Overview of Pediatric Oncology Flashcards
1
Q
- The most common cancer in children, accounting for 30% of all childhood malignancies
- Acute lymphoblastic leukemia (ALL) accounts for 80% of cases
A
Acute leukemia
2
Q
- second most common of all childhood malignancies
- the most common pediatric solid organ tumor
A
primary malignant CNS tumors
3
Q
Most prevelant solid tumor in children outside of the cranium
A
neuroblastoma
4
Q
Risk factors for cancer by age?
A
0-2 years: neuroblastoma
2-5 years: ALL
10-15 years: osteosarcoma, Ewing sarcoma
15-19 years: Hodgkin lymphoma
5
Q
what are some cancer predispositon disorders?
A
- Trisomy 21 (leukemia)
- Fanconi anemia (AML and solid tumors)
- Li fraumeni syndrome
- von Hippel- llindaue disease
6
Q
- the overall risk of malignancy is up to 4x higher than that of the general population
- common tumors: Neurofibromas (most common overall; most are benign); optic pathway gliomas (most common intracranial neoplasm; most are low grasde astrocytomas and occur in kids <6)
A
Neurofibromas type 1
7
Q
- vestibular schwannoma (typically bilateral and benign)
- meningioma
- spinal tumors
A
Neurofibromatsosis Type 2
8
Q
pathogenesis of ALL
A
- B cell (85%) > T cell (10-15%)
- consecutive genetic lesions (i.e a sentinel hit followed by a secondary hit, resulting in an arrest in development and abnormal proliferation
- aberrant lymphoblast cell proliferation and survival is the hallmark of ALL
9
Q
- slight predilection for boys than girls
- Age 2-5 years
- often nonspecific
- common presenting signs and symptoms: Fever, pallor, bruising and/or petechiae, lymphadenopathy, anorexia, weight loss, bone pain, abdominal pain and/or distention, hepatosplenomegaly
A
ALL
10
Q
What are some specific scenerios that can be seen in ALL?
A
CNS involvement
- Headache, vomiting, lethargy, cranial nerve palsies, and/or papilledmea
Testicular involvement
- Persistant, painless, unilateral testicular enlargement/solid mass
- more commonly seen at a time of relapse
Mediastinal mass (esp. T-Cell)
- Orthopnea, facial flushing and or swelling, chest pain and/or stridor
11
Q
- leukemia
- adrenocortical carcinoma
- CNS tumors
- rabdomyosarcoma
- osteosarcoma
SLAB
A
Li Fraumeni Syndrome
12
Q
- retinal and CNS hemangioblastoma
- clear cell renal cell carcinoma
- pheochromocytoma
A
Von Hippel-lindau disease
13
Q
Evaluation of ALL
A
Labs
- Thrombocytopenia and/or anemia
- WBC count can be decreased, increased, or normal (may or may not have circulating blasts, often have a low absolute neutrophil count (ANS)
- elevated LDH and uric acid
Lumbar puncture
- Evaluate for the presence of leukemic blasts in the CSF
- Administer the first dose of intrathecal (IT) chemotherapy
Bone marrow aspirate and biopsy
- Gold standard for diagnosis
- > 25% blasts in the bone marrow (if 25%–> lymphoma)
- perform flow cytometry, immunohistochemistry, and cytogenetics
14
Q
ALL management
A
- most protocols take 2.5 years to complete
- multi drug regimen
- includes CNS-direted therapy w/IT chemo for all patients
15
Q
- Fever, malaise, bone pain, lymphadenopathy, hepatosplenomegaly
- gingival hypertrophy, chloroma
- bleeding/DIC (especially common in acute promyelocytic leukemia)
- clinical signs: Bleeding or oozing from various sites, peripheral cyanosis of the fingers or toes (microvascular thrombosis) signs and symptoms of stroke, hemodynamic instability
- lab findings: thrombocytopenia, elevated D-dimer, and PT/INR, decreased fribrinogen
A
AML
16
Q
large nuclei, usually with prominent nucleoli; may have auer rods (caused by abnormal fusion of primary granules)
A
AML
17
Q
Scant amount of cytoplasm, absence of cytoplasmic granules
A
ALL
18
Q
AML managment
A
- induction: typically 2 cycles of intense chemo
- conslidation (post-remission therapy): more chemo, allogenic BMT is offered to children with high-risk cytogenetics in
- IT chemotherapy is given to all children with AML
19
Q
treatment of APML
A
ATRA (all trans retinoic acid) can reduce the risk of DIC and should be started quickly when APML is suspected even if the diagnosis is not confirmed
20
Q
- clonal transformation of cells of B-cell origin
- pathognomic bi-nucleated reed-sternberg cells
- bimodal age distribution, peaks in the AYA population
- females> males
- possible causes: Genetic susceptibility, history of radiation or chemotherapy, certain infections, immunosuppressive agents, congential immunodeficiency, autoimmune disorders
A
Hodgkins lymphoma
21
Q
hodgkins lymphoma clinical presentation
A
- 80% present with painless lymphadenopathy (usually cervical, supraclavicular or axillary)
- rubbery and more firm than inflammatory adenopathy
- fatigue, anorexia, pruritus
- B symptoms: night sweats, weight loss, persistent unexplaind fevers
- mediastinal mass- more common among > 12 yers old
22
Q
evaluation of HL?
