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Year 2 Hematology > Pediatric oncology II > Flashcards

Pediatric oncology II Flashcards

1
Q

what is the most common malignancy in childhood?

A

ALL

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2
Q

what is the pathogenesis of ALL?

A
  1. lymphoid progenitor cell undergoes clonal expansion

2. altered expression of genes whose products contribute to normal development of B and T cells

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3
Q

what are the three main signs of leukemia?

A
  1. bone marrow infiltration
  2. extramedullary disease
  3. fever of malignancy
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4
Q

what are the manifestations of bone marrow infiltration in leukemia?

A
  1. anemia
  2. low platelets
  3. neutropenia
  4. bone pain
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5
Q

what are the manifestations of extramedullary disease in leukemia?

A
  1. lymphadenopathy
  2. hepatosplenomegaly
  3. orthopnea, cough
  4. gingival hypertrophy
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6
Q

orthopnea could alert physican to what type of mass?

A

mediastinal

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7
Q

what is the most helpful test in the diagnosis of ALL?

A

CBC

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8
Q

what % of ALL cases will show elevated WBC?

A

50%

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9
Q

what % of ALL patients will have 2 or more cytopenias?

A

95%

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10
Q

what are the primary accepted nongenetic risk factors for ALL?

A

prenatal exposure to x rays and high dose radiation

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11
Q

what is the presentation for precursor B cell ALL?

A
  1. bone marrow involvement

2. frequently also extramedullary - CNS, skin, gonads, lymph nodes, liver, spleen

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12
Q

what is the presentation for precursor T cell ALL?

A
  1. mediastinal mass
  2. high WBC
  3. prominent lymphadenopathy
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13
Q

what are the main prognostic factors in B cell leukemia?

A
  1. WBC count

2. age

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14
Q

what is the greatest predictor of outcome for ALL?

A

response of induction therapy in bone marrow within 28 days

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15
Q

what are the late effects of treatment for ALL?

A
  1. osteonecrosis, osteopenia
  2. neuropathies
  3. neuropsychometric concerns
  4. obesity
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16
Q

what are the prognostic indicators for AML?

A
  1. age
  2. race
  3. cytogenetics
17
Q

what is the most common brain tumor in children?

A

astrocytoma

18
Q

what are the signs of posterior fossa tumors?

A

ataxia, tremors, dysarthria, stiff neck, papilledema

19
Q

what are the signs of brainstem tumors?

A
  1. extremity weakness

2. cranical nerve signs

20
Q

what are the signs of hemispheric tumors?

A
  1. hemiparesis
  2. hemianopsia
  3. aphasia
  4. seizures
21
Q

what is generally needed for brain tumor diagnosis?

A

surgery

22
Q

what has the greatest impact on brain tumor outcome?

A

surgery

23
Q

what is the treatment for a tumor that disseminates via CSF?

A

neuro-axis radiation prophylaxis

24
Q

what role does chemotherapy have in childhood cancer cases?

A

adjunctive

25
Q

astrocytomas are associated with what mutation in 50-80% of cases?

A

NF-1

26
Q

what is the leading cause of morbidity and mortality among pediatric cancers?

A

brain tumors

27
Q

what is the gene mutation associated with wilms tumor?

A

WT1

28
Q

what are the anomalies and syndromes associated with wilms tumor?

A
  1. hemihypertrophy
  2. aniridia
  3. denys-drash syndrome
  4. beckwith-widemann syndrome
29
Q

what are the main history symptoms associated with wilms tumor?

A
  1. hypertension

2. gross hematuria

30
Q

what tests are used to evaluate for wilms tumor?

A
  1. CBC
  2. CT abdomen
  3. CT chest
  4. CXR
  5. abdominal US
31
Q

what is the cause of retinoblastoma?

A

loss of both alleles of RB gene

32
Q

where do neuroblastomas occur?

A

any neural crest tissue - adrenal, paraspinal sympathetic tissue

33
Q

what is the clinical presentation for neuroblastoma?

A
  1. large abdominal mass crossing midline
  2. lower extremity weakness
  3. cervical, high thoracic mass - horner’s syndrome
34
Q

what is the most common extracranial solid tumor in children?

A

neuroblastoma

35
Q

what is the average age of diagnosis for neuroblastoma?

A

18 months

36
Q

what are the poor prognositc indicators for neuroblastoma?

A
  1. age over 18 months
  2. myc-n amplification
  3. unfavorable histology
37
Q

what are the tests to evaluate for neuroblastoma?

A
  1. urine catecholamines
  2. imaging - CT, bone scan
  3. BM biopsy
  4. lymph node evaluation
38
Q

what are the differences between osteogenic sarcomas and ewings sarcomas?

A
  1. osteogenic - metaphysis of long bone

2. ewings - diaphysis of long bone, all other bones

Year 2 Hematology (34 decks)

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