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Langerhans Cell Histiocytosis (LCH)

1 What are the former names of Langerhans cell histiocytosis? What is known today as Langerhans cell histiocytosis (LCH) was first described in the early 1900s. By 1953, the diagnoses of eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease, and Hashimoto-Pritzker disease were grouped together as a disease spectrum known as Histiocytosis X. The “X” stood for “unknown,” representing the lack of understanding of the underlying pathology. In…

Retinoblastoma

1 Describe the underlying genetic defect in retinoblastoma. Retinoblastoma, a primitive neuroectodermal cell tumor, develops as a result of an absence of pRb, the retinoblastoma protein, which functions as a regulator of the cell cycle by modifying transcription factors, which allow progression into the S phase. Lack of functional pRb primarily occurs as a result of mutations in RB1, the retinoblastoma gene. These mutations can occur…

Post-Transplant lymphoproliferative disorder (PTLD)

1 What infectious agent plays a central role in the development of most post-transplant lymphoproliferative disorder and why? Epstein-Barr virus (EBV), a ubiquitous human herpesvirus transmitted primarily via saliva, is causally involved in most lymphoproliferative disease. Initial infection with EBV occurs during childhood but persists as a latent infection throughout life. EBV colonizes antibody-producing B cells and thus evades recognition and destruction by cytotoxic T cells.…

Neuro-Oncology

1 How common are childhood central nervous system tumors? Approximately 3500 cases of pediatric central nervous system (CNS) tumors are diagnosed each year in the United States with an incidence of 6.1 per 100,000. They are the most common solid tumors in the pediatric age group and are the leading cause of cancer-related death in this population. 2 How are brain tumors classified? Brain tumors are…

Non-hodgkin lymphoma in children and adolescents

1 Describe the epidemiology of non-Hodgkin lymphoma in children and adolescents. Non-Hodgkin lymphoma (NHL) is a heterogeneous category that includes a wide diversity of malignancies derived from lymphocytic cell origin. Although the World Health Organization (WHO) classification of lymphoid neoplasms includes more than 50 distinct diagnostic entities, many of them are extremely rare in the pediatric population. Five lymphomas account for more than 90% of all…

Hodgkin lymphoma in children and adolescents

1 Describe the incidence and epidemiology of Hodgkin lymphoma in children and adolescents. There are approximately 1200 new cases of Hodgkin lymphoma (HL) diagnosed in the United States annually among children and adolescents. Among adolescents, HL is associated with a higher socioeconomic background. In contrast, the incidence of HL among young children tends to increase with increasing family size and lower socioeconomic status. The mixed cellularity…

Kidney tumors in children and adolescents

1 What is the differential diagnosis of a child with an abdominal mass? The most likely diagnoses in a child with an abdominal mass are neuroblastoma and Wilms tumor or nephroblastoma. Clinically, children with advanced stage neuroblastoma appear ill, often having signs or symptoms of metastatic disease such as periorbital ecchymoses or pain from skeletal lesions. Patients with Wilms tumor are often asymptomatic, and the mass…

Hepatic tumors

1 What is the differential diagnosis of a liver mass in a child? Liver tumors in children are rare but mostly malignant. Differential diagnosis varies by age. In infants and toddlers, the most common malignant liver mass is hepatoblastoma (HB; 43%). Rhabdoid tumors (1%) and malignant germ cell tumors (1%) are less likely. The most common benign liver masses in this age group are vascular tumors/hemangiomas…

Neuroblastoma

1 What is neuroblastoma? Neuroblastoma is the most common extracranial malignant tumor of infants and children. It originates from neural crest cells that normally give rise to the adrenal gland and the sympathetic nervous system. It can have widely varying outcomes: tumors can spontaneously regress, differentiate into benign ganglioneuromas, or metastasize with high mortality rate. 2 What is the incidence of neuroblastoma in the United States?…

Bone tumors

1 What are the most common malignant bone tumors in children, adolescents, and young adults? Malignant bone tumors account for 6% of all childhood malignancies. Osteosarcoma (OS) represents 56% of new malignant bone tumor diagnosis, followed by Ewing sarcoma (ES) with 34% of new diagnosis. OS is the most common malignant bone tumor in this patient population, with approximately 400 to 450 new diagnoses each year…

