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Symptom Management in Children with Cancer

Progress in understanding the causes of pediatric cancer, as well as advances in cancer-directed therapies, hold great promise for curing and extending the lives of many children diagnosed with cancer. However, as advances in medicine and technology improve the survival of children with life-threatening illnesses, attention to health-related quality of life and progress in symptom management have not kept pace with advances in disease-directed therapies. As…

Palliative Care in Pediatric Oncology

More than 2000 children die each year in the United States of cancer-related causes, and many more are living with advanced cancer. In children with cancer, it is not always possible to determine whether the disease will be responsive to cancer-directed therapy, nor is it possible to determine which type of path the dying process will take. Some children may die suddenly and unexpectedly, such as…

Nursing Care of Patients with Childhood Cancer

History of Pediatric Oncology Nursing Nursing is the largest health care occupation in the United States, representing 2.7 million jobs—a number that is expected to grow by 26% or more before 2020, faster than the national average of 14%. Because its roots are embedded in daily life and the caretaking role women traditionally assumed throughout history, nursing was not recognized as a profession until the mid-1800s,…

Oncologic Emergencies

Expert and timely management of emergent situations is crucial in the practice of pediatric oncology. Although many emergencies are common across the spectrum of pediatric patients, such as seizure, respiratory arrest, and electrolyte abnormalities, others stem from the underlying cancer or cancer-directed therapy and are unique to this population of patients. These types of emergencies require special consideration and specific management to minimize adverse outcomes. Emergencies…

Infectious Disease in the Pediatric Cancer Patient

Introduction Infections are important issues faced by children with cancer. Invasive bacterial and fungal infections are problematic mainly in those receiving intensive treatment. Viruses may also be life-threatening in specific circumstances. Furthermore, non–life-threatening infections such as upper respiratory tract infection may still affect treatment and quality of life, particularly if febrile illnesses occur during neutropenia. In an effort to provide a comprehensive discussion of infection in…

Imaging in the Evaluation and Management of Childhood Cancer

The incidence of cancer in childhood is relatively rare when compared to the incidence in adults; approximately 6500 new cases are diagnosed in the United States each year. Nevertheless, cancer remains the second most common cause of death during childhood. Beginning in the mid-1960s treatment of childhood cancer has witnessed remarkable advances in chemotherapy, radiotherapy, and surgical intervention, with improvements in survival for the majority of…

Rare Tumors of Childhood

This chapter covers the rare tumors of childhood. No attempt has been made to consider the rarest of the rare tumors, and the focus is on the more common of the rare pediatric tumors that do not fall into other categories covered in this text. The chapter is generally organized from top to bottom, with cancers of the head and neck at the beginning and cancers…

Histiocytoses

The histiocytoses constitute a collection of rare hematologic diseases that resist easy classification, at least in part because of the imprecise definition of “histiocyte.” “Histiocyte” broadly refers both to cells of the macrophage lineage and to dendritic cells (DCs), only some types of which are derived from macrophages. The numerous descriptive and functional subsets of macrophages and DCs only add to the confusion. Nonetheless, their collective…

Pediatric Germ Cell Tumors

Germ cell tumor (GCT) is the designation given to neoplasms arising from the cells of the germline—the cells that are destined to become either the egg or the sperm. A number of unique features of these tumors including their bimodal and wide age distribution, remarkable phenotypic diversity, and varying biologic behavior make GCTs a particular challenge for the surgeon, pathologist, and oncologist. Successful treatment regimens developed…

Osteosarcoma

Osteosarcoma is the most common primary bone tumor. There are approximately 600 cases/year in the United States. The term osteosarcoma was first used in the early 1800s by Alexis Boyer, the imperial family surgeon for Napoleon. Although low-grade forms of osteosarcoma do exist, more than 90% of osteosarcomas are high-grade malignant lesions. Low-grade osteosarcomas will be discussed, but most of what follows pertains to high-grade osteosarcomas.…

