The Thorax: Medical, Radiological, and Pathological Assessment

Description:

The current medical practice has become more of a team effort rather than an isolated practice.  The current evaluation of patients in the daily practice is essentially performed by medical assessment of the patient in question followed by diagnostic imaging, and when needed and possible the evaluation of tissue for diagnosis with its subsequent assessment of biomarkers and other ancillary tools that play an important role in the evaluation and prognosis.  The book herein proposed will exactly provide such assessment focused in the thoracic assessment of patients afflicted with any particular disease of the thorax.  Expert clinician in pulmonary medicine will provide the state of the art in the evaluation of such patients, which will provide the most important background in the clinical impression and further assessment of these patients.  This assessment in most cases is followed by the evaluation of imaging, which provides a highly important information of not only the exact location of the process but also of the nature of whether is localized, infiltrative, diffuse, bilateral, etc., as well as the possible compromise of other adjacent structures.  Such information is crucial as imaging and clinical information will provide a working diagnosis, which ultimately will be defined by the pathological assessment.  Therefore, in real practice neither one of these subspecialties works alone or in isolation.  On the contrary, each one depends on the other for the final diagnosis and proper management of patients with thoracic diseases.  Based on such experience is that the current text will provide in the same text of the needed information that a clinician, radiologist or pathologist will need in order to arrive to the best possible conclusion.
The scope of this book is unique in its nature as currently, even though there are several text on either one of those specialties, those publication are strictly on the either radiology, medicine or pathology.  Our proposed text will bring all those together in a single text and written by experts in their respective field.  This book should be of interest to all of those in the practice of thoracic medicine (radiologist, pulmonologist, pathologists) and any one who in the general practice is confronted with the evaluation of these patients.

Preface

The thorax is a host of a wealth of conditions that range from reactive, infectious, idiopathic, benign, and malignant tumoral entities representing all the different lineages and etiologies. It is important to highlight that the diagnosis of numerous conditions requires strong clinical information and expert radiological interpretation so that the histopathological findings can be placed in the proper perspective. Also, because the thorax is a common host for metastatic diseases, it is imperative that those involved in the practice and management of patients suffering from thoracic conditions adhere to the practice of proper clinical-radiological-pathological correlations in order to clearly define the process that may be affecting either the pleura, lung, or mediastinum. Although in some instances the finding of a pulmonary mass or a mediastinal mass may be enough, more often than not, the correlation of clinical and pathological findings aids extensively in the understanding of whatever the process might be.

In the current text, we provide the collective experience of the many authors who have provided us with their expertise and knowledge to further advance the understanding of thoracic diseases. More importantly is the emphasis that we provide in terms of close clinicalradiological-pathological correlations in the numerous entities addressed in this text. We are also aware that as inclusive as we have been, there are entities that have escaped discussion. However, the text facilitates the daily practice of the most common and also unusual conditions that may affect the thorax.

We are fortunate and indebted to the numerous authors who contributed their knowledge to make this text a companion of the daily practice, regardless of whether your interest lies on the medical, radiological, or pathological information of the entities herein presented. The goal of the text is to provide an overall knowledge of the three subspecialties and how global interpretation of many entities can be consolidated so that we can bring it to the bedside whenever it is necessary.

Finally, we hope that this text further improves the communication that must exist among clinicians, radiologists, and pathologists involved in the management of thoracic conditions, which in the end can be translated into better patient care.

