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Vascular Diseases

Introduction Pulmonary vascular diseases encompass a wide range of disorders of the lung. Abnormalities of the pulmonary vasculature not only cause lung disease, but can also result from secondary involvement by nonvascular pulmonary diseases. Many of the primary vascular diseases of the lung are severe, have no adequate available therapy, and pose dilemmas to both the clinician and pathologist. Diseases of the pulmonary vasculature can affect…

Lung Neoplasms in Infants and Children

Introduction Primary lung neoplasms in children are rare, occurring much less frequently than congenital malformations, inflammatory masses, and metastatic tumors. In one published review of pulmonary masses at a large pediatric hospital, the ratio of primary neoplasms to metastatic lesions to nonneoplastic lesions was 1:5:60. In most pediatric series, primary malignant pulmonary neoplasms outnumber benign neoplasms. However, numbers vary depending on patient population and definition of…

Acquired and Idiopathic Disorders in Neonates and Young Children

Introduction Disorders presenting in neonates and young children comprise a heterogeneous group of conditions that can be divided into two broad categories, entities with a known etiology and those of unknown or poorly understood etiology. The well-characterized disorders include those that reflect altered lung growth and development or a poor transition from intrauterine to extrauterine life, including pulmonary hypoplasia, hyaline membrane disease (HMD), chronic lung disease…

Congenital, Developmental, and Inherited Disorders

Congenital and developmental disorders of the lung manifest almost exclusively in pediatric and neonatal patients and include a wide array of disorders resulting from abnormalities of lung architecture, cellular function, and metabolism. All of these disorders are uncommon, and most are rarely encountered in clinical practice; however, together, they account for significant morbidity and mortality in the pediatric population and can often be a diagnostic challenge…

A Pattern-Based Approach to Diagnosis

Introduction Each of us follows well-established patterns as we go about our daily lives, and in turn we interact with our world through the recognition of patterns: in the people we know, the places we live, and the structures that form our physical environment. Similarly, the specialty of anatomic pathology relies heavily on pattern recognition, especially given the observational nature of this medical discipline. The experienced…

The Uses and Abuses of the Lung Biopsy

Introduction The lung biopsy is widely recognized as a valuable tool for the diagnosis and management of diverse pulmonary disorders. The various procedures currently in use, such as the open lung biopsy (OLBx), video-assisted thorascopic surgery (VATS) biopsy, and transbronchial lung biopsy (TBBx) and cryobiopsy (TBLC), can be diagnostic when performed under appropriate circumstances and if evaluated by the pathologist with several caveats in mind. Although…

Normal Anatomy, Tissue Artifacts, and Incidental Structures

Normal Anatomy The lungs occupy most of the volume of the thoracic cavity. The average weights of male and female lungs are approximately 850 grams and 750 grams, respectively. The right lung is composed of ten distinct segments comprising three lobes (upper, middle, and lower), and the left lung has ten segments organized into two lobes (upper and lower). Each lobe is covered with pleura (visceral…

Biopsy Pathology of Neurodegenerative Disorders in Adults

Neurodegenerative disorders are broad ranging and highly complex, with diverse etiologies and frequently overlapping clinical manifestations. The neuropathologic diagnosis of these diseases can be exceedingly challenging even at autopsy when the amount of tissue for examination is not a limiting factor. Many neurodegenerative diseases have a predilection for specific anatomic regions, and the manner of progression can be either stereotypic or unusual. In contrast to most…

Vascular and Ischemic Disorders

Introduction Reproducible patterns of cerebrovascular injury result from two basic pathologic processes that are occasionally encountered in surgical practice: (1) cerebral infarct resulting from blockage or stenosis of vessels that deprives the brain of oxygen and nutrients and (2) hemorrhage from diseased vessels that may then secondarily cause tissue destruction and hypoxia. A third major pattern results from global hypoxic/ischemic damage, such as occurs with cardiac…

Pathology of Epilepsy

Introduction Epilepsy is a common clinical manifestation of a variety of underlying neuropathologic conditions. Most patients can be adequately managed with pharmacotherapy; approximately 75% of patients diagnosed with epilepsy may be ultimately seizure free on medications alone. However, approximately 5% to 10% of patients will develop pharmacoresistant or medically intractable epilepsy, which may require surgical intervention. The focus of this chapter is on specific pathologic processes…