Ewing Sarcoma

In 1921 James Ewing described the cancer that came to carry his name: a primary bone tumor composed of small round blue cells and devoid of the malignant osteoid that characterizes osteosarcoma. Subsequently, pathologists described other clinicopathologic entities initially thought to be distinct, such as peripheral primitive neuroectodermal tumor (PNET) of bone or soft tissue or the Askin tumor of the chest wall. However, biologic study…

Nonrhabdomyosarcoma Soft Tissue Sarcomas and Other Soft Tissue Tumors

Soft tissue sarcomas of childhood and adolescence constitute a heterogeneous group of tumors that exhibit features of mesenchymal differentiation ( Tables 60-1 and 60-2 ). From a pediatric oncology perspective, the various types of rhabdomyosarcoma, the most common soft tissue sarcoma in younger children, are grouped separately such that the remainder of these tumors are collectively termed the nonrhabdomyosarcoma soft tissue sarcomas (NRSTS). This term seems…

Rhabdomyosarcoma

Pathologic Classification of Rhabdomyosarcoma The term rhabdomyosarcoma (RMS) comprises a heterogeneous family of soft tissue cancers that are related in poorly understood ways to the skeletal muscle lineage. Some of these tumors occur in the vicinity of skeletal muscle, but others occur in areas without obvious skeletal muscle; thus these cancers cannot be defined solely as tumors of skeletal muscle. Instead, this family is derived from…

Hepatoblastoma and Other Liver Tumors in Children

Introduction and Epidemiology Liver tumors in children account for approximately 1.1% of all malignancies in children younger than 20 years of age according to the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) reports. The age-adjusted incidence rate of liver tumors is approximately 11 per million in infants younger than the age of 1 year and approximately 6 per million in children between 1 and…

Tumors of the Brain and Spinal Cord

Tumors of the central nervous system (CNS) account for approximately 25% of pediatric cancer but are now the leading cause of cancer-related mortality in children. The complexities of tumors in this site are related to the large number of different histologies within the CNS and a historic nomenclature that is confusing, even to those in this field. With the need to modify therapies to spare important…

Retinoblastoma

Retinoblastoma is the most frequent neoplasm of the eye in childhood and the third most common intraocular malignancy in all ages, following malignant melanoma and metastatic carcinoma. Retinoblastoma represents 2.5% to 4% of all pediatric cancers but 11% of cancers in the first year of life. The average age-adjusted incidence rate of retinoblastoma in the United States and Europe is 2 to 5 per million children…

Pediatric Renal Tumors

Pediatric renal tumors account for approximately 5% of malignancies in children younger than 15 years old and 3.6% of malignancies in children younger than 20 years old. Among 9731 patients registered with the National Wilms Tumor Study Group (NWTSG) (1969–2002), Wilms tumor (WT) composed the vast majority of childhood renal tumors (92%), followed by clear cell sarcoma of the kidney (3.4%), congenital mesoblastic nephroma (1.7%), malignant…

Neuroblastoma

Neuroblastoma is an embryonal tumor of the sympathetic nervous system arising from the neural crest. It is a common extracranial solid tumor of childhood and is a disease distinguished by its clinical and biologic heterogeneity. The prognosis for persons with neuroblastoma is variable and largely dependent on tumor biology. Patients with localized disease and favorable tumor biology may be successfully treated with surgery alone or with…

Pediatric Lymphoma

Lymphomas are neoplasms caused by the malignant transformation of the constituent cells of the immune system. Combined, Hodgkin and non-Hodgkin lymphomas are the third most common malignancies in children and adolescents, with Hodgkin lymphoma being the most common cancer in people between the ages of 15 and 18 years. The treatment of children and adolescents with lymphoma is one of the important success stories in pediatric…

Infant Leukemias

The biologic features and clinical characteristics of infant leukemias differ significantly from those of leukemias in older children. Infant leukemias are distinguished not only by the young age of patients at diagnosis but by their unique morphologic, immunologic, clinical, and genetic presentation. For example, acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) present in infants with a somewhat unique constellation of clinical features, including hyperleukocytosis,…