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Simmons NURP 503 Exam 3 > 9: Peds Cancer > Flashcards

9: Peds Cancer Flashcards

1
Q

What is the most common cancer in 10-14 yo (3)?

A
  1. Acute leukemia (21%)
  2. Brain and CNS (21%)
  3. Lymphoma (21%)
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2
Q

What meds are used during induction phase of ALL treatment (3)?

A
  1. Vincristine
  2. Prednisone
  3. L-asparaginase
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3
Q

Risk factors for cancer in children (6)?

A
  1. Prenatal X-ray
  2. DES exposure
  3. Transplacental transmission of maternal cancers
  4. Radiation and chemo (secondary cancers)
  5. Viral exposure (EBV)
  6. In utero expsoure: meds, pesticides, EMFs, motor vehicle exhaust (uncertain risk)
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4
Q

3 classification systems of non-Hodgkin lymphoma.

A
  1. Lymphoblastic lymphoma
  2. Small, noncleaved cell lymphoma (Burkitt, non-Burkitt, B-cell)
  3. Large cell lymphoma
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5
Q

According to the St. Jude system, is this NHL stage 1, 2, 3, or 4?
On the same side of the diaphragm, 2 or more nodal areas, or 2 single (extranodal) tumors with or without regional node involvement.

A

2

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6
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?

Localized tumor with complete gross excision, microscopic residual disease, or both.

A

1

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7
Q

Is this a sign of anemia, thrombocytopenia, or neutropenia?

Petechiae, bleeding, purpura.

A

Thrombocytopenia

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8
Q

What imaging is ordered with Wilms tumor (4)?

A
  1. CXR (4-field)
  2. Renal U/S
  3. Abd CT
  4. Abd MRI (caval patency)
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9
Q

Stains used of biochemical markers.

A

Cytochemistry

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10
Q

T/F The prognosis of children with HL is similar to that of adults with HL.

A

True

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11
Q

T/F With osteosarcoma, there can be a tender, warm, palpable mass. But signs are indistinguishable from those of osteomyelitis.

A

True

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12
Q

Children’s oncology stage 1, 2, 3, 4, or 5 for Wilms tumor?
Residual, nonhematogenous tumor present following surgery and confined to abdomen.
Positive lymph nodes in abdomen or pelvis.
Penetration through peritoneal surface.
Peritoneal implants present.
Gross or microscopic tumor remains postoperatively, including positive margins of resection.
Tumor spillage is noted, including biopsy.
Tumor treated with preoperative chemotherapy.
Tumor removed in more than 1 piece.

A

3

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13
Q

Stage 4S neuroblastoma has small primary tumors and metastatic disease confined to liver, skin, and bone marrow. If it develops in neonates, it can have skin lesions that are confused with _____. Severe skin involvement is termed _____ baby.

A

Congenital rubella

Blueberry muffin baby

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14
Q

Maintenance therapy is targeted at eliminating _____ disease.

A

Residual

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15
Q

Which marker has the worst prognosis in neuroblastoma?

A

MYCN amplification tend to have rapid tumor progression and poor prognosis.

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16
Q

Treatment for Ewing sarcoma (3).

A
  1. Chemo
  2. Radiation
  3. Surgery
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17
Q

What is the most common site of origin in NHL?

A

Lymphoid structures of the intestinal tract.

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18
Q

What are B symptoms of prognostic significance in HL (3)?

A
  1. Unexplained fever above 38 C for 3 days.
  2. Unexplained weight loss of 10% or more in previous 6 months.
  3. Drenching night sweats.
    (Ann Arbor uses A and B with stages of 1-4 to indicate presence or absence of these symptoms)
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19
Q

Why might there be weakness, limping, paralysis, and bladder/bowel dysfunction in neuroblastoma?

A

Symptoms arise from tumors of the paraspinal sympathetic that grow through the spinal foramina into the spinal canal and impinge on the spinal cord.

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20
Q

According to the Ann Arbor system, is this HL stage 1, 2, 3, or 4?
Diffuse or disseminated involvement of one or more extralymphatic organs (liver, bone marrow, lung) or tissues with or without associated lymph node involvement (spleen considered a nodal site).

A

4

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21
Q

Labwork in neuroblastoma (5)?

A
  1. CBC
  2. CMP
  3. UA
  4. Coags
  5. LDH (Lactate dehydrogenase)
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22
Q

Favorable or unfavorable Shimada classification of neuroblastoma?
Younger than 18 months with stroma-poor tumors, an MKI <200/5000, and differentiated or undifferentiated neuroblasts.

