key: cord-0036941-q2l7vb9d authors: Farver, Carol F.; Arrossi, Andrea V.; Tazelaar, Henry D. title: Lung date: 2006 journal: Essentials of Anatomic Pathology DOI: 10.1007/978-1-60327-173-8_22 sha: 7ff6ee97347ded6404722e9ad6796aaf421b563e doc_id: 36941 cord_uid: q2l7vb9d nan Intralobar Clinical 0 90% lower lobes; 60% on left; equal incidence in both sexes 0 50% older than 20 years; usually presents with recurrent infections Macroscopic (Figure 1) Firm, cystic area within lobe 0 Arterial supply from elastic artery from thoracic aorta or below the diaphragm 0 No communication with normal tracheobronchial tree 0 Invested by normal visceral pleura Macroscopic ( Figure 6A ) t Diffuse or localized enlarged, fibrotic cartilaginous airways; dilated airways extend to pleural surface; commonly filled with mucopurulent material Microscopic ( Figure 6B) t Ectatic, dilated airways; chronically inflamed wall; follicular bronchitis may be present t Acute and organizing pneumonia is common Temporally uniform injury Patchy, immature fibroblastic proliferation within bronchiolar lumina and peribronchiolar airspaces; usually sharply demarcated with adjacent normal parenchyma 0 Foamy macrophages are commonly found in airspaces surrounding fibrosis I~ Interstitial chronic inflammation and Type II pneumocyte hyperplasia in area of fibrosis Diffuse alveolar damage/acute interstitial pneumonia: -More acute clinical course -More diffuse process, involving both bronchioles and alveoli -Organizing fibrosis is interstitial 0 Usual interstitial pneumonia: -Temporally heterogenous injury -Interstitial fibrosis is predominantly subpleural and paraseptal with scattered fibroblastic foci -Collagen deposition honeycomb foci can be found Clinical 0 Usually middle-aged adults, 90% are smokers; insidious onset of dyspnea 0 Chest X-ray: bilateral, lower lobe, ground-glass opacities 0 Favorable response to corticosteroids 0 Mean survival = 12 years Lung 22-11 Clinical 0 Insidious onset of dyspnea with dry cough, fatigue, and malaise 0 Exposure source not identified in 2/3 of cases diagnosed by pathology; diffuse interstitial infiltrates on chest X-ray 0 Corticosteroids help after exposure has been eliminated Microscopic ( Figure 15 ) 0 Triad of features: interstitial pneumonitis; bronchiolitis with areas of organiziation (BOOP) and ill-formed, nonecrotizing granulomas or giant cells in parenchyma Differential Diagnosis 0 Usual interstitial pneumonia: -Injury is temporally heterogenous with fibroblastic foci -Granulomas usually not seen 0 Sarcoidosis: -Rarely has interstitial pneumonia -Granulomas are well-formed in lymphatic distribution 0 Lymphoid interstitial pneumonia: -Pathology is more diffusely distributed Occurs almost exclusively in smokers; M : F = 4 : 1 ; symptoms may be minimal; 4th decade Chest X-ray: multiple, bilateral nodules 0.5-1.0 cm in upper lung lobes with cystic lesions Microscopic ( Figure 17A , B) Discrete, nodular/stellate lesions; bronchiolocentric 0 Langerhans cell: convoluted (kidney-bean) nuclei lmmunohistochemistry 0 S100+, CDla+, HLR-DR+ Birbeck granule ("tennis racket" morphology) ¢ Occurs exclusively in women of reproductive years ¢ Progressive dyspnea, chylous pleural effusions, recurrent pneumothoraces ¢ Chest X-ray: enlarged lungs; can show cystic or "honeycomb" changes ¢ Found in patients with tuberous sclerosis Macroscopic ( Figure M : F = 9 : 1; young adults; smokers; DRwl5, DQw6 0 Cytotoxic, antibody-mediated, immune reaction; antibodies to basement membrane in serum cross react to both kidney and lung 0 Hemoptysis, anemia, azotemia, and diffuse lung infiltrates Microscopic Capillaritis can be seen, but no large vessel vasculitis 0 Extensive intraalveolar hemorrhage; nonspecific Type II pneumocyte hyperplasia * Reactions of the lung to asbestos with accompanying cations (i.e., iron, calcium, magnesium, sodium); serpentine and amphibole are the most common types Fibrosis occurs 15-20 years after exposure and can progress after exposure stops Firm, fibrotic lungs with areas of honeycomb change Marked interstitial fibrosis with minimal inflammatory infiltrate; UIP-like reactions common * The presence of asbestos bodies, fibrosis, and exposure history are needed for definitive diagnosis ¢ Hyalinizing pleural plaques, pleural fibrosis, and rounded atelectasis can also be seen Usual interstitial pneumonia: -Temporally heterogenous -Lack of asbestos bodies Triad: upper airway, lower airway (lung), and kidney; saddle nose; rarely lung only (so called "limited") 40% c-ANCA+ (anti-proteinase 3) in remission; 90% c-ANCA+ in active disease 0 Chest X-ray: multiple well-demarcated peripheral nodules, lower lobes, rarely as a solitary pulmonary lobule Microscopic ( Figure 26A , B) Triad: parenchymal (basophilic) necrosis, vasculitis, granulomatous inflammation Essentials of Anatomic Pathology, 2nd Ed. Bloodborne or airborne dissemination; immunocom-promised patient, inhalation injuries and chronic obstructive pulmonary disease patient 0 Laryngotracheobronchitis, bronchopneumonia Mixture of acute and chronic inflammation with microabscess formation 0 Silver stain is best for diagnosis: fine, filamentous organisms--may be very difficult to find 0 Weakly acid-fast (Fite's stain) and Gram+ Ziehl-Neelsen stain for acid fast organisms ( Figure 38 ) Auramine-rhodamine more sensitive Wegener's granulomatosis ( Essentials of Anatomic Pathology, 2nd Ed. -Grade A2: frequent perivascular mononuclear infiltrates surrounding venules and arterioles readily recognizable at low magnification -Grade A3: readily recognizable cuffing of venules and arterioles by dense perivascular mononuclear cell infiltrates, usually associated with endo-thelialitis; interstitial mononuclear cell infiltrates -Grade A4: diffuse perivascular, interstitial, and air space infiltrates of mononuclear cells and prominent alveolar pneumocyte damage usually associated with inflammatory cell debris 0 Increased number of neuroendocrine cells 0 Precursor to the development of multiple tumorlets and carcinoids; typical and atypical carcinoids can arise in this setting 0 Usually secondary to airway fibrosis and/or inflammation; rarely, seen as diffuse idiopathic variant Microscopic ( Figure 65A , B) Dense, lymphoid infiltrate in lymphatic distribution; population may be monotonous I Germinal centers are commonly seen; lymphoepithelial lesions can be seen 0 Large mass lesions may be present Airway, vascular, and pleural invasion is common Light chain restriction can usually, but not always, be seen by immunohistochemistry Patients have monoclonal proteins in serum or urine 0 Associated diseases include multiple myeloma, lymphoid interstitial pneumonitis, low-grade lymphomas, and Sjrgren's syndrome Five types: nodular, diffuse, alveolar-septal, senile, tracheobronchial Waxy, hard irregular nodules Microscopic (Figure 69) Amorphous, eosinophilic material in vessels, airway, or as nodules 0 Congo red shows apple green birefringence Kappa light chain disease: -Congo red stain not birefringent Pulmonary hyalinizing granuloma: -Congo red stain birefringent+ Pathology of Tropical and Extraordinary Diseases Chronic eosinophilic pneumonia Lymphangiomyomatosis: physiologic pathologic-radiologic correlations Natural history and treated course of usual and desquamative interstitial pneumonia Pulmonary veno-occlusive disease Quantitative assessment of lung pathology in idiopathic pulmonary fibrosis Histologic diagnosis of extrinsic allergic alveolitis Asbestos fiber content of the lungs in patients with and without asbestos airways disease Pulmonary angiitis and granulomatosis revisited Bronchopulmonary dysplasia in the adult Bronchiolitis obliterans organizing pneumonia Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis Acute diffuse interstitial fibrosis of the lung The pathology of hypertensive pulmonary vascular disease: a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects Bronchocentric granulomatosis, mucoid impaction, and hypersensitivity reactions to fungi Definite alveolar damage: the role of oxygen, shock and related factors Non-specific interstitial pneumonia/fibrosis: histologic patterns and clinical significance Acute interstitial pneumonia: a clinicopathologic, ultrastructural, and cell kinetic study Bronchiolitis obliterans and usual interstitial pneumonia: a comparative clinicopathologic study Burns Amberson lecture: pulmonary angiitis and granulomatosis Desquamative interstitial pneumonia Surgical pathology of the lung in anti-basement membrane antibody associated Goodpasture's syndrome The pulmonary biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis: a study based on 35 open lung biopsies Pulmonary alveolar proteinosis and aluminum dust exposure Bronchocentric granulomatosis: disease or diagnosis? Pathologic manifestations of bronchiolitis, constrictive bronchiolitis, cryptogenic organizing pneumonia, and diffuse panbronchiolitis Respiratory bronchiolitis causing interstitial lung disease: a clinicopathologic study of six cases Hantavirus pulmonary syndrome in the United States: a pathological description of a disease caused by a new agent Giant-cell interstitial pneumonia and hard-metal pneumoconiosis: a clinicopathologic study of four cases and review of the literature Congenital and developmental diseases Acute eosinophilic pneumonia: histopathologic findings in nine patients Pulmonary pathology of the adult respiratory distress syndrome Surgical pathology of