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Hematology > CHildhood CA > Flashcards

CHildhood CA Flashcards

1
Q

How many children are diagnosed with CA each day?

A

46

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2
Q

What is the death rate of cancer in children?

A

> 3000

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3
Q

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

A

Cancer

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4
Q

What is the most common cause of death ages 104?

A

Congenital abnormalities (CA second)

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5
Q

What is the average age at diagnosis for childhood cancer?

A

6

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6
Q

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

A

15 in adult, 71 in children

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7
Q

What is the funding like for childhood cancer?

A

Very small

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8
Q

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

A

1:750

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9
Q

What is the most common type of childhood cancer?

A

leukemia

ALL

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10
Q

What is the second most common CA in children?

A

CNS

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11
Q

What is WIlm’s tumor?

A
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12
Q

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

A

Hodgkin’s lymphoma

Thyroid carcinoma

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13
Q

What is happening to the mortality rate of childhood cancer?

A

Lower

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14
Q

Survival is greatest for what childhood cancer? Second?

A

Wilm’s tumor

ALL

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15
Q

What is the children’s oncology group?

A

Cooperative of Drs that treat childhood CA

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16
Q

What are the causes of childhood cancer?

A

Largely Unknown–environmental causes have been difficult to identify

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17
Q

What is neurofibromatosis?

A
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18
Q

What is familial polyposis?

A
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19
Q

What is Li-Fraumeni syndrome?

A

p53 mutation

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20
Q

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

A
  • Immunodeficiency
  • Metabolic disorders
  • Disorders of chromosome stability
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21
Q

What are the ssx of childhood CA?

A

Nonspecific, and often mimic childhood problems

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22
Q

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

A

3

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23
Q

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

A

CXR
abd US

Renal or abdominal tumors (NBL)

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24
Q

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

A

Abd US

Any malignancy

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25
Q

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

A

CBC, manual diff

Leukemia/NBL

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26
Q

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

A

surgical consult
CXR
CBC, manual diff

Leukemia/lymphoma

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27
Q

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?

A

Hormonal assays
CT hypothalamic areas
Abd CT

Pituitary tumors
Hypothalamic tumors
Gonadal tumors
Adrenal tumors

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28
Q

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?

A

Neuro/surgery consult

Brain tumor

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29
Q

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?

A

Opthalmological consult

Rb, or metastatic neuroblastoma

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30
Q

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

A

CBC, diff, imaging

LCH, RMS

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31
Q

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

A

CBC/diff
Imaging

mediastinal masses

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32
Q

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

A

CBC/diff, imaging

RSM, lymphoma, nasopharyngeal carcinomas

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33
Q

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

A

Dental consult

LCH, burkitt’s lymphoma

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34
Q

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

A

CBC/diff, imaging

Soft tissues tumors, mediastinal tumors

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35
Q

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

A

abd US, CBC

Wilms tumor
neuroblastoma
Hepatocellular carcinoma

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36
Q

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

A

UA, CBC, US

Germ cell tumors

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37
Q

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

A

CBC, x-rays

Osteosarcoma
Ewig’s sarcoma
Neuroblastoma

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38
Q

What is Ewing’s sarcoma?

A
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39
Q

What are the ssx of leukemia? (6)

A
  • fever 101F >7 days
  • petechiae
  • unexplained anemia/pallor
  • Generalized lymphadenopathy
  • HSM
  • bone or joint pain
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40
Q

What are the ssx of brain tumors?

A

Szs, weakness, coordination difficulties

Headaches and vomiting

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41
Q

Why are headaches and vomiting common in childhood brain tumors?

A

Compression of CSF circulation

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42
Q

When are headaches concerning for brain tumors?

A

Changing character

Persistent

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43
Q

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

A

Brain tumor

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44
Q

What size are lymph nodes considered “large”?

A

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

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45
Q

What are the two common bacterial causes of lymphadenopathy?

A

Cat scratch disease

Staph and strep

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46
Q

What are the common viruses that cause lymphadenopathy?

A

EBV and herpes

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47
Q

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

A
  • Generalized

- Regional not involving the head to neck

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48
Q

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

A

posterior auricular
Epitrochlear
Mediastinal

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49
Q

When is a biopsy of a node recommended?