A

Favorable

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23
Q

Why do pruritis, urticaria and fatigue occur in HL?

A

Cytokines produced by HL-RSC or supporting environment within the affected lymph nodes.

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24
Q

Is this a sign of anemia, thrombocytopenia, or neutropenia?

Fatigue, pallor.

A

Anemia

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25
Q

Is this a sign of anemia, thrombocytopenia, or neutropenia?

Fever, recurrent infections.

A

Neutropenia

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26
Q

What are the median age at diagnosis and peak age (unilaterla and bilateral) for Wilms tumor?

A 
Median = 3.5 years
Peak = 2-3 years unilateral
Peak = <3 bilateral
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27
Q

How does EBV exposure increase risk of Non-Hodgkin Lymphoma?

A

Causes B-cell proliferation and in vitro immortalization.

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28
Q

What gene?

Normal role in cell division and growth. If mutated, they become carcinogenic.

A

Proto-Oncogenes

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29
Q

Adults or Peds?

Less difficulty with acute toxicities.

A

Peds

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30
Q

Common physical NHL findings:

Firm, fixed, and nontender cervical or _____ masses or adenopathy.

A

Supraclavicular

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31
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?

Limited to infants.

A

4S

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32
Q

T/F In HL, treatment strategies focus on reducing late effects of therapy while maintaining excellent cure rates with risk-adapted chemotherapy alone or response-adjusted combined-modality regimens.

A

True

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33
Q

What gene?

“Parents” gene. When damaged or missing, cell ignores inhibitory signals and grows out of control.

A

Tumor Suppressor Genes

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34
Q

Labwork in osteosarcoma (4)?

A
  1. CBC
  2. CMP
  3. LDH/Alkaline Phosphatase (prognostic significance)
  4. UA
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35
Q

How is a thoracic neuroblastoma usually diagnosed?

A

Usually diagnosed by imaging studies obtained for other reasons. Symptoms of mild airway obstruction or cough lead to CXR.

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36
Q

Common physical NHL findings:

Thoracic dullness to percussion indicate _____.

A

Pleural effusion

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37
Q

According to the St. Jude system, is this NHL stage 1, 2, 3, or 4?
On both sides of the diaphragm, 2 or more nodal areas, or 2 single (extranodal) tumors with or without regional node involvement.

A

3

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38
Q

T/F Combined modality (chemo + radiation) is the preferred approach for peds with HL.

A

True

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39
Q

According to the Ann Arbor system, is this HL stage 1, 2, 3, or 4?
Single lymph node region or single extranodal site.

A

1

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40
Q

Children’s oncology stage 1, 2, 3, 4, or 5 for Wilms tumor?

Bilateral renal involvement by tumor present at diagnosis.

A

5

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41
Q

What causes HTN in Wilms tumor?

A

Renin secretion by tumor cells or compression of renal vasculature by tumor.

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42
Q

Common physical NHL findings:

Dyspnea or stridor indicates _____.

A

Mediastinal mass

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43
Q

What test is used to r/o CNS involvement in ALL?

A

Lumbar Puncture

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44
Q

A large mediastinal shift indicates _____.

A

Superior vena cava syndrome

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45
Q

What are the 2 peaks for HL?

A
  1. 25

2. 50-60

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46
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?

Any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, liver, skin, and/or other organs.

A

4

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47
Q

Which site has an intermediate prognosis in osteosarcoma?

A

Distal femur

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48
Q

What are uncommon findings with NHL (4)?

A
  1. Nasopharyngeal mass
  2. Parotid enlargement
  3. Nephromegaly
  4. Testicular enlargement
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49
Q

According to the St. Jude system, is this NHL stage 1, 2, 3, or 4?
Any other involvement with initial CNS or marrow (< 25%) involvement.

A

4

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50
Q

Common physical NHL findings:

Abdominal distention or mass with or without tenderness, rebound tenderness, and/or shifting _____.

A

Dullness

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51
Q

When is NHL most common?

A

2nd decade of life. Peaks at 5-15 yo.

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52
Q

According to the Ann Arbor system, is this HL stage 1, 2, 3, or 4?
Lymph node regions on both sides of the diaphragm.

A

3

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53
Q

T/F Orbital inflammation implies extraocular extension in retinoblastoma.

A

False. Eyes have necrotic tumors, but not necessarily extraocular extension.

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54
Q

What is the most common cancer in 5-9 yo (2)?