the lung in Wegener's granulomatosis: review of 87 open lung biopsies from 67 patients Atypical pathologic manifestations of Pneumocystis carinii pneumonia in the acquired immune deficiency syndrome: review of 123 lung biopsies from 76 patients with emphasis on cysts, vascular invasion, vasculitis, and granulomas Pulmonary Langerhans cell granulomatosis (histiocytosis X): a clinicopathologic study of 48 cases Diseases of the small airways Revision of the 1990 working formulation for the classification of pulmonary allograft rejection: lung rejection study group Molecular aspects of tumor cell invasion and metastasis The pathology of lung cancer-changing concepts and newer diagnostic techniques The molecular biology of lung cancer pathogenesis Dominant oncogenes and tumors suppressor genes in the pathogenesis of lung cancer COMMON LUNG NEOPLASMS American Joint Committee on Cancer. Lung Carcinoma in-situ and early invasive carcinoma occurring in the tracheal bronchial trees in cases of bronchial carcinoma Bronchioalveolar carcinomas: cell types, patterns of growth, and prognostic correlates Pulmonary lymphomas: current concepts Pulmonary lymphomas stimulating lymphomatoid granulomatosis Atlas of Tumor Pathology. Tumors of the Lower Respiratory Tract Prognostic significance of histopathologic subtype and stage in small cell lung cancer Lymphomatoid granulomatosis and angiocentric lymphoma: a spectrum of post-thymic T-cell proliferations Lymphomatoid granulomatosis: a clinicopathologic study of 152 cases The histologic spectrum and significance of clear-cell change in lung carcinoma Association of Directors of Anatomic and Surgical Pathology. Standardization of the surgical pathology report Atypical carcinoid tumor of the lung: a clinicopathologic study of 17 cases Revisions in the International System for Staging Lung Cancer Histological Typing of Lung Tumors Clinical features of adenosquamous lung carcinoma in 127 patients Neuroendocrine tumors of the lung with proposed criteria for large-cell neuroendocrine carcinoma: an ultrastructural, immunohistochemical, and flow cytometric study of 35 cases Long-term follow-up of classical bronchial carcinoid tumors: clinicopathologic observations Primary pathology Hodgkin's disease: a clinicopathologic study of 15 cases Essentials of Anatomic Pathology Large cell carcinoma of the lung Typical and atypical carcinoid tumors of lung: a clinicopathologic and DNA analysis of 20 tumors Benign clear cell tumor (sugar tumor) of the lung Intravascular, bronchiolar and alveolar tumor of the lung (IVBAT): an analysis of twenty cases of a peculiar sclerosing endothelial tumor Tumors and tumor-like lesions of the lung and chest wall in childhood: clinical and pathologic review Localized benign and malignant fibrous tumors of the pleura Pleomorphic (spindle/giant cell) carcinoma of the lung: a clinicopathologic study of 78 cases Clear cell tumor of the lung: a clinicopathologic, immunohistochemical, and ultrastructural study of eight cases Sclerosing hemangioma of the lung: a clinicopathologic study of 51 cases Benign histiocytic tumor of lung: a light-and electron microscopic study Inflammatory pseudotumors of the lung: progression from organizing pneumonia to fibrous histiocytoma or to plasma cell granuloma in 32 cases Sarcomatoid neoplasms of the respiratory tract Mucoepidermoid tumors of the lung Pulmonary hyalinizing granuloma Pulmonary blastoma: an immunohistochemical analysis with comparison with fetal lung in its pseudoglandular stage ¢ Tis: Carcinoma in-situ ¢ TI: Tumor <3 cm in greatest dimension, surrounded by lung or visceral pleura, without bronchoscopic evidence of invasion more proximal than the labor bronchus* (i.e., not in the main bronchus) ¢ T2: Tumor with any of the following features of size or extent: ->3 cm in greatest dimension -Involves main bronchus, 2 cm or more distal to the carina -Invades the visceral pleura -Associated with atelectasis or obstructive pneumonitis that extends to the hilar region but does not involve the entire lung ¢ T3: Tumor of any size that directly invades any of the following: chest wall (including superior suleus tumors), diaphragm, mediastinal pleura, parietal pericardium or tumor in the main bronchus <2 cm distal to the carina, but without involvement of the carina: or associated atelectasis or obstructive pneumonitis of the entire lung ¢ T4: Tumor of any size that invades any of the following: mediastinum, heart, great vessels, trachea, esophagus, vertebral body, carina: or tumor with a malignant pleural or pericardial effusion, or with satellite tumor nodule(s) within the ipsilateral primary tumor lobe of the lung * N:Regional lymph node: -NX: Regional lymph nodes cannot be assessed