Table of contents :

Preface
Acknowledgments
Contents
Contributors
Part I: Serosal Surface
1: The Pleura
Introduction
Pleural Anatomy
General Radiology Concepts
Nonneoplastic
Fibrous and Fibrinous Pleuritis
Histological Features
Pleural Infections
Histopathological Features
Uremia
Hemothorax
Coronary Artery Bypass Surgery
Apical Pleural Plaques
Pneumoconiosis
Connective Tissue Diseases
Drug-Induced Fibrous Pleuritis
IgG4-Related Fibrous Pleuritis
Pleuroparenchymal Fibroelastosis
Mesothelial Hyperplasia
Pleural Endometriosis
Adenomatoid Tumor
Neoplastic
Malignant Mesothelioma
Radiography
Computed Tomography
Magnetic Resonance Imaging
PET/CT
Ultrasound
Limitations of Routine Imaging
Therapy Response Assessment
Volumetric and Future Imaging of MPM
Staging
Pathological Features
Macroscopic Features
Microscopic Features
Malignant Mesothelioma In Situ
Invasive Malignant Mesothelioma
Immunohistochemical Features
Molecular Analysis
Pseudomesotheliomatous Adenocarcinoma
Pathological Features
Macroscopic Features
Microscopic Features
Immunohistochemical Features
Pleural Thymoma
Pathological Features
Macroscopic Features
Histological Features
Immunohistochemmical Features
Primary Salivary Gland Type Tumors of the Pleura
Mucoepidermoid Carcinoma (MEC)
Epithelial-Myoepithelial Carcinoma
Solitary Fibrous Tumor
Pathological Features
Macroscopic Features
Histological Features
Immunohistochemical Features
Desmoid Tumor
Pathological Features
Macroscopic Features
Histological Features
Immunohistochemical Features
Calcifying Fibrous Tumor
Pathological Features
Macroscopic Features
Histological Features
Immunohistochemical Features
Vascular Neoplasms
Epithelioid Hemangioendothelioma
Pleural Angiosarcoma
Histopathological Features
Epithelioid Hemangioendothelioma (EH)
Pleural Angiosarcoma
Immunohistochemical Features
Molecular Features
Neuroectodermal Tumors
Pathological Features
Macroscopic Features
Microscopic Features
Immunohistochemical Features
Molecular Features
Other Pleural Sarcomas
Primary Smooth Muscle Tumors of the Pleura
Synovial Sarcoma
Pathological Features
Macrocopic Features
Microscopic Features
Immunohistochemical Features
Molecular Features
Body Cavity Lymphoma
Primary Effusion Lymphoma
Pyothorax-Associated Lymphoma
Pleural Manifestations of Hematologic Malignancy
Pleural Metastasis
Pleural Effusions
Epidemiology and Associated Conditions
Management
References
Part II: The Mediastinum
2: Normal Thymus
Radiological Aspects of the Mediastinum
Mediastinal Compartments
Imaging Modalities
The Thymus
Practical Embryological Aspects of the Thymus
Histological Features
Other Cellular Components
Thymic Involution
Immunohistochemical Features
References
3: Thymoma
Epidemiological Features
Clinical Features
Diagnostic Imaging
Histological Classification
Pathological Staging
Pathological Features
Clinical Outcome
References
4: Thymic Carcinoma
Pathogenesis
Diagnostic Criteria
Epidemiological Features
Clinical Features
Diagnostic Imaging
Pathological Staging
Pathological Features
Thymic Adenocarcinoma
NUT Carcinoma
Salivary Gland Type Carcinomas
Clinical Features
Mucoepidermoid Carcinoma
Adenoid Cystic Carcinoma
Epithelial-Myoepithelial Carcinoma
Basaloid Carcinoma
References
5: Neuroendocrine Neoplasms
Neuroendocrine Carcinomas
Clinical Features
Diagnostic Imaging
Pathological Staging
Histological Grading and Nomenclature
Pathological Features
Macroscopic Features
Microscopic Features
Immunohistochemical Features
Paraganglioma
Diagnostic Imaging
Pathological Features
Macroscopic Features
Histopathological Features
Immunohistochemical Features
Parathyroid Tumors
Diagnostic Imaging
Pathological Features
Macroscopic Features
Microscopic Features
Immunohistochemical Features
References
6: Mediastinal Germ Cell Tumors
Mediastinal Germ Cell Tumors
Estimated Occurrence
Classification
Staging
Clinical Symptomatology
Diagnostic Imaging
Pathological Features
Teratoma
Immunohistochemical Features
Seminoma
Non-Seminomatous Germ Cell Tumors
Yolk Sac Tumor
Embryonal Carcinoma
Choriocarcinoma
Immunohistochemical Features
Clinical Outcome
References