Soft tissue sarcomas

1 What is a soft tissue sarcoma? Soft tissue sarcomas (STS) are a diverse group of malignant tumors believed to originate from developing mesenchyme, which normally give rise to components of connective tissue (muscle, bone, fat, cartilage, blood vessels). The subtypes of STS have classically been named based on the normal tissue type that any given STS most closely resembles. 2 What are the key recurrent…

Chronic myeloid leukemia/other leukemia

1 What is the chromosomal abnormality associated with chronic myeloid leukemia? The Philadelphia chromosome—or t(9;22)(q34.1;q11.2) resulting in a BCR-ABL1 fusion gene—is the hallmark of 90% to 95% of chronic myeloid leukemia (CML) cases. The remaining cases consist of variant and cryptic translocations that also result in the characteristic BCR-ABL1 fusion gene. 2 What should be included in the initial evaluation of a patient with suspected CML?…

Acute myeloid leukemia

1 Discuss the incidence of acute myeloid leukemia in children in the United States. There are 500 to 600 new cases of pediatric acute myeloid leukemia (AML) diagnosed annually in the United States and approximately 10,000 cases diagnosed each year worldwide. The incidence is 5 to 7 cases per million people per year. AML accounts for about 25% of pediatric leukemias. There is a bimodal distribution,…

Acute lymphoblastic leukemia

1 Describe the frequency of acute lymphoblastic leukemia of childhood. Acute lymphoblastic leukemia (ALL) is the most common malignancy in children, accounting for one-fourth of all childhood cancers and 72% of all childhood leukemia. The annual incidence rate is 3 out of 100,000 children. About 4900 children are diagnosed with ALL each year in the United States. Peak incidence is 3 to 5 years old. 2…

Principles of pediatric surgical oncology

1 What are the surgical guidelines for the treatment of neuroblastoma? How does stage affect operative management decisions? Surgical goals include establishing a definitive diagnosis, accurate staging, and gross total resection, if possible. For stage I to II disease, upfront resection is encouraged with complete dissection of the primary tumor site, regional lymph nodes, and adjacent vasculature. En-bloc resection and/or injury to surrounding vital structures is…

Global health issues in pediatric oncology

1 How does the epidemiology of childhood cancer vary in different geographic regions of the world? Variation in the epidemiology of childhood cancer worldwide is largely influenced by the impact of infection-associated malignancies ( Figure 28.1 ). Although acute lymphoblastic leukemia (ALL) is the most common pediatric malignancy observed in most geographic regions of the world, equatorial sub-Saharan Africa has long been characterized by the uniquely…

An overview of the Emotional and educational needs of children with hematological and oncological conditions

1 What are the emotional and educational needs of children with hematological/oncological conditions? Although every child is different, there are certain considerations when it comes to the emotional well-being of children with chronic and acute medical conditions. A group of psychosocial experts came together to establish standards of care for children with oncological conditions, many which are applicable across pediatric medical conditions. They highlight, among other…

Palliative Care

1 What are the differences between palliative care, end-of-life care, and hospice care? Palliative care for children and adolescents is an evolving field but can be defined as an interdisciplinary collaborative approach to improve the quality of life of children with life-threatening or profoundly challenging conditions, as well as that of their families, inclusive of physical, emotional, social, and spiritual elements. Hospice, or end-of-life care, is…

Integrative therapies

1 Describe complementary and integrative health therapies. Complementary and integrative health therapies (CIHTs) are nonmainstream practices used in combination or together with conventional medicine. CIHTs are divided into three categories: natural products, mind and body practices, and whole-systems. Natural products include dietary supplements such as botanicals, vitamins and minerals, and probiotics. Mind and body practices include acupuncture, acupressure, aromatherapy, healing touch, hypnotherapy, massage therapy, movement therapies,…

Survivorship and late effects

1 Describe the goals of survivorship care and the role of the survivorship clinic. Childhood cancer survivors are at an increased risk for significant adverse health effects and impaired quality of life after disease-related therapy. Multimodal regimens, consisting of chemotherapy, radiation, and surgery, can result in a myriad of severe chronic health conditions, secondary malignancies, physical limitations, psychological and neurocognitive dysfunction, infertility, and premature frailty and…