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Flashcards in CHildhood CA Deck (148):
1

How many children are diagnosed with CA each day?

46

2

What is the death rate of cancer in children?

>3000

3

What is the number 1 cause of disease related death for children?

Cancer

4

What is the most common cause of death ages 104?

Congenital abnormalities (CA second)

5

What is the average age at diagnosis for childhood cancer?

6

6

What is the average years of life lost in an adult CA patient? Child?

15 in adult, 71 in children

7

What is the funding like for childhood cancer?

Very small

8

What is the odds of encountering a 20 year who survived childhood cancer?

1:750

9

What is the most common type of childhood cancer?

leukemia
(ALL)

10

What is the second most common CA in children?

CNS

11

What is WIlm's tumor?

***

12

What are the two most common causes of cancer in adolescence?

Hodgkin's lymphoma
Thyroid carcinoma

13

What is happening to the mortality rate of childhood cancer?

Lower

14

Survival is greatest for what childhood cancer? Second?

Wilm's tumor
ALL

15

What is the children's oncology group?

Cooperative of Drs that treat childhood CA

16

What are the causes of childhood cancer?

Largely Unknown--environmental causes have been difficult to identify

17

What is neurofibromatosis?

***

18

What is familial polyposis?

***

19

What is Li-Fraumeni syndrome?

p53 mutation

20

What are the three categories of diseases linked with increased cancer risk?

-Immunodeficiency
-Metabolic disorders
-Disorders of chromosome stability

21

What are the ssx of childhood CA?

Nonspecific, and often mimic childhood problems

22

How many providers are seen before a diagnosis of childhood cancer is made?

3

23

What are the orders you want to make with HTN in children? What are the tumors associated with this?

CXR
abd US


Renal or abdominal tumors (NBL)

24

What are the orders you want to make with sudden onset weight loss in children? What are the tumors associated with this?

Abd US

Any malignancy

25

What are the orders you want to make with petechiae in children? What are the tumors associated with this?

CBC, manual diff

Leukemia/NBL

26

What are the orders you want to make with adenopathy unresponsive to ABs in children? What are the tumors associated with this?

surgical consult
CXR
CBC, manual diff


Leukemia/lymphoma

27

What are the orders you want to make with endocrine abnormalities (growth failure, electrolyte disturbances, sexual abnormalities, Cushings) in children? What are the tumors associated with this?

Hormonal assays
CT hypothalamic areas
Abd CT

Pituitary tumors
Hypothalamic tumors
Gonadal tumors
Adrenal tumors

28

What are the orders you want to make with (headache, CN palsy, ataxia, dilated pupils, afebrile szs, hallucinations, unilateral weakness) in children? What are the tumors associated with this?

Neuro/surgery consult

Brain tumor

29

What are the orders you want to make with (white spots on eyes, ptosis, wandering eye) in children? What are the tumors associated with this?

Opthalmological consult
Rb, or metastatic neuroblastoma

30

What are the orders you want to make with bulging external ear mass, in children? What are the tumors associated with this?

CBC, diff, imaging

LCH, RMS

31

What are the orders you want to make with puffy face in children? What are the tumors associated with this?

CBC/diff
Imaging

mediastinal masses

32

What are the orders you want to make with pharyngeal masses in children? What are the tumors associated with this?

CBC/diff, imaging

RSM, lymphoma, nasopharyngeal carcinomas

33

What are the orders you want to make with periodontal masses in children? What are the tumors associated with this?

Dental consult

LCH, burkitt's lymphoma

34

What are the orders you want to make with thoracic masses in children? What are the tumors associated with this?

CBC/diff, imaging

Soft tissues tumors, mediastinal tumors

35

What are the orders you want to make with intra abdominal masses in children? What are the tumors associated with this?

abd US, CBC

Wilms tumor
neuroblastoma
Hepatocellular carcinoma

36

What are the orders you want to make with testicular masses or masculinization/feminization in children? What are the tumors associated with this?

UA, CBC, US

Germ cell tumors

37

What are the orders you want to make with musculoskeletal issues in children? What are the tumors associated with this?

CBC, x-rays

Osteosarcoma
Ewig's sarcoma
Neuroblastoma

38

What is Ewing's sarcoma?

***

39

What are the ssx of leukemia? (6)

-fever 101F >7 days
-petechiae
-unexplained anemia/pallor
-Generalized lymphadenopathy
-HSM
-bone or joint pain

40

What are the ssx of brain tumors?

Szs, weakness, coordination difficulties

Headaches and vomiting

41

Why are headaches and vomiting common in childhood brain tumors?

Compression of CSF circulation

42

When are headaches concerning for brain tumors?