7: Mesenchymal Tumors of the Mediastinum
Vascular Tumors
Lymphangioma
Hemangioma
Epithelioid Hemangioendothelioma
Angiosarcoma
Fibroblastic and Fibrohistiocytic Tumors
Solitary Fibrous Tumor
Sclerosing Epithelioid Fibrosarcoma
Low-Grade Fibromyxoid Sarcoma
Neurogenic Tumors
Benign Peripheral Nerve Sheath Tumors
Malignant Peripheral Nerve Sheath Tumors (MPNST)
Ganglionic Tumors
Myogenic Tumors
Smooth Muscle Tumors
Skeletal Muscle Tumors
Lipomatous Tumors
Benign Lipomatous Tumors
Malignant Lipomatous Tumors
Bone and Cartilaginous Tumors
Tumors of Unknown Etiology
References
8: Mediastinal Lymphoproliferative Disorders
Benign Lymphoproliferative Lesions of the Mediastinum
Introduction
Sarcoidosis
Granulomatous Lymphadenitis Caused by an Infectious Organism or a Known External Substance
Castleman Disease
Hyaline Vascular Castleman Disease
Plasma Cell-Rich Castleman Disease
Sclerosing Mediastinitis with Emphasis in Mediastinal IgG4-Related Disease
Langerhans Cells Histiocytosis (LCH)
Rosai-Dorfman Disease (RDD)
Malignant Lymphoproliferative Disorders and Other Hematopoietic Disorders of the Mediastinum
Introduction
Non-Hodgkin Lymphomas of B-Cell Origin
Primary Mediastinal (Thymic) Large B-Cell Lymphoma (PM-LBCL)
Mediastinal Diffuse Large B-Cell Lymphoma (DLBCL)
B-Cell Lymphoma, Unclassifiable, with Features Intermediate Between Diffuse Large B-Cell Lymphoma and Classic Hodgkin Lymphoma/Mediastinal Gray Zone Lymphoma (M-GZL)
Thymic Marginal Zone Lymphoma of the Mucosa-Associated Lymphoid Tissue (Thymic MALT Lymphoma)
Mediastinal Plasmacytoma
Non-Hodgkin Lymphomas of T-Cell Origin
T-Lymphoblastic Leukemia/Lymphoma (T-ALL/ LBL)
Anaplastic Large Cell Lymphoma (ALCL)
Classic Hodgkin Lymphoma (CHL)
Lymphoma Imaging
Lymphoma Imaging Classification
Diffuse Large B Cell Lymphoma
Primary Mediastinal (Thymic) Large B Cell Lymphoma
Hodgkin Lymphoma
Myeloid Neoplasms: Mediastinal Myeloid Sarcoma
Other Tumors: Follicular Dendritic Cell (FDC) Sarcoma
References
9: Miscellaneous Conditions
Fibrosing/Sclerosing Mediastinitis
Diagnostic Imaging
Pathological Features
Multilocular Thymic Cyst (MTC)
Diagnostic Imaging
Pathological Features
Thymic Hyperplasia and Thymic Hyperplasia with Lymphoepithelial Sialadenitis (LESA)-Like Features
Diagnostic Imaging
Pathological Features
Ependymoma
Pathological Features
References
Part III: Lung: Neoplastic Conditions
10: Non-Small Cell Carcinoma
Introduction
Epidemiology
Incidence and Prognosis
Risk Factors
Clinical Presentation
Imaging Modalities
Staging of Lung Cancer
T Classification
N Classification
M Classification
Special Considerations in TNM-8
Imaging Characteristics
Non-Small Cell Carcinoma
Squamous Cell Carcinoma
Adenocarcinoma
Invasive Adenocarcinoma
Large Cell Carcinoma
Adenosquamous Carcinoma
Lymphoepithelioma-Like Carcinoma
Sarcomatoid Carcinoma
Pathological Features
Squamous Cell Carcinoma
Invasive Squamous Cell Carcinoma
Histological Variants
Adenocarcinoma
Histological Variants
Atypical Adenomatous Hyperplasia, In Situ Adenocarcinoma, Minimally Invasive Adenocarcinoma, Adenocarcinoma with Lepidic Growth Pattern, Brochioloalveolar Carcinoma
Adenocarcinoma, Histological Variants
Other Uncommon Variants
Adenosquamous Carcinoma
Pathological Features
Other Unusual Variants of Non-Small Cell Carcinoma
Lymphoepithelioma-Like Carcinoma
Rhabdoid Carcinoma
Primary Mammary-Like Carcinoma
Large Cell Carcinoma
Immunohistochemical Antibodies, Biomarkers, and Molecular Biology in Non-Small Cell Carcinoma
Sarcomatoid Carcinoma, Pleomorphic Carcinoma, and Giant Cell Carcinoma
Summary
References
11: Neuroendocrine Neoplasms of the Lung
Introduction
Pulmonary Neuroendocrine System
Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia
Epidemiology and Demographics
Clinical Presentation
Diagnostic Evaluation
Treatment and Prognosis
Pulmonary Carcinoids (Typical and Atypical)
Epidemiology and Demographics
Clinical Presentation
Diagnosis
Large Cell Neuroendocrine Carcinoma
Epidemiology
Clinical Presentation
Prognosis
Diagnostic Imaging
Diffuse Idiopathic Neuroendocrine Cell Hyperplasia
