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Neuroendocrine neoplasms of the lung

Introduction and general considerations Neuroendocrine tumors (NETs) are grouped together based on common “neuroendocrine” morphological features and the finding of intracellular ultrastructural neurosecretory granules whose presence is detected today mainly by immunohistochemistry (IHC). There has been movement toward uniformity of nomenclature within these tumors throughout the body, especially within the gastrointestinal tract and pancreas. This has resulted in grading schema that incorporate low-grade, intermediate-grade, and high-grade…

Pathology of lung transplantation

Introduction Lung transplantation may offer a longer survival and improved quality of life to patients with end-stage lung disease. Common indications for lung transplantation are listed in Table 13.1 . Bilateral lung transplantation is the norm for cystic fibrosis and is in general the most common form of lung transplantation. Indeed, in 2017, 81% of all lung transplantations were bilateral. Other transplantations include single lung, heart-lung,…

Pulmonary hypertension

Introduction Pathologists may encounter pulmonary hypertension in biopsies, explanted lungs, or autopsies. Probably the most common setting is biopsies performed for the diagnosis of interstitial lung disease; in this situation recognition of pulmonary hypertension (and equally important, mimics of pulmonary hypertension), is important because the presence of pulmonary hypertension is an adverse prognostic factor in all types of interstitial lung disease and may affect treatment. In…

Pulmonary vasculitis and pulmonary hemorrhage

Pulmonary vasculitis Overview of pulmonary vasculitis Inflammation of arteries and veins can occur in many inflammatory lung diseases, including infections. By convention, the diagnostic term pulmonary vasculitis is restricted to a relatively limited number of diseases in which vascular inflammation is thought to be a major component of the pathologic process. Most pulmonary vasculitides are believed to be immune-mediated diseases, although their etiology and pathogenesis continue…

Pneumoconioses

Overview and general considerations Pneumoconiosis literally means “dust in the lung” and the term has come to refer to disease of the lung related to the inhalation of dusts. Pneumoconioses are for the most part due to the inhalation of inorganic dusts in the workplace. The reaction of the lungs to these dusts is generally fibrotic. Pneumoconioses typically evolve over several decades, although there are some…

Nonneoplastic pathology of the large and small airways

Three main histologic patterns of injury are encountered in the setting of airways disease; minimal change, nodules, and fibrosis. Minimal change is the classic pattern encountered in the setting of small airways disease or constrictive bronchiolitis from any etiology. In this setting it is important to examine the bronchovascular bundle for subtle histologic evidence of injury, including loss of associated airways. Nodules, especially in a bronchovascular…

Chronic diffuse lung diseases

Diffuse or interstitial lung diseases (ILDs) include a spectrum of primarily nonneoplastic inflammatory conditions that share the common property of multilobar (but not necessarily complete) involvement of the lung parenchyma. The term ILD has become so thoroughly entrenched across multiple medical disciplines that it seems practical to continue its usage, although we would emphasize that many of the diseases discussed in this chapter also involve the…

Lung infections

Histologic patterns of lung infection Infectious pathology can result in a variety of basic patterns of pulmonary pathology including acute lung injury, alveolar filling, cellular infiltrates, nodules, and fibrosis in order of decreasing frequency ( Table 7.1 ). Acute lung injury is the most common basic pattern encountered in infection and may be secondary to all forms of infection (bacterial, viral, fungal, protozoan, parasitic). Alveolar filling,…

Acute lung injury

Practical approach to the differential diagnosis of acute lung injury for pathologists The basic elements of the acute lung injury pattern include interstitial edema, alveolar edema, fibrin, hyaline membranes, reactive pneumocytes, and organization (see Box 6.2 and Fig. 6.1 A–C). These histologic elements of acute lung injury occur in various combinations and result in a broad spectrum of histopathology. Very early examples may appear nearly normal,…

Developmental and pediatric lung disease

The diagnostic approach to pediatric lung biopsy differs somewhat from that in the adult patient. Many of the usual questions that arise in adult pulmonary pathology are replaced by separate issues involving abnormal development, altered lung growth due to prematurity, genetic disease, and infections secondary to an immature immune system. Moreover, the spectrum of diseases observed in the pediatric lung biopsy differs from its adult counterpart,…