A
Evaluation
- labs are non-specific
- imaging: CXR; CT neck , chest abdomen and pelvis; FDG-PET CT skull base to thigh
Tissue Biopsy
- excisional biopsy is the gold standard
- needle biopsy does not provide adequate tissue because malignant cells may represent < 1% of the cellular infiltrate
- bone marrow aspirates are recommended for select patients
23
Q
HL staging
A
- lugano classification
- each disease stage is further noted according the the presence or absence of B symptoms, bulky disease, extranodal involvement
- stage 1: localized disease, one region or single organ
- stage 2: two or more lymph node regions on the same side of diaphragm
- stage 3: two or more lymph node regions about and below diaphragm
- stage 4: widespread disease, multiple organs with or without lymph node involvment
24
Q
HL management
A
low risk: 2-4 cycles of chemotherapy +/- radiation
intermediate risk: 2-6 cycles of chemotherapy + response based radiation
high risk: 3-5 cycles of chemotherpay + response based radiation
25
Q
- 5th most common diagnosis of pediatric cancer in children < 15 years
- pathogenesis similar to HL
- Congenital and acquired immunodeficiency syndromes and DNA damage repair syndromes place affected pts at risk
- Common varriable immunodeficiency (CVID), wiskott aldrich syndrome (WAS), Ataxia-telangiectasia, x-linked lymphoproliferative disease, HIV infection
A
Non-hodgkins lymphoma
26
Q
NHL clinical presentation
A
- painless lymphadenopathy is most common
- mediastinal mass: dyspnea, wheezing, stridor, dysphagia
- any organ, tissue, or anatomic area can be involved
- burkitt: abdominal pain that mimics appendicitis, intussuception, abdominal mass, sick!
- T- lymphoblastic leukemia: males > females, median age 12 years, 20% of pediatric NHL
27
Q
evaluation of NHL
A
- CT of the neck, chest, abdomen and pelvis
- FDG-PET scan
- skeletal scintigraphy can detect bone involvement
- bone marrow biopsy for cytology, immunophenotyping, and genetic evaluation should be performed on all pts
- lumbar puncture for examination of CHF
28
Q
NHL management
A
Burkitt lymphoma
- multi-agent combination of chemotherapy with or without immunotherapy (2-4 cycles of chemotherapy)
- no role for surgical debulking or radiation
T-lymphoblastic leukemia
- combination chemotherpay based on regimens used for ALL
29
Q
- Pilocytic astrocytoma (most common)
- > 1000 diagnosed annually
- median age: 8 years
A
low grade glioma (50%)
30
Q
DIPG
- 300 diagnosed annually
- mean age 5-9 years
- median survival:10-11 months
A
high grade glioma (10-15%)
31
Q
Embyonal tumors
A
Medulloblastoma
- 500 diagnosed annually
- median age 5-9
Atypical tertoid rhabdoid tumor
- < 100 diagnosed annually
- median age < 3 years
- median survival 12-24 months
Cranial and pasaspinal nerve turmors: Neurofibroma
- benign
- usually present by age 3
32
Q
Clinical presentation of CNS tumors
A
- may be subtle or nonspecific and may vary with the childs age and tumor location
common presenting signs and symptoms
- headache (most common- Early morning headache relieved by vomiting)
- nausea and vomiting
- clumsiness, incoordination, worsening handwriting
- speech changes: slurring, aphasia
- cranial nerve palsies
- vision changes: decreased visual acuity, squinting, head tilt
- seizues
- developmental delay and behavioral change
33
Q
how does age and location of the CNS tumor change clinical presentation?