Changing character
Persistent

43

Regression of developmental milestones are concerning for what type of tumor?

Brain tumor

44

What size are lymph nodes considered "large"?

> 10 mm except for epitrochlear nodes (>5 mm) or inguinal (>15 mm)

45

What are the two common bacterial causes of lymphadenopathy?

Cat scratch disease
Staph and strep

46

What are the common viruses that cause lymphadenopathy?

EBV and herpes

47

What are the two characteristics of lymph nodes that make them concerning for CA?

-Generalized
-Regional not involving the head to neck

48

What lymph nodes are particularly concerning for malignancy? (3)

posterior auricular
Epitrochlear
Mediastinal

49

When is a biopsy of a node recommended?

Enlarging after 2-3 weeks of abx

unchanged 6-8 weeks

50

True or false: any nodes associated with any abnormal CXR are concerning for CA

True

51

What are the masses that always require a CA work up?

Abdominal, thoracic soft tissue

52

True or false: bone pain is usually an early symptom of malignancy

False--late symptom

53

Bone or joint pain is a presenting symptom of what percent of pts with ALL?

30%

54

Ostalgia caused by CA is usually confused with what?

RA

55

When is bone/joint pain particularly concerning?

Persistent
Associated with swelling or mass
LROM

56

How do you differentiate ITP from malignancy?

Low platelets, ITP is usually preceded by a viral infection

57

What is the most common malignancy in childhood?

ALL

58

What is the peak age for ALL?

2-5 years

59

ALL accounts for what fraction of all childhood cancers?

1/3

60

In what race is ALL most common?

caucasians

61

What sex has more cases of ALL?

Males

62

What is the pathogenesis of ALL?

Lymphoid progenitor cells becomes genetically altered and undergo dysregulated proliferation and clonal expansion

63

What is the test to distinguish between what type of lymphoma it is?

Flow cytometry

64

Where does ALL arise from?

Bone marrow, but may present anywhere in the body

65

What are the ssx of ALL? (4)

anemia
Decreased platelets
Neutropenia
Ostalgia

66

What are the ssx of extramedullary leukemia? (lymph, respiratory, neuro, GU, skin, mouth)

-Lymphadenopathy/HSM
-Orthopnea/cough
-facial nerve palsy
-Testicular enlargement
-Gingival hypertrophy

67

What percent of ALL pts have leukocytosis?

50%

68

What percent of ALL pts with have lymphoblasts on peripheral smear?

80%

69

What percent of ALL pts have >2 cytopenias?

95%

70

What percent of ALL pts will have 1 cytopenia?

4%

71

What percent of ALL pts will have a normal CBC and diff?

1%

72

Why do you want a manual differential when diagnosing ALL?

May mistake cells

73

What are the two infections and two diseases that mimic leukemia?

1. EBV
2. Parvovirus B19
3. Acute anemia
4. ITP

74

Incidence of ALL (specifically) is highest in what ethnicity?

Hispanic

75

Children with what disease have an increased risk of ALL?

Down syndrome

76

The primary nongenetic risk factor for developing ALL is what?

prenatal exposure to x-rays

77

What type of cell is usually implicated in ALL?

B cells

78

What is the usual presentation of B-ALL?

BM involvement, but frequently CNS, gonads, lymph nodes

79

What percent of children have T-ALL?

15%

80

Who is usually afflicted with T-ALL?

Male adolescence

81

How does T-ALL usually present?

Looks like mumps with mediastinal mass and leukocytosis

82

What is the standard risk for ALL?

Age 1-10 yo
WBC

83

What is the high risk standard for ALL?

Age 10 yrs
WBC >50

84

What are the two cell types that are considered high risk ALL?

Precursor T
Mature B

85

What is the greatest predictor of overall outcome of ALL?

Response to therapy in the first few days

86

What is the maintenance period for males? Females?

Males = 3.5 years
Females == 2.5 years

87

CNS prophylaxis is indicated in what types of ALL?

T cell or with CNS involvement

88

What are the f/u periods for ALL?

5 years after chemo, then every 10 years

89

What percent of ALL pts attain remission?

95%

90

What is the infection that ALL pts are predisposed to?

PCP pneumonia

91

What is the CA-free rate at 5 years in ALL?

75-85%

92

What are the four late effects of ALL?

1. Osteonecrosis/osteopenia
2. Neuropathies
3. Neuropsychometric concerns
4. Obesity

93

What is the side effect of vincristine use?

Neuropathy

94

What is the MOA of vincristine?

Inhibits tubule formation/breakdown

95

What is the treatment for AML?

