CHildhood CA Flashcards
(148 cards)
1
Q
How many children are diagnosed with CA each day?
A
46
2
Q
What is the death rate of cancer in children?
A
> 3000
3
Q
What is the number 1 cause of disease related death for children?
A
Cancer
4
Q
What is the most common cause of death ages 104?
A
Congenital abnormalities (CA second)
5
Q
What is the average age at diagnosis for childhood cancer?
A
6
6
Q
What is the average years of life lost in an adult CA patient? Child?
A
15 in adult, 71 in children
7
Q
What is the funding like for childhood cancer?
A
Very small
8
Q
What is the odds of encountering a 20 year who survived childhood cancer?
A
1:750
9
Q
What is the most common type of childhood cancer?
A
leukemia
ALL
10
Q
What is the second most common CA in children?
A
CNS
11
Q
What is WIlm’s tumor?
A
12
Q
What are the two most common causes of cancer in adolescence?
A
Hodgkin’s lymphoma
Thyroid carcinoma
13
Q
What is happening to the mortality rate of childhood cancer?
A
Lower
14
Q
Survival is greatest for what childhood cancer? Second?
A
Wilm’s tumor
ALL
15
Q
What is the children’s oncology group?
A
Cooperative of Drs that treat childhood CA
16
Q
What are the causes of childhood cancer?
A
Largely Unknown–environmental causes have been difficult to identify
17
Q
What is neurofibromatosis?
A
18
Q
What is familial polyposis?
A
19
Q
What is Li-Fraumeni syndrome?
A
p53 mutation
20
Q
What are the three categories of diseases linked with increased cancer risk?
A
- Immunodeficiency
- Metabolic disorders
- Disorders of chromosome stability
21
Q
What are the ssx of childhood CA?
A
Nonspecific, and often mimic childhood problems
22
Q
How many providers are seen before a diagnosis of childhood cancer is made?
A
3
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)
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
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