A
  1. Acute leukemia (32%)

2. Brain and CNS (28%)

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55
Q

Stage 1, 2, 3, A, or B for osteosarcoma?

Metastatic disease.

A

3

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56
Q

This is a HALLMARK finding of Hodgkin Lymphoma (HL).

A

Reed-Sternberg Cells

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57
Q

This is associated with Wilms tumor and includes aniridia, GU abnormalities, and mental retardation.

A

WAGR

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58
Q

What is the greatest predictor of death in retinoblastoma?

A

Extraocular extension either directly through the sclera or via extension along the optic nerve.

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59
Q

Treatment for osteosarcoma (3)?

A
  1. Resection (essential for cure)
  2. Presurgical chemo (shrinking of tumors and assessment of responsiveness of tumor)
  3. Postsurgical chemo
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60
Q

What gene?

Cause the cell cycle to go out of control.

A

Oncogenes

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61
Q

How does previous exposure to malaria increase risk of Non-Hodgkin Lymphoma?

A

Resultant T-cell suppression and EBV.

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62
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?

Enlarged contralateral lymph nodes, which are negative for tumor microscopically.

A

2B

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63
Q

Where is the mutation in retinoblastoma?

A

13q14

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64
Q

Favorable or unfavorable Shimada classification of neuroblastoma?
Older than 60 months stroma-poor, differentiated neuroblasts, and an MKI less than 100.

A

Unfavorable

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65
Q

T/F Fundoscopic exam must be performed in all cases of childhood strabismus.

A

True. Strabismus can be the result of visual loss and could indicate retinoblastoma.

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66
Q

T/F Chemo is followed by radiation to the primary site of the lesion in osteosarcoma.

A

False. No radiation d/t high level of resistance.

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67
Q

T/F Treatment for NHL is similar to that for ALL.

A

True. Therapies are longer and less intensive than those for small noncleaved cell lymphomas or LCL, which use relatively high doses of alkylating agents and antimetabolites.

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68
Q

What gene?
Ensure DNA strands are copied correctly during cell division. Change causes accumulation of mutations in critical growth-regulating genes.

A

DNA Repair Genes

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69
Q

Favorable or unfavorable Shimada classification of neuroblastoma?
Older than 18 months with stroma-poor tumors, differentiated neuroblasts, and an MKI of 100-200/5000.

A

Unfavorable

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70
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?
Localized primary tumor (as defined for stages 1, 2A, or 2B) with dissemination limited to skin, liver, and/or bone marrow (< 10% involvement).

A

4S

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71
Q

Group of malignant hematologic diseases where normal bone marrow elements are replaced by abnormal, poorly differentiated lymphocytes (i.e., blast cells).

A

Acute Lymphoblastic Leukemia (ALL)

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72
Q

Which cells are usually involved in non-Hodgkin lymphoma (3)?

A
  1. T cells
  2. B cells
  3. Indeterminate lymphocyte cells
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73
Q

Children’s oncology stage 1, 2, 3, 4, or 5 for Wilms tumor?
Tumor completely resected.
No evidence of tumor at or beyond margins of resection.
Tumor extends beyond kidney (penetration of renal capsule, involvement of renal sinus).

A

2

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74
Q

Stage 1, 2, 3, A, or B for osteosarcoma?

Low-grade lesions.

A

1

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75
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?

Unresectable unilateral tumor infiltrating across the midline, regional lymph node involvement, or both.

A

3

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76
Q

Is Wilms tumor more common in AA or Caucasian children?

A

AA

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77
Q

In NHL, what labs would you get if the patient had a fever or evidence of sepsis (3)?

A
  1. PT/PTT
  2. Fibrinogen
  3. D-Dimer
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78
Q

Which site has the best prognosis in osteosarcoma?

A

Distal extremity

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79
Q

Why does treatment of Hodgkin Lymphoma increase risk for Non-Hodgkin Lymphoma?

A

Combined effects of chemo and radiation with immunosuppression.

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80
Q

Labwork for retinoblastoma (5).

A
  1. CBC
  2. CMP
  3. LDH
  4. UA
  5. Blood specimens for DNA analysis (patients, parents, siblings)
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81
Q

How is response to treatment measured in NHL?

A

PET/CT

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82
Q

What is the mainstay of therapy for osteosarcoma?

A

Removal of the lesion. Chemo is required to treat micrometastatic disease which is present but not detectable in most patients at diagnosis.

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83
Q

Common physical NHL findings:

Decreased breath sounds indicate _____ or _____.

A

Bronchial obstruction or pleural effusion

84
Q

Where is the lymphadenopathy in HL?