Low-Grade (Typical) Carcinoid and Intermediate-Grade (Atypical) Carcinoid
High-Grade Large Cell Neuroendocrine Carcinoma
Small Cell Carcinoma
Epidemiology and Demographics
Clinical Presentation
Diagnostic Evaluation
Staging Systems
Prognosis and Prevention
High-Grade Small Cell Carcinoma
Intrapulmonary Paraganglioma
Pathological Features
Classifications, Nomenclature, Definitions, and Staging
Histological Grading
Macroscopic Features
Histological Features
Low- and Intermediate-Grade Carcinomas
Immunohistochemical and Molecular Features
Large Cell Neuroendocrine Carcinoma
Small Cell Carcinoma
Intrapulmonary Paraganglioma
Pathological Features
References
12: Salivary Gland-Type Tumors of the Lung
Introduction
Mucoepidermoid Carcinoma
Diagnostic Imaging
Pathological Features
Adenoid Cystic Carcinoma
Diagnostic Imaging
Pathological Features
Immunohistochemical and Molecular Features
Pleomorphic Adenoma (Mixed Tumor)
Diagnostic Imaging
Pathological Features
Immunohistochemical Features
Epithelial–Myoepithelial Carcinoma
Diagnostic Imaging
Pathological Features
Acinic Cell Carcinoma
Diagnostic Imaging
Pathological Features
Hyalinizing Clear Cell Carcinoma
Diagnostic Imaging
Pathological Features
Oncocytoma
Diagnostic Imaging
Pathological Features
Mucous Gland Adenoma
Diagnostic Imaging
Pathological Features
Summary
References
13: Biphasic Neoplasms of the Lung
Pulmonary Biphasic Neoplasms
Pulmonary Blastoma
Clinical Features
Diagnostic Imaging
Pathologic Features
Carcinosarcoma
Clinical Features
Diagnostic Imaging
Pathological Features
Summary
References
14: Benign and Malignant Mesenchymal Tumors of the Lung
Benign Mesenchymal Tumors of the Lung
Cartilaginous Hamartoma
Leiomyoma
Schwannoma
Endobronchial Lipoma
Solitary Fibrous Tumor (SFT)
Malignant Mesenchymal Tumors of the Lung
Smooth Muscle Tumors
Skeletal Muscle Tumors
Synovial Sarcoma
Sarcomas of Bone and Cartilage
Pulmonary Artery Sarcoma
Primary Pulmonary Myxoid Sarcoma
Malignant Vascular Neoplasms
Kaposi’s Sarcoma (KS)
Epithelioid Hemangioendothelioma (EHE)
Angiosarcoma
Summary
References
15: Pulmonary Lymphoproliferative Disorders
Benign Lymphoproliferative Lesions of the Lung
Introduction
Pulmonary Nodular Lymphoid Hyperplasia (PNLH)
Introduction
Clinical Features
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Follicular Bronchiolitis
Introduction
Clinical Features
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Lymphoid Interstitial Pneumonia (LIP)/Diffuse Lymphoid Hyperplasia
Introduction
Clinical Features
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
IgG4-Related Lung Disease (IgG4-RLD)
Introduction
Clinical Features
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Malignant Lymphoproliferative Disorders and Other Hematopoietic Disorders of the Lung
Introduction
Pulmonary Non-Hodgkin Lymphomas of B-Cell Origin
Pulmonary Marginal Zone Lymphoma of the Mucosa-Associated Lymphoid Tissue (Pulmonary MALT Lymphoma)
Introduction
Clinical Features
Diagnostic Imaging
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Pulmonary Diffuse Large B-Cell Lymphoma (DLBCL)
Introduction
Clinical Features
Diagnostic Imaging
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Pulmonary Intravascular Large B-Cell Lymphoma (LBCL)
Introduction
Clinical Features
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Lymphomatoid Granulomatosis (LYG)
Introduction
Clinical Features
Diagnostic Imaging
Pathology
Immunohistochemistry and Other Ancillary Studies
Grading System
Differential Diagnosis
Molecular Findings
Other Small B-cell Lymphomas: Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL), Mantle Zone Lymphoma, Follicular Lymphoma and Lymphoplasmacytic Lymphoma
Introduction
Clinical Features
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Extraosseous (Pulmonary) Plasmacytoma
Introduction
Clinical Features
Diagnostic Imaging
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Pulmonary Non-Hodgkin Lymphomas of T-Cell Origin
Pulmonary Anaplastic Large Cell Lymphoma (ALCL)
Introduction
Clinical Features
Diagnostic Imaging
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Pulmonary Peripheral T-Cell Lymphoma, Not Otherwise Specified (PTCL, NOS)