A
Age
- infants: macrocephaly is the most common
- infants and young children are more irritable
- older: headache, abnormal gait, poor coordination, papilledema, and seizures
Location
- supratentorial and central: generally nonspecific but most common is headache
- posterior fossa: nausea/vomiting, headache, abnormal gait, and coordination
- brainstem: abnormal gait and coordination, cranial nerve palsies
- spinal cord: back pain and/or weakness and abnormal gait
34
Q
evaluation of CNS tumors
A
- CT (preferred in emergent situations)
- MRI with contrast (best modality for imaging CNS tumors)
- Biopsy and reduction of tumor done at the same time
- long term prognosis of some types is dependent on degree of surgical resection
35
Q
management of CNS tumors
A
- Surgery (near total resection can be achieved in most cases)
- Chemotherapy (routinely used in young children with specific tumor types with the goal of delaying or replacing radiation therapy
36
Q
- usually avoided in children < 3 years due to risk of severe neurocognitive sequelae
- associated with several complication
- acute: headache, nausea, drowsiness
- somnolence syndrome (occurs anywhere from a few weeks to 3 months after treatment)
- late: impaired neurocognitive funciton and social/behavioral deficits, hearing and or vision impairment
A
Radiation therapy
CNS tumor management
37
Q
NBL pathogenesis
A
- abnormal growth of embryonic neural crest cells that normally make up the sympathetic ganglia and adrenal medulla
- most common extracranial solid tumor in children
- genetic syndromes associated: Congenital hypoventilation syndrome, neurofibromatosi, costello syndrome, noon syndrome, li-fraumeni syndrome
38
Q
NBL clinical presentation
A
- metastatic disease to the bone marrow, liver and kin is seen in 50% of patients at presentation
- constitutional symptoms (fever, malaise, pallor, fussiness)
- blueberry muffin rash
- spinal cord compression: pain, numbness, limb weakness
- hornor syndrome: suggests impingement of the oculosympathetic tract; ptosis, miosis, anhydrosis
- opsoclonus myoclonus syndrome: rapid multidirectional eye movements, involuntary muscle spasms, irritability
- raccoon eyes: periorbital ecchymosis represent metastatic spread to the retrobulbar region
39
Q
NBL management
A
- Half of all cases of neuroblastoma found in infants < 6 months spontaneously resolve
- most patients > 12 months of age are stage IV and require intense multimodal therapy
- MIBG therapy
40
Q
- thought to arise from a mesenchymal stem cell that differentiates towards fibrous tissue, cartilage or bone
- risk factors: genetic syndromes and germline conditions -Heredirtary RB, Li-fraumeni syndrome, round-Thomson syndrome
- prior cancer treatment, especially exposure to alkylating chemotherapy agents
- rapid bone growth (peak incidence during an adolescent growth spurt)
- males > females
- African American race
A
Osterosarcoma
41
Q
OSt: Clinical presentation
A
- but is the most common primary malignant bone tumor
- most common symptom is localized pain (frequently begins after injury)
- systemic symptoms: (ex. fever, weight loss and malaise) are generally ABSENT
- most important physical finding is a large soft tissue mass that is tender to palpation
42
Q
Ost Evaluation
A
- plain film radiographs (sunburst: variable ossification of the soft tissue mass in a radial pattern, formation of codman’s triangle ,pathlogic fracture)
- MRI of the entire length of the involved long bone is superior to CT
- CT is the best modality to evaluate the chest for metastatic disease
- radionuclide bone scan and/or PET scan for whole body staging
- BIOPSY is required for definitive diagnosis
43
Q
- increasing evidence supports a mesenchymal progenitor cell origin
- nonrandom chromosomal translocations that involve breakpoints
- 2nd most common primary bone malignancy
- 200 new diagnoses per year, peak incidence in children 10-15 years
- males > females
- caucasian
A
Ewing sarcoma
44
Q
EWS clinical presentation
A
- can develop in any bony or soft tissue but tend to develop in the diaphysis of long bones and the axial skeleton
- localized pain or swelling of varying duration: may be mild at first but intensifies rapidly, may be aggravated by exercise, and often worse at night
- minor trauma may be the initiating event that calls attention to the lesion
- constitutional symptoms: seen in 10-20% of patients and often associated with advanced disease
- fever, fatigue, and weight loss