A

Usually cervical (70-80%)

85
Q

This is ordered for classification and staging of cancer.

A

Biopsy

86
Q

Risk factors for non-Hodgkin lymphoma (8).

A
  1. Certain HLA types + certain blood types
  2. Pesticide exposure
  3. High birth weight
  4. Immunosuppression
  5. EBV
  6. Successful Hodgkin treatment
  7. Previous malaria exposure
  8. Genetics
87
Q

What is MIBG?

A

Compound that accumulates in catecholaminergic cells. Provides specific way of identifying primary and metastatic disease if present in neuroblastoma.

88
Q

This cancer is staged with the St. Jude system.

A

Non-Hodgkin Lymphoma (NHL)

89
Q

What does urine show in neuroblastoma?

A

Elevated catecholamines detectable in urine. Specifically, HVA and VMA levels.

90
Q

What is the survival rate for Wilms tumor?

A

80-90%

91
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?

Localized tumor with incomplete gross resection.

A

2A

92
Q

Adult cancer locations commonly include breast, colon, lung, prostate. Peds is _____, _____, and _____.

A

CNS
Muscle
Bone

93
Q

T/F There are links between viral exposure, such as EBV, and childhood ALL.

A

False. Cause remains largely unknown. A few cases associated with inherited genetic syndromes and congenital immunodeficiencies. Environmental risk factors have not been shown to cause ALL. No direct link established with viral exposure.

94
Q

Favorable or unfavorable Shimada classification of neuroblastoma?
Any age with stroma-rich tumors and a nodular pattern.

A

Unfavorable

95
Q

Why are lumbar puncture and bone marrow aspiration and biopsy performed in retinoblastoma?

A

Useful in early diagnosis of distant metastasis.

96
Q

Appearance of cells under microscope.

A

Morphology

97
Q

Adults or Peds?

Fewer long-term consequences.

A

Adults

98
Q

Imaging studies in Ewing sarcoma (3).

A
  1. X-rays
  2. MRI (extent of disease)
  3. CT scan (bony involvement)
99
Q

Stage 1, 2, 3, A, or B for osteosarcoma?

Intramedullary lesion.

A

A

100
Q

In neuroblastoma, thoracic tumors extending to the neck are known as _____.

A

Horner syndrome

101
Q

If ALL is T-lineage, what might you find?

A

Mediastinal mass that causes respiratory distress and stridor.

102
Q

How common is it for both kidneys to be affected or for them to be affected one right after the other in Wilms tumor?

A

5-10%

103
Q

Favorable or unfavorable Shimada classification of neuroblastoma?
Younger than 60 months with stroma-poor tumors, and MKI < 100/5000, and well-differentiated tumor cells.

A

Favorable

104
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?

Ipsilateral lymph nodes negative for tumor (Nodes attached to the primary tumor may be positive for tumor).

A

1

105
Q

What is the most common cancer in 15-19 yo (2)?

A

Lymphoma (26%)

Carcinoma (21%)

106
Q

Adults or Peds?

More long-term consequences.

A

Peds

107
Q

When would you get an echocardiogram in NHL?

A

To obtain baseline findings before chemotherapy with anthracyclines (cardiomyopathy).

108
Q

Favorable or unfavorable Shimada classification of neuroblastoma?
Any age with stroma-poor tumors, undifferentiated or differentiated neuroblasts, and an MKI more than 200/5000.

A

Unfavorable

109
Q

What is the most common way to diagnose Wilms tumor?

A

After incidental detection of asymptomatic abdominal mass.

110
Q

What labs should be ordered for general diagnostic workup in cancer (4)?

A
  1. CBC
  2. Electrolytes
  3. LFTs
  4. Renal FTs
111
Q

What is the classic triad in Horner syndrome?

A
  1. Miosis (constricted pupils)
  2. Partial ptosis
  3. Loss of hemifacial sweating
112
Q

T/F Lymphadenopathy is common in osteosarcoma.

A

False

113
Q

Adults or Peds?

Tolerate higher doses of meds.

A

Peds

114
Q

What tests should be ordered for general diagnostic workup in cancer (2)?

A
  1. Biopsy

2. Bone Marrow Aspirate/Biopsy

115
Q

What are the 3 phases of treatment for ALL?

A
  1. Induction Phase
  2. Intensification (Consolidation) Phase
  3. Maintenance (Continuation) Therapy
116
Q

If the spermatic vein is obstructed in Wilms tumor, what may be noted?