Introduction
Clinical Features
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Pulmonary Classic Hodgkin Lymphoma (CHL)
Introduction
Clinical Features
Diagnostic Imaging
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Myeloid Neoplasms: Pulmonary Acute Myeloid Leukemia/Myeloid Sarcoma
Introduction
Clinical Features
Diagnostic Imaging
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Histiocytic Disorders of the Lung
Pulmonary Langerhans Cell Histiocytosis (LCH)
Introduction
Clinical Features
Diagnostic Imaging
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Alterations
Extranodal Pulmonary Rosai–Dorfman Disease (RDD)
Introduction
Clinical Features
Diagnostic Imaging
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Pulmonary Erdheim–Chester Disease (ECD)
Introduction
Clinical Features
Diagnostic Imaging
Pathology
Immunohistochemistry and Other Ancillary Studies
Differential Diagnosis
Molecular Findings
Pulmonary Juvenile Xanthogranuloma (PJXG)
Introduction
Clinical Features
Pathology
Immunohistochemistry
Differential Diagnosis
Pulmonary Crystal-Storing Histiocytosis
Introduction
Pathology
Immunohistochemistry
References
16: Tumors of Uncertain Histogenesis
Clear Cell “Sugar” Tumor
Pathological Features
Inflammatory Pseudotumor (Myofibroblastic Tumor)
Pathological Features
Pneumocytoma (Sclerosing Hemangioma)
Pathological Features
Granular Cell Tumor
Pathological Features
Hemangioblastoma-like Clear Cell Stromal Tumor
Pathological Features
Summary
References
17: Uncommon Tumors of the Lung
Glomangioma/Glomangiosarcoma
Clinical Features
Diagnostic Imaging
Pathological Features
Bronchial (Pulmonary) Melanoma
Clinical Features
Diagnostic Imaging
Pathological Features
Pulmonary Meningioma
Clinical Features
Pathological Features
Pulmonary Meningotheliomatosis
Diagnostic Imaging
Pathological Features
Intrapulmonary Thymoma
Clinical Features
Diagnostic Imaging
Pathological Features
Ganglioneuroblastoma
Pathological Features
Pulmonary Adenofibroma
Clinical Features
Pathological Features
Alveolar Adenoma
Pathological Features
Angiolymphoid Hyperplasia with Eosinophilia
Clinical Features
Pathological Features
Amyloid Tumor (Amyloidoma)
Clinical Features
Pathological Features
Placental Transmogrification
Clinical Features
Diagnostic Imaging
Pathological Features
Summary
References
Part IV: Lung: Non-neoplastic Conditions
18: Interstitial Lung Diseases
Idiopathic Pulmonary Fibrosis
Introduction
Epidemiology
Pathogenesis
Risk Factors
Cigarette Smoking and Environmental Risk Factors
Microbial Agents
Gastroesophageal Reflux
Genetic Risk Factors
Clinical Presentation and Natural History
Diagnostic Evaluation
Laboratory Testing
Pulmonary Function Tests
Chest Radiograph
Bronchoalveolar Lavage
Transbronchial Lung Biopsy
Transbronchial Lung Cryobiopsy
Surgical Lung Biopsy
Diagnosis
Multidisciplinary Discussion
Desquamative Interstitial Pneumonia
Introduction
Epidemiology
Etiologic Risk Factors
Cigarette Smoking
Occupational and Environmental Exposures
Systemic Diseases and Medications
Clinical Presentation
Diagnostic Evaluation
Laboratory Testing
Pulmonary Function Test
Imaging
Chest Radiographs
CT and HRCT Findings
Evolution of Findings
Differential Diagnosis
Bronchoalveolar Lavage
Transbronchial Biopsy, Transbronchial Lung Cryobiopsy, and Surgical Lung Biopsy
Histopathology
Nonspecific Interstitial Pneumonia
Introduction
Clinical Presentations and Prognosis
Diagnostic Evaluation
Laboratory Testing
Pulmonary Function Tests
Bronchoalveolar Lavage
Causes and Clinical Entities
Multidisciplinary Discussion and Decision to Biopsy
Imaging of Nonspecific Interstitial Pneumonia
Evolution of Imaging in NSIP
Exacerbation Versus Infection
Differential Diagnosis
Histopathology
Usual Interstitial Pneumonia
Introduction
Clinical Presentations
Causes and Clinical Entities
Diagnostic Evaluation
Laboratory Testing
Pulmonary Function Tests
Bronchoalveolar Lavage
Multidisciplinary Discussion and Decision to Biopsy
Imaging of Usual Interstitial Pneumonia (UIP)
Chest Radiography
HRCT
UIP Pattern