A

Enlarging after 2-3 weeks of abx

unchanged 6-8 weeks

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50
Q

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

A

True

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51
Q

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

A

Abdominal, thoracic soft tissue

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52
Q

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

A

False–late symptom

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53
Q

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

A

30%

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54
Q

Ostalgia caused by CA is usually confused with what?

A

RA

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55
Q

When is bone/joint pain particularly concerning?

A

Persistent
Associated with swelling or mass
LROM

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56
Q

How do you differentiate ITP from malignancy?

A

Low platelets, ITP is usually preceded by a viral infection

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57
Q

What is the most common malignancy in childhood?

A

ALL

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58
Q

What is the peak age for ALL?

A

2-5 years

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59
Q

ALL accounts for what fraction of all childhood cancers?

A

1/3

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60
Q

In what race is ALL most common?

A

caucasians

61
Q

What sex has more cases of ALL?

A

Males

62
Q

What is the pathogenesis of ALL?

A

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

63
Q

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

A

Flow cytometry

64
Q

Where does ALL arise from?

A

Bone marrow, but may present anywhere in the body

65
Q

What are the ssx of ALL? (4)

A

anemia
Decreased platelets
Neutropenia
Ostalgia

66
Q

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

A
  • Lymphadenopathy/HSM
  • Orthopnea/cough
  • facial nerve palsy
  • Testicular enlargement
  • Gingival hypertrophy
67
Q

What percent of ALL pts have leukocytosis?

A

50%

68
Q

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

A

80%

69
Q

What percent of ALL pts have >2 cytopenias?

A

95%

70
Q

What percent of ALL pts will have 1 cytopenia?

A

4%

71
Q

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

A

1%

72
Q

Why do you want a manual differential when diagnosing ALL?

A

May mistake cells

73
Q

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

A
  1. EBV
  2. Parvovirus B19
  3. Acute anemia
  4. ITP
74
Q

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

A

Hispanic

75
Q

Children with what disease have an increased risk of ALL?

A

Down syndrome

76
Q

The primary nongenetic risk factor for developing ALL is what?

A

prenatal exposure to x-rays

77
Q

What type of cell is usually implicated in ALL?

A

B cells

78
Q

What is the usual presentation of B-ALL?

A

BM involvement, but frequently CNS, gonads, lymph nodes

79
Q

What percent of children have T-ALL?

A

15%

80
Q

Who is usually afflicted with T-ALL?

A

Male adolescence

81
Q

How does T-ALL usually present?

A

Looks like mumps with mediastinal mass and leukocytosis

82
Q

What is the standard risk for ALL?

A

Age 1-10 yo

WBC

83
Q

What is the high risk standard for ALL?

A

Age 10 yrs

WBC >50

84
Q

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

A

Precursor T

Mature B

85
Q

What is the greatest predictor of overall outcome of ALL?

A

Response to therapy in the first few days

86
Q

What is the maintenance period for males? Females?

A

Males = 3.5 years

Females == 2.5 years

87
Q

CNS prophylaxis is indicated in what types of ALL?

A

T cell or with CNS involvement

88
Q

What are the f/u periods for ALL?

A

5 years after chemo, then every 10 years

89
Q

What percent of ALL pts attain remission?

A

95%

90
Q

What is the infection that ALL pts are predisposed to?

A

PCP pneumonia

91
Q

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

A

75-85%

92
Q

What are the four late effects of ALL?

A
  1. Osteonecrosis/osteopenia
  2. Neuropathies
  3. Neuropsychometric concerns
  4. Obesity
93
Q

What is the side effect of vincristine use?

A

Neuropathy

94
Q

What is the MOA of vincristine?

A

Inhibits tubule formation/breakdown

95
Q

What is the treatment for AML?

A

Cytarabine (ara-b)

Daunorubicin

96
Q

What are the three prognostic indicators for AML?

A

age
race
Cytogenetics

97
Q

What is the cure for AML?

A

Bone marrow transplant

98
Q

What are the side effects of daunorubicin?

A

LVH

***cardiomyopathy

99
Q

What is the most common SOLID tumor of childhood?

A

Brain tumors

100
Q

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

A

Astrocytoma

101
Q

Astrocytomas are usually associated with what genetic disorder?

A

Neurofibromatosis

102
Q

What is the treatment for astrocytomas?

A

Brain surgery

103
Q

What are the ssx of increased intracrainial pressure?

