22 Lung Carol F. Farver, MD, Andrea V. Arrossi, MD, and Henry D. Tazelaar, MD CONTENTS I. Non-neoplastic Diseases Interstitial Diseases ........................................ 22-9 of the Lung...................................... 22-4 Acute Lung Injury ................................ 22-9 Congenital Anomalies and Pediatric Lesions ....22-4 Diffuse Alveolar Damage Pulmonary Sequestration ...................... 22-4 (DAD)/Acute Interstitial Intralobar ...................................... 22-4 Pneumonia (AIP) .................... 22-9 Extralobar .................................... 22-4 Cryptogenic Organizing Bronchogenic Cysts .............................. 22-4 Pneumonia (COP)/ Congenital Pulmonary Airway Bronchiolitis Obliterans Malformation .................................. 22-4 Organizing Pneumonia Pulmonary Lymphangiomatosis ............ 22-5 (BOOP) .................................. 22-10 Bronchopulmonary Dysplasia .............. 22-5 Idiopathic Interstitial Pneumonias ...... 22-10 Infantile Lobar Emphysema .................. 22-5 Desquamative Interstitial Pediatric Interstitial Lung Disease ........ 22-6 Pneumonia (DIP) .................. 22-10 Airways and Obstructive Diseases ........ 22-6 Usual Interstitial Pneumonia Emphysema .................................. 22-6 (UIP) ...................................... 22-11 Large Airway Disease ............................ 22-7 Non-Specific Interstitial Bronchiectasis .............................. 22-7 Pneumonia/Fibrosis .............. 22-11 Chronic Bronchitis ...................... 22-7 Other Interstitial Diseases .................... 22-12 Asthma ........................................ 22-7 Extrinsic Allergic Alveolitis Bronchocentric Granulomatosis....22-8 (Hypersensitivity Small Airway Disease ............................ 22-8 Pneumonitis).......................... 22-12 Respiratory Bronchioloitis Eosinophilic Pneumonia ............ 22-12 (Smokers') .............................. 22-8 Eosinophilic Granuloma Respiratory Bronchiolitis- (Pulmonary Histiocytosis-X/ Associated Interstitial Lung Langerhans' Cell Disease .................................... 22-8 Granulomatosis) .................... 22-13 Follicular Bronchiolitis ................ 22-9 Sarcoidosis ................................ 22-13 Constrictive (Obliterative) Pulmonary Alveolar Bronchiolitis ............................ 22-9 Proteinosis ............................ 22-14 Diffuse Panbronchiolitis .............. 22-9 Lymphangio(leio) Cellular Bronchiolitis .................. 22-9 myomatosis ............................ 22-14 915 22-2 Essentials of Anatomic Pathology, 2nd Ed. Goodpasture's Disease Lipoma ...................................... 22-29 (Anti-Basement Membrane Mesenchymal Cystic Antibody Disease Hamartoma ............................ 22-30 [ABMA]) .............................. 22-15 Pre-Invasive Lesions ...................................... 22-30 Idiopathic Pulmonary Malignant Tumors ........................................ 22-31 Hemosiderosis ...................... 22-15 Tumors of Salivary Gland Type .......... 22-31 Pneumoconioses .................................. 22-15 Mucoepidermoid Carcinoma ...... 22-31 Silicosis ...................................... 22-15 Adenoid Cystic Carcinoma ........ 22-31 Asbestos-Related Reactions ...... 22-16 Epithelial Tumors ................................ 22-31 Coal Worker's Squamous Cell Carcinoma ........ 22-32 Pneumoconiosis (CWP) ........ 22-16 Adenocarcinoma ........................ 22-32 Hard Metal Pneumoconiosis ...... 22-16 Small Cell Carcinoma ................ 22-34 Berylliosis .................................. 22-17 Large Cell Undifferentiated Vascular Conditions ...................................... 22-17 Carcinoma ............................ 22-34 Vasculitides .......................................... 22-17 Adenosquamous Carcinoma ...... 22-35 Wegener's Granulomatosis ........ 22-17 Carcinomas with Pleomorphic, Churg-Strauss Syndrome Sarcomatoid, and (Allergic Angiitis Sarcomatous Elements .......... 22-35 Granulomatosis) .................... 22-18 Carcinoid Tumorlet .................... 22-35 Necrotizing Sacroid Typical and Atypical Granulomatosis (NSG) ........ 22-18 Carcinoid .............................. 22-36 Necrotizing Capillaritis .............. 22-18 Large Cell Neuroendocrine Pulmonary Hypertension .................... 22-19 Carcinoma ............................ 22-36 Plexogenic Arteriopathy ............ 22-19 Unusual Tumors with Pulmonary Veno-Occulsive Neuroendocrine Disease with Secondary Differentiation ...................... 