Probable UIP
Indeterminate and Inconsistent UIP Patterns
Nonidiopathic UIP Pattern
Connective Tissue Disorders
Differential Diagnosis
Histopathologic Pattern
Hypersensitivity Pneumonitis (Extrinsic Allergic Alveolitis)
Introduction
Epidemiology
Pathogenesis
Antigens and Physiologic Susceptibility
Immune Dysregulation
Cigarette Smoking
Clinical Presentation
Diagnostic Evaluation
Exposure Questionnaire
Laboratory Testing
Pulmonary Function Test
Bronchoalveolar Lavage
Transbronchial Biopsy and Transbronchial Lung Cryobiopsy
Surgical Lung Biopsy
Imaging of Hypersensitivity Pneumonitis
Chest Radiograph
Chest CT
Acute /Subacute HP
Chronic HP
Differential Diagnosis
Histopathology
Pulmonary Lymphangioleiomyomatosis
Introduction
Epidemiology
Pathogenesis
Clinical Manifestations and Diagnosis
Differential Diagnosis
Imaging of Lymphangiomyomatosis (LAM)
Chest Radiograph
Chest CT
Abdominal Imaging
Diagnostic Evaluation
Laboratory Tests
Pulmonary Function Tests
Lung Biopsy
Histopathology
Prognosis
Treatment
Other Considerations About LAM
References
19: Connective Tissue Disease-Associated Interstitial Lung Disease
Rheumatoid Arthritis
Introduction
Interstitial Lung Disease
Airway Disease
Rheumatoid Nodules
Pleural Disease
Caplan Syndrome
Pulmonary Hypertension
Pulmonary Infections
Drug-Induced Lung Disease
Other
Systemic Lupus Erythematosus
Introduction
Pleural Disease
Parenchymal Disease
Interstitial Lung Disease
Acute Lupus Pneumonitis
Vascular Disease
Diffuse Alveolar Hemorrhage
Pulmonary Hypertension
Pulmonary Embolism
Diaphragmatic Involvement
Shrinking Lung Syndrome
Other Pulmonary Involvement
Sjogren Syndrome
Introduction
Airway Disease
Interstitial Lung Disease
Pulmonary Amyloidosis
Pulmonary Lymphoma and Other Hematological Involvement
Pulmonary Hypertension
Other Respiratory Manifestations
Systemic Sclerosis
Introduction
Etiology
Diagnosis
Disease Presentation
Evaluation
Interstitial Lung Disease
Pulmonary Hypertension
Other Manifestations
Dermatomyositis and Polymyositis
Interstitial Lung Disease
Pulmonary Hypertension
Spontaneous Pneumomediastinum
Malignancy
Other
Ankylosing Spondylitis
Modified New York criteria [285] Assessment of SpondyloArthritis International Society (ASAS) Criteria [286] Pulmonary Apical Fibrocystic Disease
Obstructive Sleep Apnea
Other
Relapsing Polychondritis
Introduction
Airway Disease
Myelodysplastic Syndrome
Other
Mixed Connective Tissue Disease
Interstitial Lung Disease
Pulmonary Arterial Hypertension
Other
References
20: Pneumoconiosis
Asbestos-Related Lung Diseases: A Review
Introduction
Types of Asbestos and Occupations Associated with Exposure
Pathogenesis of Asbestos-Related Lung Conditions
Fate of Asbestos Fibers in Lungs
Asbestos-Related Pleuro-pulmonary Conditions
Benign Conditions
Pleural Diseases
Pleural Plaque
Benign Asbestos Pleural Effusion
Rounded Atelectasis
Diffuse Pleural Thickening
Parenchymal Conditions
Asbestosis
Malignant Conditions
Pleural Diseases
Malignant Pleural Mesothelioma
Parenchymal Diseases
Lung Cancer
Silicosis
Introduction
Epidemiology
Occupational Risk for Silicosis and Risk Mitigation
Pathogenesis
Clinico-Pathologic Types of Silicosis
Clinical Features
Pulmonary Function Tests (PFT)
Radiographic Features
Diagnostic Approach
Management
Hard Metal Lung Disease
Introduction
Pathogenesis
Clinical Features
Radiology
Diagnosis
Pulmonary Function Test
Laboratory Tests
Bronchoscopy
Pathology
Management
References
21: Pulmonary Vasculitides
Part I: Pulmonary Disease in Primary Vasculitides
Introduction
Takayasu Arteritis
Introduction
Incidence and Risk Factors
Pathogenesis
Clinical Presentation
Pulmonary Disease
Lung Parenchyma
Vasculature
Diagnostic Evaluation
Laboratory Data
Imaging
Histopathological Features
Management
Prognosis and Conclusion
Giant Cell Arteritis
Introduction
Incidence and Risk factors
Pathogenesis
Clinical Presentation
Pulmonary Disease
Upper Respiratory Tract
Lower Respiratory Tract and Lung Parenchymal
Pleura
Vasculature
Diagnostic Evaluation
Laboratory Data
Imaging
Management
Glucocorticoids
Methotrexate
Tocilizumab
Other DMARDs
Prognosis and Conclusion