Computed tomography of diffuse lung diseases and solitary pulmonary nodules

Foundations High-resolution computed tomography Computed tomography (CT) is the widely recognized imaging standard of reference for the assessment of most pulmonary abnormalities ( Fig. 4.1 ). With a diffuse lung disease (DLD), the high-resolution option is used. An actual collimation of 0.5 to 2 mm and an edge-enhancing algorithm for high spatial frequency reconstruction serve to generate the final images. , The narrow collimation reduces the voxel…

Optimal processing of diagnostic lung specimens

Optimal specimen handling is essential for the accurate interpretation of biopsies and cytologic preparations obtained in the course of evaluating the patient with lung disease. The limited number of sampling techniques available can be divided into three general categories: bronchoscopy, transthoracic needle core biopsy or aspiration, and surgical wedge biopsy of peripheral lung through a transthoracic approach. , The focus of this chapter is on these…

Pulmonary function testing for pathologists

As the name implies, pulmonary function testing provides quantification of pulmonary physiologic function. By definition this information is disease independent and static, representing a point-in-time evaluation. Nonetheless, this information can be very helpful for building a differential diagnosis. In fact, many pulmonary diseases are clinical syndromes and without either a definitive molecular marker for disease or without unique defining functional features. These diseases therefore often require…

Lung anatomy

Development and gross anatomy Airway development During early embryogenesis (at approximately day 21 after fertilization), the lungs begin as a groove in the ventral floor of the foregut ( Fig. 1.1 ). This foregut depression becomes a diverticulum of endoderm, surrounded by an amorphous condensation of splanchnic mesoderm that lengthens caudally in the midline, anterior to the esophagus. By the fourth week of gestation, two lung…

Joint-Associated Lesions

Synovial-based lesions encompass both reactive and neoplastic processes. Although any joint may be affected, the most common site of disease is the knee. Pathologic changes in the small joints of the fingers are typically due to rheumatoid disease or localized tenosynovial giant cell tumor, whereas changes in the ankles and wrists may be due to intra-articular diffuse tenosynovial giant cell tumor. Malignant lesions of the joints…

Crystal Arthropathies

The term crystalline arthropathy is a general term that refers to the presence of crystalline deposits in or around joints, sometimes within tendon sheaths and bursae as well. Essentially three crystalline substances may be found in articular and periarticular tissues. These three are monosodium urate (MSU), the cause of gout and gouty arthritis; calcium pyrophosphate dihydrate (CPPD), a finding in many degenerate joints and the cause…

Degenerative Joint Disease

Osteoarthritis Definition and Synonyms Bone morphogenetic protein (BMP): a member of a superfamily of proteins that promotes the formation of bone and the skeleton Collagen IX: a fibrous protein that is a heterodimeric molecule expressed mainly in cartilage Collagen X: the fibrous protein constituent of hypertrophic and mineralizing cartilage Endochondral ossification: the process of bone development from cartilage seen during skeletal development of long bones and…

Notochordal and Related Lesions

The notochord is a rod-shaped structure that forms during the third week of gestation and organizes formation of the axial skeleton. As vertebral bodies undergo ossification, the notochord becomes fragmented, and by the second month of development, notochordal tissue is typically only present in the intervertebral space, forming the nucleus pulposus of the intervertebral disk. Because of this process of notochordal degeneration, notochordal tissue is not…

Metastases to Bone

Overview Metastases to bone of tumors from nonosseous primary sites account for the largest percentage of bone tumors. The host bone responds either by osseous resorption (osteolytic), osseous formation (osteoblastic), or often both processes. This chapter will cover the clinical and radiologic features as well as the pathologic classification of metastases to bone in adults and children. Pathophysiology (Microenvironment) Metastases to bone occur either by direct…

“Soft Tissue” Tumors of Bone, Avascular Necrosis and Bone Infarct, and Paget Disease

Benign “Soft Tissue” Tumors Primary in Bone Schwannoma Definition and Synonyms Schwannoma, also known as neurilemmoma and neurinoma, is a benign nerve sheath tumor that occurs primarily in soft tissue but can also occur primarily within bone or secondarily involve bone. Incidence and Demographics Intraosseous schwannomas are rare, with a reported incidence of less than 1%. They typically affect young adults in the second to fifth…