A

Papilledema
Loss of vision
Bulging fontanelles/spreading sutures

104
Q

What are the ssx of posterior fossa and brainstem?

A 
ataxia
tremors
dysarthria
stiff neck
CN signs
105
Q

What are the ssx of hemispheric tumors?

A

Hemiparesis or hemianopsia

106
Q

What are the three ways to diagnose brain tumors?

A

CSF
Imaging
Biopsy

107
Q

Surgery, XRT, or chemo for: medulloblastoma?

A

surgery and chemo

108
Q

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

A

surgery and focal XRT

109
Q

Surgery, XRT, or chemo for: optic glioma?

A

NOT surgery

110
Q

Surgery, XRT, or chemo for: ependymomas?

A

Surgery XRT, and focal chemo

111
Q

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

A

XRT and chemo

112
Q

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

A

surgery

113
Q

What are the two exceptions of surgery for brain tumors?

A

GCT BSG

114
Q

What is the single best treatment for brain tumors?

A

Surgery

115
Q

What is neuroaxis prophylaxis?

A

treating neuro axis via CSF

116
Q

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

A

True

117
Q

What is the limiting factor for chemo in brain tumor?

A

Blood brain barrier (maybe)

118
Q

Astrocytomas are usually associated with what genetic disorder?

A

NF-1

and Li-Fraumeni syndrome

119
Q

Why are some childhood CA survivors usually present with astrocytomas?

A

Radiation to the head and neck

120
Q

What is the genetic defect in Wilm’s tumor?

A

WT1 tumor suppressor gene that is critical for normal renal development

121
Q

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

A

AAs

122
Q

What is the peak age incidence of Wilm tumor?

A

2-3 yo

123
Q

What are the ssx of wilm tumors? (4)

A
  1. hemihypertrophy
  2. aniridia
  3. Large, palpable abdominal mass
  4. HTN/gross hematuria
124
Q

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

A

Denys-drash

Beckwideth-Widemann syndrome

125
Q

What is the treatment for Wilm tumor?

A

surgery

126
Q

What is stage I-V of Wilms tumor?

A 
I = limited to kidney
II = extent beyond kidney
III = Residual tumor in abdo
IV = hematogenous spread
V= both kidneys
127
Q

What are the orders for evaluating Wilms tumor?

A

CBC
CMP
CT/abdominal US

128
Q

What is the prognosis for Wilm’s tumor?

A

80-90%

129
Q

When does retinoblastomas usually present?

A
130
Q

What is the cause of retinoblastoma?

A

Loss of both alleles of the RB gene

131
Q

What is the treatment for RB?

A

Radiation, chemo, enucleation of eye

132
Q

What are neuroblastomas?

A

Anywhere there was neural crest cells in tissues

133
Q

True or false: neuroblastomas are not usually metastatic

A

False

134
Q

What are the ssx of metastatic neuroblastomas? (3)

A
  • Raccoon eyes
  • large abdo mass
  • Lower extremity weakness (if spinal cord involved)
135
Q

What is Horner’s syndrome?

A

Miosis
Ptosis
Anhydrosis

136
Q

What is the most common extracranial solid tumor in children?

A

Neuroblastomas

137
Q

What are the three poor prognostic indicators in neuroblastomas?

A

age

138
Q

How do you evaluate neuroblastomas?

A

Urine Catecholamine
HVA VMA
DXA scan

139
Q

What are rhabdomyosarcomas?

A

CAs from undifferentiated mesenchymal cells that differentiate into muscle

140
Q

What are the ssx of rhabdomyosarcoma?

A

depend on age and site

141
Q

What are the ssx of head/neck rhabdomyosarcomas?

A

proptosis

Periorbital swelling

142
Q

What are the ssx of paramenigneal rhabdomyosarcomas?

A

CN palsy

sinus drainage

143
Q

What are the ssx of GU rhabdomyosarcomas?

A

hematuria

Urinary obstruction

144
Q

What are the ages where rhabdomyosarcomas usually peak?

A

2-6 yo

145
Q

What are Ewing’s sarcomas?

A

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

146
Q

What is the treatment for Ewing’s sarcomas?

A

methotrexate
Doxorubicin
Cisplatin

147
Q

Are ewing’s tumors radiosensitive?

A

No

148
Q

Osteogenic sarcomas usually are found where?

A

long bones

Hematology (32 decks)

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