There are several excellent recent texts published in the field of pulmonary pathology, so why another? First, this book covers the whole thorax (lung, pleura and pericardium, heart, thymus, and mediastinum), as well as vascular diseases, including vasculitis. Second, its format of bulleted text and extensive illustrations is designed to make it an easy reference for a trainee or practicing pathologist looking up the common and not-so-common diseases when faced with a diagnostic question.A\Within each organ, the contents are organized by disease categories such as congenital, inflammatory, infectious, and neoplastic. The text gives the salient facts in pathogenesis, epidemiology, and clinical features. Diagnostic elements of gross and microscopic pathology are emphasized. These are illustrated by gross pictures and low-, intermediate-, and high-power photomicrographs. Special stains and immunohistochemical stains are given extensively so that users can compare with their own results easily. Because electronic references are so easily accessible, only a few specific references are given.A\Acknowledgment:I would like to thank the residents and pathology assistants who, over the years, have taken many of the gross photographs included in this book. They remain unnamed but are gratefully acknowledged. Aliya N. Husain, MD

I.LUNG 1

A.Normal Lung 2

      Development of Lung 2

      Normal Lung 4

B.Incidental Findings 7

      Meningothelioid (Meningothelial-Like/Meningothelialj Nodule 7

      Osseous Metaplasia 10

      Corpora Amylacea 12

C.Developmental and Pediatric Diseases 13

      Pulmonary Hypoplasia 13

      Congenital Pulmonary Airway Malformation (CRAM) 15

      Pulmonary Sequestration 18

      Bronchogenic Cyst 20

      Peripheral Lung Cysts 21

      Infantile (Congenital) Lobar Emphysema 22

      Interstitial Pulmonary Emphysema (IPE) 24

      Congenital Pulmonary Lymphangiectasis 26

      Alveolar Capillary Dysplasia 28

      Surfactant Dysfunction Disorders 30

      Hyaline Membrane Disease 32

      Bronchopulmonary Dysplasia (BPD) 34

      Pulmonary Interstitial Glycogenosis (PIG) 36

      Chronic Granulomatous Disease |CGD) of Childhood 37

D.Pediatric Tumors 38

      Pleuropulmonary Blastoma 38

      Inflammatory Myofibroblastic Tumor (IMT) 41

      Congenital Pulmonary Myofibroblastic Tumor 43

      Metastatic Pediatric Tumors 44

E.Obstructive Lung (Airway) Diseases 46

      Large Airway Diseases 46

      Bronchiectasis 46

      Cystic Fibrosis 48

      Chronic Bronchitis 50

      Asthma 51

      Allergic Bronchopulmonary Aspergillosis 53

      Small Airway Diseases and Emphysema 55

      Acute Bronchiolitis 55

      Chronic Bronchiolitis 56

      Follicular Bronchiolitis 57

      Bronchiolitis Obliterans/Constrictive Bronchiolitis Obliterans/Constrictive Bronchiolitis 58

      Emphysema 60

F.Restrictive (Interstitial) Lung Diseases 62

      Acute Restrictive Lung Diseases 62

      Pulmonary Edema 62

      Acute Lung Injury (ALI), Acute Respiratory Distress Syndrome (ARDS), Diffuse Alveolar Damage (DAD) 64