Behcet’s Disease
Introduction
Incidence and Risk Factors
Pathogenesis
Clinical Presentation
Pulmonary Disease
Vasculature
Lung Parenchyma
Diagnostic Evaluation
Laboratory Data
Imaging
Management
Pharmacologic Treatment
Surgical Treatment
Prognosis and Conclusion
Polyarteritis Nodosa
Introduction
Incidence and Risk factors
Pathogenesis
Clinical Presentation
Pulmonary Disease
Lung Parenchyma
Vasculature
Diagnostic Evaluation
Laboratory Data
Imaging
Management
Prognosis and Conclusion
Necrotizing Sarcoid Granulomatosis
Introduction
Incidence and Risk Factors
Pathogenesis
Clinical Presentation
Pulmonary Disease
Lung Parenchyma
Diagnostic Evaluation
Laboratory Data
Imaging
Management
Prognosis and Conclusion
Kawasaki Disease
Introduction
Incidence and Risk Factors
Pathogenesis
Clinical Presentation
Pulmonary Disease
Lung Parenchyma and Pleural Disease
Diagnostic Evaluation
Laboratory Data
Imaging
Management
Prognosis and Conclusion
Isolated Pauci-immune Pulmonary Capillaritis
Introduction
Incidence and Risk Factors
Pathogenesis
Clinical Presentation
Diagnostic Evaluation
Laboratory Data
Bronchoscopy and Lung Biopsy
Imaging
Management
Prognosis and Conclusion
Part II: Granulomatosis with Polyangiitis
Introduction
Incidence and Risk Factors
Pathogenesis
Clinical Presentation
Pulmonary Disease
Airway Disease
Diagnostic Evaluation
Diagnostic Imaging
Chest Radiography
CT and HRCT
Nodules and Masses
Ground Glass Opacities and Consolidation
Bands and Linear Opacities
Additional Findings
Evolution Over Time
Tracheobronchial Abnormalities
Role of PET and 18F-FDG PET/CT
Cardiac Involvement in GPA
Differential Diagnosis
Pathological Features
Management
Induction
Maintenance
Plasma Exchange
Other Considerations
Prognosis and Conclusions
Eosinophilic Granulomatosis with Polyangiitis
Introduction
Incidence and Risk Factors
Pathogenesis
Diagnostics
Laboratory Data
Diagnostic Imaging
Radiography
CT
Clinical Presentation
Pulmonary Disease
Vasculature
Management
Microscopic Polyangiitis
Introduction
Incidence and Risk factors
Pathogenesis
Clinical Presentation
Pulmonary Disease
Diagnostic Evaluation
Laboratory Data
Imaging
Management
Prognosis and Conclusions
Antiglomerular Basement Membrane Disease
Introduction
Incidence and Risk Factors
Pathogenesis
Clinical Presentation
Diagnostic Evaluation
Laboratory Data
Pulmonary Disease
Management
Prognosis and Conclusion
Pulmonary Hemosiderosis
Introduction
Incidence and Risk Factors
Pathogenesis
Clinical Presentation
Diagnostic Evaluation
Laboratory Data
Bronchoscopy
Diagnostic Imaging
Chest Radiograph and Computed Tomography of IPH
Other Imaging Modalities
Differential Diagnosis
Histopathological Features
Management
Prognosis and Conclusion
References
22: Emphysema and Cystic Lung Disease
Introduction
Emphysema
Epidemiology
Pathogenesis
Risk Factors
Clinical Features
Diagnosis
Treatment
Pharmacotherapy
Alpha 1-Antitrypsin Deficiency
Epidemiology
Pathophysiology
Risk Factors
Clinical Presentation and Diagnosis
Treatment
Birt-Hogg-Dube Syndrome
Epidemiology
Pathogenesis
Clinical Presentation
Diagnosis
Lymphoid Interstitial Pneumonia
Pathogenesis
Clinical Features
Diagnosis
Treatment
Neurofibromatosis-Related Lung Disease
Epidemiology
Risk Factors
Pathophysiology
Clinical Presentation
Diagnosis
Treatment
Ehlers-Danlos Syndrome
Epidemiology
Pathophysiology
Clinical Features
Diagnosis
Treatment
Marfans Syndrome
Epidemiology
Pathogenesis
Clinical Characteristics
Diagnosis
Treatment and Management
Bronchogenic Cysts
Epidemiology
Clinical Features
Diagnosis
Treatment
Cystic Fibrosis
Epidemiology
Pathophysiology
Clinical Features
Diagnosis
Treatment
Cystic Adenomatoid Malformations
Epidemiology
Pathophysiology
Clinical Features
Diagnosis
Pathological Features
Treatment
Light Chain Deposition Disease
Epidemiology
Pathophysiology
Clinical Features
Diagnosis
Treatment
References
23: Airspace Diseases and Pulmonary Nodules
Cryptogenic Organizing Pneumonia
Introduction
Clinical Presentation
Diagnostic Imaging
Chest Radiograph
CT and HRCT
Multifocal Alveolar Disease
Nodular Form
Mixed Ground Glass and Solid Lesions