Cytarabine (ara-b)
Daunorubicin

96

What are the three prognostic indicators for AML?

age
race
Cytogenetics

97

What is the cure for AML?

Bone marrow transplant

98

What are the side effects of daunorubicin?

LVH
***cardiomyopathy

99

What is the most common SOLID tumor of childhood?

Brain tumors

100

Of the brain tumors, which type has the highest incidence in children?

Astrocytoma

101

Astrocytomas are usually associated with what genetic disorder?

Neurofibromatosis

102

What is the treatment for astrocytomas?

Brain surgery

103

What are the ssx of increased intracrainial pressure?

Papilledema
Loss of vision
Bulging fontanelles/spreading sutures

104

What are the ssx of posterior fossa and brainstem?

ataxia
tremors
dysarthria
stiff neck
CN signs

105

What are the ssx of hemispheric tumors?

Hemiparesis or hemianopsia

106

What are the three ways to diagnose brain tumors?

CSF
Imaging
Biopsy

107

Surgery, XRT, or chemo for: medulloblastoma?

surgery and chemo

108

Surgery, XRT, or chemo for: low grade astrocytoma?

surgery and focal XRT

109

Surgery, XRT, or chemo for: optic glioma?

NOT surgery

110

Surgery, XRT, or chemo for: ependymomas?

Surgery XRT, and focal chemo

111

Surgery, XRT, or chemo for: germ cell tumors?

XRT and chemo

112

Surgery, XRT, or chemo for: high grade astro/GBM

surgery

113

What are the two exceptions of surgery for brain tumors?

GCT BSG

114

What is the single best treatment for brain tumors?

Surgery

115

What is neuroaxis prophylaxis?

treating neuro axis via CSF

116

True or false: chemo is usually an adjuvant therapy in brain CAs?

True

117

What is the limiting factor for chemo in brain tumor?

Blood brain barrier (maybe)

118

Astrocytomas are usually associated with what genetic disorder?

NF-1
and Li-Fraumeni syndrome

119

Why are some childhood CA survivors usually present with astrocytomas?

Radiation to the head and neck

120

What is the genetic defect in Wilm's tumor?

WT1 tumor suppressor gene that is critical for normal renal development

121

What ethnicity has the highest incidence of Wilm's tumor?

AAs

122

What is the peak age incidence of Wilm tumor?

2-3 yo

123

What are the ssx of wilm tumors? (4)

1. hemihypertrophy
2. aniridia
3. Large, palpable abdominal mass
4. HTN/gross hematuria

124

What are the two syndromes that are associated with wilm tumor?

Denys-drash
Beckwideth-Widemann syndrome

125

What is the treatment for Wilm tumor?

surgery

126

What is stage I-V of Wilms tumor?

I = limited to kidney
II = extent beyond kidney
III = Residual tumor in abdo
IV = hematogenous spread
V= both kidneys

127

What are the orders for evaluating Wilms tumor?

CBC
CMP
CT/abdominal US

128

What is the prognosis for Wilm's tumor?

80-90%

129

When does retinoblastomas usually present?

130

What is the cause of retinoblastoma?

Loss of both alleles of the RB gene

131

What is the treatment for RB?

Radiation, chemo, enucleation of eye

132

What are neuroblastomas?

Anywhere there was neural crest cells in tissues

133

True or false: neuroblastomas are not usually metastatic

False

134

What are the ssx of metastatic neuroblastomas? (3)

-Raccoon eyes
-large abdo mass
-Lower extremity weakness (if spinal cord involved)

135

What is Horner's syndrome?

Miosis
Ptosis
Anhydrosis

136

What is the most common extracranial solid tumor in children?

Neuroblastomas

137

What are the three poor prognostic indicators in neuroblastomas?

age

138

How do you evaluate neuroblastomas?

Urine Catecholamine
HVA VMA
DXA scan

139

What are rhabdomyosarcomas?

CAs from undifferentiated mesenchymal cells that differentiate into muscle

140

What are the ssx of rhabdomyosarcoma?

depend on age and site

141

What are the ssx of head/neck rhabdomyosarcomas?

proptosis
Periorbital swelling

142

What are the ssx of paramenigneal rhabdomyosarcomas?

CN palsy
sinus drainage

143

What are the ssx of GU rhabdomyosarcomas?

hematuria
Urinary obstruction

144

What are the ages where rhabdomyosarcomas usually peak?

2-6 yo

145

What are Ewing's sarcomas?

small, round blue cell tumors that present with pain, swelling,

146

What is the treatment for Ewing's sarcomas?

methotrexate
Doxorubicin
Cisplatin

147

Are ewing's tumors radiosensitive?

No

148

Osteogenic sarcomas usually are found where?

long bones