      Acute Interstitial Pneumonia (AIP) 66

      Chronic Restrictive Lung Diseases 67

      Usual Interstitial Pneumonia (UIP) 67

      Nonspecific Interstitial Pneumonia (NSIP) 69

      Cryptogenic Organizing Pneumonia (COP) 71

      lgG4-Related Sclerosing Disease 73

      Rheumatoid Arthritis (RA) 75

      Systemic Lupus Erythematosus (SLE) 77

      Scleroderma 79

      Polymyositis and Dermatomyositis-Associated Lung Disease 81

      Sjogren Syndrome (SS) 84

      Inflammatory Bowel Disease (IBD)-Associated Interstitial Lung Disease 86

      Asbestosis 87

      Silicosis 90

      Coal Workers' Pneumoconiosis (CWP) 92

G.Granulomatous Diseases (NONINFECTIOUS) 94

      Sarcoidosis 94

      Hypersensitivity Pneumonitis (HSP) or Extrinsic Allergic Alveolitis 97

      Aspiration Pneumonitis 101

H.SmoKing-Related Diseases 102

      Desquamative Interstitial Pneumonia (DIP) 102

      Respiratory Bronchiolitis-Associated Interstitial Lung Disease (RB-ILD) 104

      Langerhans Cell Histiocytosis (LCH) 105

I.Miscellaneous Diseases 107

      Pulmonary Alveolar Proteinosis (PAP) 107

      Pulmonary Amyloid osis 109

      Eosinophilic Pneumonia 111

      Lipoid Pneumonia 113

      Lymphangioleiomyomatosis 115

      Middle Lobe Syndrome (Right Middle Lobe Syndrome) 117

      Acute Chest Syndrome (ACS) of Sickle Cell Disease 119

      Intravenous Drug Abuse 123

J.Diseases of Vascular Origin 125

      Pulmonary Embolism and Infarction 125

      Arteriovenous Malformation of the Lung 130

      Pulmonary Hypertension 132 Pulmonary Hypertension 132

      Pulmonary Arterial Hypertension (PAH) 133

      Pulmonary Venoocclusive Disease (PVOD) 136

      Pulmonary Capillary Hemangiomatosis (PCH) 138

      Chronic Thromboembolic Pulmonary Hypertension 139

      Pulmonary Hypertension Associated with Lung Diseases or Hypoxemia 141

      Diffuse Pulmonary Hemorrhage Syndromes 142

      Goodpasture Syndrome: Anti-Glomerular Basement Membrane (GBM) Antibody Disease 142

      Idiopathic Pulmonary Hemosiderosis (JPH) 144

      Vasculitis 146

      Pulmonary Wegener Granulomatosis 146

      Pulmonary Churg-Strauss Syndrome 148

K.PULMONARY INFECTIONS 150

      Noninfcctious Pulmonary Lesions in HIV/AIDS Patients 150

      Bacterial Pneumonias 152

      Lobar Pneumonia and Bronchopneumonia 152

      Aspiration Pneumonia 154

      Tuberculosis 155

      Atypical Mycobacterial Infection 157

      Actinomycosis 159

      Nocardiosis 161

      Legionnaires Disease 162

      Lung Abscess 163

      Viral Pneumonia 166

      Lymphocytic Pneumonia 166

      Cytomegalovirus (CMV) Pneumonia 168

      Herpes Simplex Virus (HSV) Pneumonia 170

      Adenovirus Pneumonia 172

      Respiratory Syncytial Virus (RSV) Pneumonia 174

      Measles Pneumonia 176

      Human Metapneumovirus Pneumonia 178

      H1N1 Pneumonia 179

      Severe Acute Respiratory Syndrome (SARS) 180

      Fungal Pneumonia 181

      Aspergillosis 181

      Blastomycosis 184

      Candidiasis 187

      Coccidioidomycosis 188

      Cryptococcosis 190

      Histoplasmosis 193

      Pneumocystis Pneumonia (PCP) 195

      Zygomycosis 197

L.Complications of Therapies 199

      Drug-Induced Lung Injury 199

      Methotrexate 199

      Amiodarone 200

      Bleomycin 202

      Cyclophosphamide 204

      Radiation Therapy 206

      Radiation Pneumonitis 206

      Bacterial Pneumonias 208

      Lung Transplantation 208

      Lung Transplantation: Acute Rejection 210

      Lung Transplantation: Chronic Rejection (Bronchiolitis Obliterate [BO]) 213

      Graft-Versus-Host Disease (GVHDJ and Other Complications of Transplantation in the Lung 215

M.Tumors of Lung 218

      TNM Classification of Primary Non-Small Cell Carcinoma, Small Cell Carcinoma, and Carcinoid Tumor of the Lung 218