A

Left varicocele

117
Q

The histology of adult cancer is more epithelial/organ. Peds is _____.

A

Nonepithelial (leukemia, CNS, sarcomas)

118
Q

If there is testicular involvement with ALL, what might you find?

A

Unilateral, painless testicular enlargement.

119
Q

What imaging should be ordered for general diagnostic workup in cancer (4)?

A
  1. X-rays
  2. CT
  3. MRI
  4. Bone scan
120
Q

ALL peaks in children aged _____ years old and subsequently decreases with age.

A

2-6

121
Q

According to the St. Jude system, is this NHL stage 1, 2, 3, or 4?
Single extranodal tumor with regional node involvement; primary gastrointestinal (GI) tumor with or without associated involvement of mesenteric nodes, with gross total resection.

A

2

122
Q

General clinical manifestations of cancers in children (7).

A
  1. Unusual mass or swelling
  2. Unexplained paleness or loss of energy
  3. Sudden tendency to bruise
  4. Persistent, localized pain or limping
  5. Changes in coordination or behavior
  6. Prolonged, unexplained fever or illness
  7. Frequent headaches, often with vomiting
123
Q

_____ pain may indicate a paraspinal, retroperitoneal, or deep pelvic Ewing sarcoma.

A

Back

124
Q

Favorable or unfavorable Shimada classification of neuroblastoma?
Older than 18 months with stroma-poor tumors, undifferentiated neuroblasts, and an MKI more than 100/5000.

A

Unfavorable

125
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?

Localized tumor, complete gross excision, or both with ipsilateral nonadherent lymph nodes positive for tumor.

A

2B

126
Q

In NHL, small noncleaved cell involvement leads to _____ tumors.

A

Abdominal

127
Q

When is radiation therapy used in NHL?

A

Mediastinal or CNS involvement

128
Q

HTN in neuroblastoma is caused by _____, not catecholamine excess.

A

Renal artery compression

129
Q

What is the duration of symptoms before diagnosis in NHL?

A

Generally 1 month or less.

130
Q

Which version of ALL has the most pronounced male predominance?

A

T-cell ALL

131
Q

Why is there abdominal pain, fever, anemia, and hypotension in Wilms tumor?

A

Hemorrhage into the tumor.

132
Q

Common physical NHL findings:

Obtundation, agitation, and meningismus indicate _____.

A

CNS involvement

133
Q

What is the most common cancer in <5 yo?

A

Acute leukemia (35%)

134
Q

Does ALL affect black or white children more?

A

White

135
Q 
Children's oncology stage 1, 2, 3, 4, or 5 for Wilms tumor?
Hematogenous metastases (eg, lung, liver, bone, brain) or lymph node metastases beyond the abdomen or pelvis.
A

4

136
Q

T/F Blood studies provide pathognomic or suggestive results to diagnose Ewing sarcoma.

A

False. Blood tests may be helpful in evaluating other diagnoses (Blood cultures, CRP, CBC, LDH, ESR).

137
Q

According to the St. Jude system, is this NHL stage 1, 2, 3, or 4?
Single extranodal tumor or single anatomic area (nodal), excluding the mediastinum or abdomen.

A

1

138
Q

What is considered elevated with VMA/HVA in neuroblastoma?

A

3 standard deviations higher than age-related reference range levels.

139
Q

T/F With ALL, specific translocation of chromosomes on cells can affect prognosis.

A

True

140
Q

Adults or Peds?

More difficulty with acute toxicities.

A

Adults

141
Q

T/F Clinically significant cytopenias are common in NHL at diagnosis.

A

False. Clinically significant cytopenias are uncommon in NHL.

142
Q

Expression of cell surface antigens.

A

Immunophenotype

143
Q

S/Sx of ALL (7).

A
  1. Anemia (fatigue/pallor)
  2. Thrombocytopenia (petechiae, bleeding, purpura)
  3. Neutropenia (fever, recurrent infections)
  4. Bone pain
  5. Limp
  6. Lymphadenopathy
  7. Hepatosplenomegaly
144
Q

Children with more than _____ chromosomes (hyperdiploid) on cells have the best prognosis in ALL.

A

50

145
Q

Undifferentiated neuroblastomas histologically present as small, round, blue cell tumors with dense nests of cells in a fibrovascular matrix and _____.

A

Home-Wright pseudorosettes

146
Q

This is associated with Wilms tumor and includes unusual facies, islet cell hypertrophy, macrosomia, and hamartomas.

A

Perlman Syndrome

147
Q

What kind of CXR is needed in Wilms tumor?

