Pediatric oncology II

Question 1

what is the most common malignancy in childhood?

Answer 1

ALL

Question 2

what is the pathogenesis of ALL?

Answer 2

1. lymphoid progenitor cell undergoes clonal expansion

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

Question 3

what are the three main signs of leukemia?

Answer 3

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

Question 4

what are the manifestations of bone marrow infiltration in leukemia?

Answer 4

1. anemia
2. low platelets
3. neutropenia
4. bone pain

Question 5

what are the manifestations of extramedullary disease in leukemia?

Answer 5

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

Question 6

orthopnea could alert physican to what type of mass?

Answer 6

mediastinal

Question 7

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

Answer 7

CBC

Question 8

what % of ALL cases will show elevated WBC?

Answer 8

50%

Question 9

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

Answer 9

95%

Question 10

what are the primary accepted nongenetic risk factors for ALL?

Answer 10

prenatal exposure to x rays and high dose radiation

Question 11

what is the presentation for precursor B cell ALL?

Answer 11

1. bone marrow involvement

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

Question 12

what is the presentation for precursor T cell ALL?

Answer 12

1. mediastinal mass
2. high WBC
3. prominent lymphadenopathy

Question 13

what are the main prognostic factors in B cell leukemia?

Answer 13

1. WBC count

2. age

Question 14

what is the greatest predictor of outcome for ALL?

Answer 14

response of induction therapy in bone marrow within 28 days

Question 15

what are the late effects of treatment for ALL?

Answer 15

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

Question 16

what are the prognostic indicators for AML?

Answer 16

1. age
2. race
3. cytogenetics

Question 17

what is the most common brain tumor in children?

Answer 17

astrocytoma

Question 18

what are the signs of posterior fossa tumors?

Answer 18

ataxia, tremors, dysarthria, stiff neck, papilledema

Question 19

what are the signs of brainstem tumors?

Answer 19

1. extremity weakness

2. cranical nerve signs

Question 20

what are the signs of hemispheric tumors?

Answer 20

1. hemiparesis
2. hemianopsia
3. aphasia
4. seizures

Question 21

what is generally needed for brain tumor diagnosis?

Answer 21

surgery

Question 22

what has the greatest impact on brain tumor outcome?

Answer 22

surgery

Question 23

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

Answer 23

neuro-axis radiation prophylaxis

Question 24

what role does chemotherapy have in childhood cancer cases?

Answer 24

adjunctive

Question 25

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

Answer 25

NF-1

Question 26

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

Answer 26

brain tumors

Question 27

what is the gene mutation associated with wilms tumor?

Answer 27

WT1

Question 28

what are the anomalies and syndromes associated with wilms tumor?

Answer 28

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

Question 29

what are the main history symptoms associated with wilms tumor?

Answer 29

1. hypertension | 2. gross hematuria

Question 30

what tests are used to evaluate for wilms tumor?

Answer 30

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

Question 31

what is the cause of retinoblastoma?

Answer 31

loss of both alleles of RB gene

Question 32

where do neuroblastomas occur?

Answer 32

any neural crest tissue - adrenal, paraspinal sympathetic tissue

Question 33

what is the clinical presentation for neuroblastoma?

Answer 33

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

Question 34

what is the most common extracranial solid tumor in children?

Answer 34

neuroblastoma

Question 35

what is the average age of diagnosis for neuroblastoma?

Answer 35

18 months

Question 36

what are the poor prognositc indicators for neuroblastoma?

Answer 36

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

Question 37

what are the tests to evaluate for neuroblastoma?

Answer 37

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

Question 38

what are the differences between osteogenic sarcomas and ewings sarcomas?

Answer 38

1. osteogenic - metaphysis of long bone | 2. ewings - diaphysis of long bone, all other bones