      Benign Tumors 221

      Squamous Papilloma 221

      Squamous Papillomatosis 222

      Hamartoma 223

      Chondroma 224

      Pneumocytoma (Sclerosing Hemangioma) 225

      Lipoma of Lung 227

      Pulmonary Leiomyoma 228

      Granular Cell Tumor 229

      Lymphangioma and Lymphangiomatosjs 230

      Rare Benign Tumors of the Lung 231

      Epithelial Proliferations and Tumors 232

      Airway Epithelial Hyperplasia/Metaplasia 232

      Squamous Dysplasia and Carcinoma in Situ (CIS) 234

      Atypical Adenomatous Hyperplasia (AAH) 236

      Adenocarcinoma in Situ (AIS), Formerly Bronchioloalveolar Carcinoma (BAC) 239

      Adenocarcinoma 242

      Squamous Cell Carcinoma (SCC) 251

      Papillary Variant of Squamous Cell Carcinoma (SCC) 254

      Clear Cell Variant of Squamous Cell Carcinoma (SCC) 256

      Small Cell Variant of Squamous Cell Carcinoma (SCC) 258

      Basaloid Variant of Squamous Cell Carcinoma (SCC) 260

      Alveolar Space-Filling Type of Peripheral Squamous Cell Carcinoma (SCC) 262

      Lymphoepithelioma-Like Carcinoma 263

      Adenosquamous Carcinoma 265

      Large-Cell Carcinoma 268

      Sarcomatoid Carcinoma 271

      Pleomorphic Carcinoma 272

      Spindle Cell Carcinoma 274

      Giant-Cell Carcinoma 276

      Carcinosarcoma 277

      Pulmonary Blastoma 279

      Adenoid Cystic Carcinoma 282

      Mucoepidermoid Carcinoma 284

      Epithelial-Myoepithelial Carcinoma 286

      Neuroendocrine Proliferations and Tumors 288

      Diffuse Neuroendocrine Cell Hyperplasia 288

      Carcinoid Tumorlet 290

      Carcinoid Tumor 292

      Typical Carcinoid 296

      Atypical Carcinoid 298

      Small-Cell Carcinoma 300

      Large-Cell Neuroendocrine Carcinoma 304

      Mesenchymal Tumors 307

      Solitary Fibrous Tumor (SFT) of the Lung 307

      Kaposi Sarcoma 310

      Epithelioid Hemangioendothelioma 312

      Pulmonary Synovial Sarcoma (SS) 313

      Pulmonary Artery and Vein Sarcoma 316

      Rhabdomyosarcoma Lung 318

      Miscellaneous Tumors 320

      Perivascular Epithelioid Cell Tumor (PEComa,Clear Cell "Sugar" Tumor) of the Lung 320

      Primary Pulmonary Malignant Melanoma (PPMM) 322

      Lymphoproliferative Disorders 323

      Extranodal Marginal Zone B-Cell Lymphoma of Bronchial-Associated Lymphoid Tissue (BALT Lymphoma) 323