White Matter and Myelin Disorders

Introduction This chapter focuses on the surgical neuropathology of demyelinating diseases (i.e., those diseases in which myelin itself or the oligodendrocyte represents the targeted element with relative axonal sparing), as well as diseases in which white matter is preferentially targeted by processes that are more destructive than they are demyelinating. The process of segmental demyelination of individual axons results in myelin loss with preservation of axonal…

Infections and Inflammatory Disorders

Recognizing a pattern of disease is critical in surgical and autopsy neuropathology, especially for infectious diseases. However, few other central nervous system (CNS) diseases rival infections in their requirement for a multimodal approach—combining clinical, laboratory, and pathology information—in order to arrive at a precise diagnosis. Clinical information that narrows the differential diagnosis for any putative CNS infection includes patient age, geographic distribution, tempo of disease onset…

Familial Tumor Syndromes

The nervous system is affected in many familial tumor or “cancer predisposition” syndromes. The genetic and clinicopathologic features are summarized in Table 22.1 . Autosomal dominant inheritance is most common, and the majority of these disorders result from inactivation of known tumor suppressor genes. Often, the disease-causing gene is inherited from the sperm or egg of one parent, although new germline mutations are also common. This…

Therapy-Associated Neuropathology

Introduction Although pathologists typically focus their attention nearly exclusively on the neuropathologic changes of natural disease, therapy-associated neuropathology is increasingly playing a role, particularly now that post-therapy biopsies have become common. To avoid misdiagnosing a host of primary pathologic changes, such as tumor necrosis, vasculopathies, demyelination, cerebral infarction, inflammatory conditions, vasculitis, and others, it is critical to recognize iatrogenic changes induced by prior surgery, intravascular embolization…

Histologic Features of Pituitary Adenomas and Sellar Region Masses

Introduction Most pituitary region masses are pituitary adenomas; however, when other sellar region masses are encountered by clinicians or pathologists, the differential diagnosis can be broad. This chapter will focus on updates for pituitary adenomas, as well as discuss other sellar region entities that may mimic clinically nonfunctioning pituitary adenomas—including non-neuroendocrine tumors (pituicytoma, granular cell tumor of the neurohypophysis, and spindle cell oncocytoma), cystic lesions/tumors (Rathke…

Melanocytic Neoplasms of the Central Nervous System

Definitions and Synonyms Melanocytic neoplasms of the central nervous system (CNS) arise from the population of leptomeningeal melanocytes that are scattered throughout the arachnoid membranes. Lesions can manifest as diffuse disseminations within the subarachnoid space or as solid masses, and they range in histologic grade from benign to malignant. Meningeal melanocytoma (benign mass forming proliferation), meningeal melanoma (malignant mass forming proliferation), meningeal melanocytosis (benign diffuse meningeal…

Germ Cell Tumors

Definition and Synonyms Germ cell tumors (GCTs) are a heterogeneous group that are widely considered to be morphologic and immunophenotypic homologues of germinal neoplasms arising in the gonads and extragonadal sites. The World Health Organization (WHO) classification of GCTs of the central nervous system (CNS) is therefore similar to that of other sites: germinoma (denoted as seminoma in the testis and dysgerminoma in the ovary), teratoma…

Lymphomas and Histiocytic Tumors

Definition and Synonyms Primary central nervous system lymphomas (PCNSLs) and histiocytic tumors are neoplasms that correspond histologically to their nodal or systemic counterparts, but lack an apparent systemic manifestation at the time of initial presentation. The most common types (hereafter referred to as “typical”) include diffuse large B cell lymphoma (DLBCL) and Langerhans cell histiocytosis (LCH), respectively. The many other subtypes are considerably rarer in the…

Epithelial, Neuroendocrine, and Metastatic Lesions

Introduction Epithelial structures are scarce in the normal central nervous system (CNS). Only the choroid plexus ( Fig. 16.1A ) and the pituitary gland's anterior and intermediate lobes contain true epithelial cells. The latter are derived from Rathke pouch epithelium that migrates to its place in the sella during early development ( Fig. 16.1B ). Thus, epithelial and epithelioid lesions that involve the brain include a…