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Pediatric Exam 2 > Overview of Pediatric Oncology > Flashcards

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

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2
Q
  • second most common of all childhood malignancies
  • the most common pediatric solid organ tumor
A

primary malignant CNS tumors

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3
Q

Most prevelant solid tumor in children outside of the cranium

A

neuroblastoma

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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

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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
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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

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7
Q
  • vestibular schwannoma (typically bilateral and benign)
  • meningioma
  • spinal tumors
A

Neurofibromatsosis Type 2

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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
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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

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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
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11
Q
  • leukemia
  • adrenocortical carcinoma
  • CNS tumors
  • rabdomyosarcoma
  • osteosarcoma

SLAB

A

Li Fraumeni Syndrome

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12
Q
  • retinal and CNS hemangioblastoma
  • clear cell renal cell carcinoma
  • pheochromocytoma
A

Von Hippel-lindau disease

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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
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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
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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

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16
Q

large nuclei, usually with prominent nucleoli; may have auer rods (caused by abnormal fusion of primary granules)

A

AML

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17
Q

Scant amount of cytoplasm, absence of cytoplasmic granules

A

ALL

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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

Pediatric Exam 2 (8 decks)

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