      Lymphoid Interstitial Pneumonia (LIP) (Diffuse Lymphoid Hyperplasia) 326

      Posttransplant Lymphoproliferative Disorder (PTLD) 328

      Diffuse Large B-Cell Lymphoma (DLBCL) of the Lung 330

      Primary Pulmonary Hodgkin Lymphoma (PPHL) 332

      Lymphomatoid Granulomatosis 334

      Primary Pulmonary (Extraosseous) Plasmacytoma 337

      Meta static Tumors 340

      Metastases to the Lungs: Introduction 340

      Metastatic Carcinomas 341

      Metastatic Carcinomas: Breast and Female Genital Tract 344

      Metastatic Carcinomas: Gastrointestinal (Gl) Tract 346

      Metastatic Carcinomas: Genitourinary (GU) Tract and Adrenal 349

      Metastatic Carcinomas: Head and Neck 351

      Metastatic Adult Sarcomas 354

      Benign Metastasizing Leiomyoma (BML) 356

      Metastasizing Giant Cell Tumor 358

      Metastatic Melanoma 360

II.PLEURA AND PERICARDIUM363

A. NONNEOPLASTIC DISEASES 364

      Pleuritis 364

      Mesothelial Hyperplasia 367

      Adenomatoid Tumor of the Pleura 369

B.Neoplastic Diseases 370

      TNM Classification of Pleural Mesothelioma 370

      Well-Differentiated Papillary Mesothelioma (WDPM) 373

      Malignant Mesothelioma of Pleura 375

      Epithelioid Malignant Mesothelioma 377

      Sarcomatoid Malignant Mesothelioma 382

      Desmoplastic Malignant Mesothelioma 385

      Biphasic Malignant Mesothelioma 387

      Solitary Fibrous Tumor (SFT) of Pleura and Pericardium 389

      Calcifying Pseudotumor of Pleura/Calcifying Fibrous Pseudotumor 391

      Pleural Synovial Sarcoma (SS) 392

      Primitive Neuroectodermal Tumor/Extraskeletal Ewing Sarcoma 394

      Desmoplastic Small Round Cell Tumor of Pleura (DSRCT) 396

      Primary Effusion Lymphoma (PEL) 397

      Metastases to Pleura 399

III.THYMUS 403

A.Normal ThyMus 404

      Normal Thymus 404

B.NONNEOPLASTIC DISEASES 407

      Unilocular and Multilocular Thymic Cysts 407

      Thymic Dyspiasia 409

      Acute Involution of Thymus (Stress-Induced Thymic Involution) 410

      True Thymic Hyperplasia 412 Lymphoid Hyperplasia of the Thymus 413

C.ThYMoma 414

      General Features of Thymoma 414

      WHO Type A Thymoma 416

      WHO Type AB Thymoma 418

      WHO Type B1 Thymoma 419

      WHO Type B2 Thymoma 421

      WHO Type B3 Thymoma 423

D.Other Thymic Tumors 425

      Thymic Carcinoma 425

      Thymic Neuroendocrine Carcinoma (NEC)/Tumor 430

      Thymolipoma 433

IV.MEDIASTINAL LESIONS 435

A.Inflammatory Infectious Lesions 436

      Mediastinitis 436

B.Cystic Lesions 438

      Mediastinal Bronchogenic Cyst 438

      Esophageal Cyst 439

      Mediastinal Gastroenteric Cyst 440

      Pericardial Cyst 441

C.Ectopic Lesions 442

      Mediastinal Ectopic Thyroid 442

      Ectopic Parathyroid 443

D.Tumors 444

      Neurogenic Tumors 444

      Mediastinal Schwannoma (Neurilemmoma) 444

      Mediastinal Neurofibroma 446

      Mediastinal Malignant Peripheral Nerve Sheath Tumor (MPNST) 448

      Neuroblastic Tumors 450

      Mediastinal Neuroblastoma 450

      Mediastinal Ganglioneuroblastoma 453

      Mediastinal Ganglioneuroma 455

      Mediastinal Paraganglioma 457

      Mediastinal Paraganglioma 457

      Germ Cell Tumors 459 Germ Cell Tumors (GCTs) of Mediastinum 459

      Lymphoproliferative Disorders 463

      Hodglcin Lymphoma of the Mediastinum 463

      Mediastinal T-Lymphoblastic Lymphoma (T-LBL) 466

      Primary Mediastinal (Thymic] Large B-Cell Lymphoma (PMLBCL) 468

      Ana plastic Large-Cell Lymphoma (ALCL) of the Mediastinum 470

      Myeloid Sarcoma (MS) 472

      Mediastinal Castleman Disease (CD) 474

      Follicular Dendritic Cell (FDCJ Sarcoma 476

      Mesenchymal Tumors 478

      Mediastinal Lymphangioma 478

      Mediastinal Liposarcoma 480

      Rhabdomyosarcoma 482

V.HEART AND VESSELS 485

A.Normal Heart 486

      Normal Heart 486

B.Congenital Heart DiseasE 489

      Atrial Septal Defect (ASD) 489

      Ventricular Septal Defect (VSD) 490

      Hypoplastic Left Heart Syndrome (HLHS) 491

      Tetralogy of Fallot 492

C.Valvular Diseases 493

      Degenerative Valvular Disease 493

      Bicuspid Aortic Valve (BAV) 493

      Aortic Valve Stenosis 494

      Aortic Valve Insufficiency 496

      Mitral Valve Stenosis 497

      Mitral Valve Regurgitation 498

      Prosthetic Valves 499

      Endocarditis 500

      Infectious Endocarditis 500

      Nonbacterial Thrombotic Endocarditis (NBTE) 502

      Rheumatic Heart Disease 503

      Acute Rheumatic Fever and Rheumatic Heart Disease (RHD) 503

D.Myocardial Diseases 506

      Lymphocytic Myocarditis 506

      Eosinophilic Myocarditis 508

      Giant Cell Myocarditis 509

      Cardiac Amyloidosis (Amyloid Cardiomyopathy) 510

      Cardiac Sarcoidosis 512

      Dilated Cardiomyopathy 514

      Hyper trophic Cardiomyopathy 516

      Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) 518

      Cardiac Hemochromatosis 520

      Hypertensive Heart Disease 522

      Drug Cardiotoxicity 523

E.IscheMic Heart Disease 524

      Myocardial Infarction (Ml) 524

F.Heart Transplantation 527

      Heart Transplantation: Introduction 527

      Antibody-Mediated Rejection of the Heart 529

      Acute Cellular Rejection of the Heart 530

      Chronic Rejection of the Heart (Cardiac Allograft Vasculopathy [CAV]) 532

      Quilty Lesion 534

G.InfectionS 535

      Opportunistic Cardiac Infections 535

H. Tumors of the Heart 537

      Benign Tumors 537

      Cardiac Myxoma 537

      Cardiac Rhabdomyoma 539

      Cardiac Fibroelastoma 541

      Cardiac Fibroma 543

      Cardiac Hemangioma 545

      Cardiac Lipoma 547

      Lipomatous Hypertrophy of the Interatrial Septum 548

      Malignant Tumors 549

      Angiosarcoma of the Heart 549

      Synovial Sarcoma (SS) of the Heart 551

      Rare Cardiac Sarcomas 553

      Primary Cardiac Lymphoma (PCL) 555

      Cardiac Metastasis 557

I.Diseases of Vessels 559

      Atherosclerosis 559

      Thoracic Aortic Aneurysm 561

      Aortic Dissection 563

      Infectious Vasculitis 565

      Giant Cell Arteritis 566

      Takayasu Arteritis 568

      Kawasaki Disease 569

      Churg-Strauss Syndrome 570

      Polyarteritis Nodosa 572

      Microscopic Polyangiitis 574

      Thromboangiitis Obliterans 576

      Wegener Granulomatosis 578

中国医科院医学信息研究所