Perilobular Pattern
Linear and Band Opacities
Fibrotic OP
Other Findings
Evolution of the Imaging Findings
Differential Diagnosis
Pathological Features
Differential Diagnosis
Eosinophilic Lung Disorders
Introduction
Etiology
Clinical Features
Chronic Eosinophilic Pneumonia
Clinical Findings
Imaging of Acute Eosinophilic Pneumonia (AEP)
Chest Radiograph
CT
Chronic Eosinophilic Pneumonia (CEP)
Chest Radiograph
CT
Differential Diagnosis
Histopathological Features
Eosinophilic Pneumonias of Determined Origin
Eosinophilic Pneumonia of Parasitic Origin
Tropical Eosinophilia
Ascaris Pneumonia
Strongyloides stercoralis Infection
Allergic Bronchopulmonary Aspergillosis (ABPA)
Clinical Presentation
Lipoid Pneumonia (Paraffinoma)
Pulmonary Sarcoidosis
Introduction
Clinical Features
Staging and Prognosis
Diagnostic Imaging
Chest Radiography
Lymph Node Enlargement
Parenchymal Lung Disease
Airway Involvement
CT and HRCT
Reticulonodular Pattern
Macronodules and Mass-Like Consolidation
Fibrotic Changes
Pleural Involvement
Lymphadenopathy
Large and Small Airway Involvement
Pulmonary Hypertension
Nuclear Medicine
Magnetic Resonance Imaging
Cardiac Sarcoidosis (CS)
Echocardiography
CT
Cardiac Magnetic Resonance (CMR)
Nuclear Medicine
Differential Diagnosis
Cardiac Sarcoid
Summary
Diagnostic Approach
Prognosis Factors
Major Adverse Effects of Advanced (End-stage) Lung Sarcoidosis
Pulmonary Hypertension
Chronic Pulmonary Aspergillosis
Monitoring and Decision of Treatment
Histopathological Features
References
24: Infectious Diseases That May Mimic Lung Cancer
Introduction
Bacteria
Actinomycetes
Non-Tuberculosis Mycobacteria (NTM)
Mycobacteria Tuberculosis (MTB)
Endemic Mycosis (Fungal)
Blastomycosis
Coccidioidomycosis
Cryptococcosis
Histoplasmosis
Paracoccidioidomycosis (PCM)
Invasive Fungi
Aspergillosis
Zygomycosis (Mucormycosis)
Parasites
Dirofilariasis Immitis
Echinococcus
Paragonimiasis
References
25: Miscellaneous Conditions
Hermansky-Pudlak Syndrome (HPS)
Niemann-Pick (NP) and Gaucher Diseases
Dyskeratosis Congenita (DC)
Pulmonary Alveolar Microlithiasis (PAM)
Diagnostic Imaging
Pathological Features
Pulmonary Alveolar Proteinosis (Pulmonary Alveolar Lipoproteinosis)
Clinical Presentation
Diagnostic Imaging
Chest Radiograph
CT
Primary Versus Secondary form of PAP
Evolution of Imaging Findings
Differential Diagnosis
Pathological Features
Pulmonary Hyalinizing Granuloma (PHG)/IgG4-Sclerosing Disease
Placental Transmogrification
References
26: Iatrogenic Conditions
Iatrogenic Conditions: Pulmonary Drug Toxicity
Chest Radiograph
CT and HRCT
Imaging Patterns of Drug-Induced Pulmonary Reaction
Pulmonary Edema and Diffuse Alveolar Damage (DAD) Spectrum
Organizing Pneumonia (OP): Formerly “Bronchiolitis Obliterans- Organizing Pneumonia (BOOP)”
Interstitial Lung Disease with or Without Fibrosis
NSIP and UIP
Eosinophilic Pneumonia (EP)
Hypersensitivity Pneumonitis (HP)
Diffuse Alveolar Hemorrhage (DAH)
Drug-Induced Sarcoid-like Reaction (DSIR)
Pulmonary Hypertension
Drug-Related Pleural Abnormalities (DRPA)
Imaging Following Targeted Therapy and Immunotherapy
Targeted Therapy
Angiogenesis Inhibitors
Imaging of Tumor Response with Targeted Therapy
Imaging of Targeted Therapy Toxicity
Immunotherapy
Response Assessment
Immune-Related Adverse Events
Imaging of Immune-Related Adverse Events (irAE)
Pathological Response to Therapy
Imaging Patterns Following Radiation Therapy
Pathological Features of Radiation Pneumonitis
References
27: Pathology of the Lung Allograft
Introduction
Primary Graft Dysfunction
Anastomotic Complication
Hyperacute Rejection
Acute Rejection
Acute Cellular Rejection
Small Airway Inflammation/Lymphocytic Bronchiolitis
Antibody-Mediated Rejection
Chronic Lung Allograft Dysfunction (CLAD)
Bronchiolitis Obliterans Syndrome (BOS)/Obliterative Bronchiolitis (OB)
Restrictive Allograft Syndrome (RAS)/Pleuroparenchymal Fibroelastosis (PPFE)
Chronic Vascular Rejection
Infection in Lung Allograft
Bacterial Infection
Viral Infection
Fungal Infection
Posttransplant Lymphoproliferative Disorder (PTLD)
Recurrence of Native Disease
References
Index