute/chronic v specific/non-specific v 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?

 angiofibroma  nasopharyngeal carcinoma (Schmincke´ lymphoepithelioma )  EBV

Larynx - diseases

Classification? Most frequent/important ones ?

 Non-neoplastic – inflammation v acute/chronic v specific/non-specific v superficial/intersticial – pseudotumours  Neoplastic

Inflammation - Classification: Type of exsudate:  serous  nonpurulent – lymphoplasmocellular  purulent  fibrinous  gangrenous

Laryngeal Pseudotumours ?

Laryngeal Neoplasms ?

NEOPLASIA – classification HISTOGENETIC v mesenchymal v epithelial v neuroectodermal v mixed v germ cell, teratoma choriocarcinoma v mesotelioma

Epithelial Tumours v surface epithelium papillomas carcinomas / papillocarcinomas v glandular epithelium adenomas adenocarcinomas v double diff. mucoepidermoid

Laryngeal Neoplasms  papilloma  carcinoma

Bronchi &Trachea - diseases

Classification? Most frequent/important ones ?

 Non-neoplastic –inflammation v acute v 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 early symptoms  peripheralsurgery possible  PancoastHorner´ triad  pulmopleuralx meta  pulmomediastinalx meta  multifocalx meta  lobarx 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 v acute/chronic v specific/non-specific v superficial/intersticial – pseudotumours  Neoplastic

 Non-neoplastic – childhood – atelectasis, bronchopulmonary dysplasia,newborn RDS, SIDS – vascular - acute and chronic venostasis – inflammation v acute/chronic (obstructive & restrictive lung dis.) v specific/non-specific v superficial/intersticial –pseudotumours  Neoplastic

Atelectasis – imperfect expansion at birth Collapse – return to airless state Atelectasis - Collapse

Inflammation - Classification: Type of exsudate:  serous  nonpurulent – lymphoplasmocellular  purulent  fibrinous  gangrenous

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 Interstitial pneumonitis and fibrosis

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 Hypersensitivity pneumonitis - extrinsic allergic alveolitis

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 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 Interstitial emphysema

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 Acute interstitial pneumonia

 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 Asbestosis

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 Silicosis inhalation of silica or silicon dioxide

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 Coal miner´s pneumoconiosis

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 v childhood (primary, preimmune) v 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