22-37 Pulmonary Arterial Mesenchymal Tumors .......................... 22-37 Hypertension ........................ 22-19 Fibrous and Fibro-Histiocytic Thrombotic Arteriopathy: Tumors .................................. 22-37 Pulmonary Infarction ............ 22-20 Inflammatory Pulmonary Capillary Pseudotumor ................ 22-37 Hemangiomatosis .................. 22-20 Malignant Fibrous Infections ...................................................... 22-20 Histiocytoma ........................ 22-37 Viral .................................................... 22-20 Smooth Muscle Bacteria ................................................ 22-22 Tumors--Leiomyosarcoma....22-38 Mycoplasma pneumoniae .................... 22-24 Skeletal Muscle Tumor-- Fungal .................................................. 22-24 Rhabdomyosarcomas ............ 22-38 Protozoan ............................................ 22-26 Vascular Tumors and Related Lung Transplantation .................................... 22-27 Conditions ...................................... 22-38 Histologic Grading of Pulmonary Vascular Malformations ............ 22-38 Allograft Rejection ........................ 22-27 Epithelioid Hemangioendothelioma II. Neoplastic Diseases of the Lung ...... 22-28 (Intravascular Benign Tumors .............................................. 22-28 Bronchioloalveolar Tumors)....22-38 Benign Epithelial Tumor .................... 22-28 Kaposi's Sarcoma ...................... 22-39 Squamous Papillomas and Angiosarcoma ............................ 22-39 Papillomatosis ...................... 22-28 Other Vascular Tumors .............. 22-39 Papillary Adenorna of Neurogenic Tumors .................... 22-40 Type II Cells .......................... 22-28 Pleuropulmonary Blastoma ................ 22-41 Alveolar Adenoma .................... 22-28 Lymphoproliferative Lesions Mucus Gland Adenoma .............. 22-28 of the Lung ...................................... 22-41 Mucinous Cystadenoma ............ 22-29 Nodular Lymphoid Benign Mesenchymal Tumors ............ 22-29 Hyperplasia Hamartoma ................................ 22-29 (Pseudolymphoma) ................ 22-41 916 Lung 22-3 Lymphocytic Interstitial Benign Clear Cell (Sugar) Pneumonitis (Diffuse Tumor .................................... 22-43 Lymphoid Hyperplasia) ........ 22-41 Minute Pulmonary Giant Lymph Node Meningothelial-Like Lesion Hyperplasia (Castleman's (Minute Pulmonary Disease) ................................ 22-41 Chemodectoma) .................... 22-44 Lymphomas .......................................... 22-41 Granular Cell Tumor Low Grade Lymphoma (Granular Cell of the Mucosa Associated Myoblastoma) ........................ 22-44 Lymphoid Tissue (MALT) .... 22-41 Masses and Tumor-Like Lesions ........ 22-44 Angiocentric Pulmonary Amyloidosis ............ 22-45 Immuno-proliferative Pulmonary Hyalinizing Lesions/Angiocentric Granuloma ............................ 22-45 Lymphomas/Lymphomatoid Inflammatory Myofibroblastic Granulo-matosis/ Tumor .................................... 22-45 Polymorphic Reticulosis ......22-42 Tracheobronchopathia Post-Transplant Lymphoproli- Osteoplastica ........................ 22-46 ferative Disorder ............................ 22-43 Benign Metastasizing Other Lymphoproliferative Lesions .... 22-43 Leiomyoma............................ 22-46 Intravascular Lymphomatosis Tumors of the Pleura .......................... 22-46 (Angiotropic Lymphoma) ...... 22-43 Malignant Mesothelioma .......... 22-46 Primary Pulmonary Localized Fibrous Tumor Hodgkin's Disease ................ 22-43 of the Pleura .......................... 22-47 Plasmacytoma ............................ 22-43 Mast Cell Tumor ........................ 22-43 III. TNM Classification of Lung Tumors of Uncertain Cancer (2002 Revision) ................ 22-47 Histogenesis .......................... 22-43 Sclerosing Hemangioma....22-43 IV. Suggested Reading ............................ 22-48 917 22-4 Essentials of Anatomic Pathology, 2nd Ed. NON-NEOPLASTIC DISEASES OF THE LUNG Congenital Anomalies and Pediatric Lesions PULMONARY SEQUESTRATION 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 Microscopic 0 Young patients: pathology may be normal 0 Older patients: pathology shows chronic obstructive pneumonia; honeycomb changes are common Extralobar Clinical 0 60% are found in children <1 year; 90% on left side; M:F=4:I 0 Frequently found with repair of diaphragmatic defect 0 60% have other congenital anomalies such as diaphragmatic hernia or pectus excavatum (funnel chest) Macroscopic 0 Spongy, pyramidal mass outside of the normal pleura; Fig. 1.