SM_282b: Pediatric Cancer Flashcards
___ are the leading cause of disease-related death in children
Malignant neoplasms are the leading cause of disease-related death in children
___ and ___ are the most common in children
Cancers of lymphohematopoietic origin and CNS tumors are the most common in children
- Tumors of developmental origin are common in younger children
- Epithelial tumors of lung, breast, prostate, colon RARE in kids
Describe genetic conditions associated with pediatric cancer
Genetic conditions associated with pediatric cancer
- NF1 and NF2
- Down syndrome
- Beckwith-Wiedemann syndrome
- Tuberous sclerosis
- von Hippel-Lindau disease
- Xeroderma pigmentosum
- Ataxia-telangiectasia
- Nevus basal cell carcinoma syndrome
- Li-Fraumeni syndrome (p53)
Describe viruses associated with childhood cancer
Viruses associated with childhood cancer
- Hodgkin’s lymphoma: EBV
- Burkitt’s lymphoma: EBV (Africa only)
- Hepatocellular carcinoma: Hepatitis B and C
Children with cancer often present with ___
Children with cancer often present with vague signs and symptoms
- Pain is most common presenting symptom of cancer: deeper, visceral structures of organ parenchyma
- Disseminated disease common
Describe common signs and symptoms of pediatric cancer
Common signs and symptoms of pediatric cancer
- Pallor, bruising, persistent fever or infection
- Pain: persistent or unexplained
- Headache w/ neurologic deficit
- Morning headache with vomiting
- Lymphadenopathy
- Abdominal pain or mass
- Extremity swelling
- Eye changes including proptosis or lack of normal red reflex
Describe age specific challenges in diagnosis of pediatric cancer
Age specific challenges in diagnosis of pediatric cancer
- infants and young kids have limited ability to express their pain or discomfort
- Heterogeneity in normal growth and development
- Adolescents and young adults at risk for delayed diagnosis due to limited interactions with providers and potential loss of health coverage
Describe general principles of treatment for pediatric cancer
General principles of treatment for pediatric cancer
- Treat at appropriate referral centers
- Multi-disciplinary care providers
- Best chance to cure is at first diagnosis
- Maximize cures while limiting late effects
- Prioritize outpatient care
Describe multimodal treatment approach for pediatric cancer
Multimodal treatment approach for pediatric cancer
- Establish accurate diagnosis
- Initiate treatment rapidly when necessary
- Chemo is important: more sensitive and tolerate w/ less toxicity
- Biologic agents becoming more important for up-front therapy
- Avoid or delay radiation due to serious side effects
Describe lymphohematopoetic malignancies
Lymphohematopoetic malignancies
- Unregulated clonal proliferation of cells in bone marrow (leukemia) or lymph nodes (lymphoma)
- Growth advantage over normal cells and result in defect in normal function of bone marrow or lymph nodes
___ is the most common malignant neoplasm of childhood
Acute leukemia is the most common malignant neoplasm of childhood
___ accounts for most cases of childhood leukemia
ALL accounts for most cases of childhood leukemia
Describe principles of treatment for pediatric leukemia
Principles of treatment for pediatric leukemia
- Infants have particularly poor prognosis
- Patients > 10 years and those with adverse molecular features require more aggressive treatment
- Pace of improvements in cures of acute myeloid leukemia have been slow, with overall survival stagnant between 50-60%
Describe pediatric lymphomas
Pediatric lymphomas
- Third most common malignancy in kids
- Broadly distributed between Hodgkin’s and non-Hodgkin’s based upon histologic and molecular features
____ are leading cause of cancer-related death in kids
CNS tumors are leading cause of cancer-related death in kids
____ medulloblastoma has good outcomes, while ____ medulloblastoma has poor outcomes
Childhood medulloblastoma has good outcomes, while infant medulloblastoma has poor outcomes
High grade glioma and DIPG have ___ prognosis
High grade glioma and DIPG have poor prognosis
Describe neuroblastoma
Neuroblastoma
- Embryonal cancer of PNS that commonly affects younger kids
- Most common extra-CNS solid tumor in kids
- Heterogeneous presentation and course: ranges from tumors that undergo spontaneous regression to very aggressive tumors unresponsive to intense multimodal therapy
- Most cases are sporadic
- Patients with low and intermediate risk disease have good outcomes
- High risk disase has poor outcomes and requires aggressive multimodal treatment
Describe Wilms tumor (nephroblastoma)
Wilms tumor (nephroblastoma)
- Second most common extra-cranial solid tumor
- Large
- Good outcomes after surgery, chemo, and radiation
- Model disease for improvements in survival through cooperative group efforts
- Associated with Beckwith-Weideman syndrome
Describe pediatric hepatoblastoma
Pediatric hepatoblastoma
- Rare developmental tumor of liver
- Treatment includes chemo, resection, and occasional liver transplantation
- Prematurity is known risk factor particularly when umbilical vein catheterization
- Associated with Beckwith-Weidemann syndrome, hemihypertrophy, and APC gene mutations
Describe pediatric sarcomas
Pediatric sarcomas
- Occur at any age across pediatric spectrum
- Primarily disease of adolescents and young adults
- Can arise in any anatomic site
- Outcomes vary
____ is the most common pediatric soft tissue sarcoma
Rhabdomyosarcoma is the most common pediatric soft tissue sarcoma
- Neck, GU tract, extremities
Describe pediatric osteosarcoma
Pediatric osteosarcoma
- Highest risk during adolescent growth spurt, suggesting relationship between rapid bone growth and malignant transformation
- Diagnosis confirmed by biopsy and patients require surgeyr and chemo to achieve cure
- Surgery results in prostheses
____ is a ____ common in kids characterized by t(11;22)
Ewing’s sarcoma is an undifferentiated sarcoma of bone often with a prominent soft-tissue component common in kids characterized by t(11;22)
Ewing’s sarcoma is characterized by ___
Ewing’s sarcoma is characterized by t(11;22)
Describe retinoblastoma
Retinoblastoma
- Loss of function of RB1 gene via mutation or deletion
- Hereditary cases diagnosed at younger age and are multifocal and bilateral
- Sporadic cases usually diagnosed in older ki]ds w/ unilateral and unifocal involvement
- High survival rate in developed but not elsewhere
- Associated loss of vision and side effects of therapy
___ are a model for improving outcomes for pediatric cancer
Cooperative group clinical trials are a model for improving outcomes for pediatric cancer
- Research consortium
- Challenges in pediatric cancer research: lack of funding, difficulty motivating pharma companies, rare tumor with limited tumor specimens and patients, long latency between therapeutic concepts and implementation