A

4-field

148
Q

Labwork for Wilms tumor (5)?

A
  1. CBC
  2. CMP
  3. UA
  4. Coags
  5. Cytogenetics (1p and 16 q deletion may reveal 11p13 and 11p15 issues)
149
Q

Where is the most common site for osteosarcoma?

A

Femur (distal) followed by tibia (proximal) and humerus (proximal).

150
Q

In retinoblastoma, glaucoma, retinal detachment, and inflammation are signs of _____.

A

Tumor necrosis

151
Q

Common physical NHL findings:

Distended neck veins and plethora indicate _____.

A

Superior vena cava syndrome

152
Q

What labs do you order in HL (6)?

A
  1. CBC (anemia, elevated or depressed leukocytes or platelets)
  2. ESR (elevated)
  3. CRP (elevated)
  4. Serum copper and ferritin
  5. LFTs
  6. UA (proteinuria)
153
Q

An area where it is difficult to get enough concentration of chemo to kill leukemia cells.

A

Sanctuary sites

154
Q

Why is ALL treatment primarily based on chemo?

A

Systemic disease

155
Q

Genetic abnormalities in number or structure of chromosomes.

A

Chomosomal Analysis (Cytogenetics)

156
Q

The pathogenesis of adult cancer is environmental and lifestyle factors. Peds has a _____.

A

Genetic role

157
Q

T/F It is important to carefully evaluate all lymph node stations and for involvement of liver, spleen, and tonsillar tissue in HL.

A

True. Important in evaluation of response to treatment.

158
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?

Localized unilateral tumor with contralateral regional lymph node involvement.

A

3

159
Q

In NHL, lymphoblastic involvement leads to _____ tumors.

A

Intrathoracic

160
Q

What are important features of neuroblastoma (5)?

A
  1. Degree of neuroblast differentiation.
  2. +/- Schwannian stromal development (stromal rich or poor).
  3. Index of cellular proliferation (MKI).
  4. Nodular pattern.
  5. Age.
161
Q

Chronic diarrhea in neuroblastoma is secondary to tumor secretion of _____.

A

Vasoactive intestinal peptide

162
Q

What are survival rates for retinoblastoma?

A

86-92%. Rates drop with each decade of life for patients with the genomic mutation.

163
Q

Children’s oncology stage 1, 2, 3, 4, or 5 for Wilms tumor?
Tumor limited to kidney and is completely resected.
Renal capsule intact.
Tumor not ruptured or biopsied prior to removal.
Vessels of renal sinus not involved.
No evidence of tumor present at or beyond margins of resection.

A

1

164
Q

T/F Those with the genomic mutation for retinoblastoma are more likely to develop secondary cancers later in life.

A

True. They also present with either bilateral disease or unilateral multi-focal disease in retinoblastoma.

165
Q

If there is CNS involvement with ALL, what might you find (6)?

A
  1. Headache
  2. Vomiting
  3. Lethargy
  4. Papilledema
  5. Nuchal rigidity
  6. Cranial nerve deficits
166
Q

Who is most often affected by Hodgkin Lymphoma (HL)?

A

Young adults. Occurs in all age groups, but is rare in children younger than 5 yo.

167
Q

Why does pseudouveitis occur in retinoblastoma?

A

Tumor cells invade the retina diffusely without forming a discrete tumor mass.

168
Q

Stage 1, 2, 3, A, or B for osteosarcoma?

High-grade lesions.

A

2

169
Q

According to the St. Jude system, is this NHL stage 1, 2, 3, or 4?
Any primary mediastinal, pleural, or thymic intrathoracic tumor; any extensive and unresectable abdominal tumor; any primary paraspinous or epidural tumor regardless of other sites.

A

3

170
Q

What test is used to r/o testicular infiltration if the testes are enlarged?

A

Ultrasound

171
Q

What are you looking for with the CBC in ALL (3)?

A
  1. Peripheral smear for lymphoblasts
  2. Elevated leukocyte count
  3. Pancytopenia
172
Q

T/F A large number of patients with retinoblastoma (95%) have no previous family hx, even those with the bilateral, hereditary form.

A

True

173
Q

Which site has the worst prognosis in osteosarcoma?

A

Axial skeleton

174
Q

According to the Ann Arbor system, is this HL stage 1, 2, 3, or 4?
Two or more lymph node regions on the same side of the diaphragm.

A

2

175
Q

What is the more common presenting symptom of retinoblastoma in underdeveloped countries.

A

Proptosis

176
Q

Adults or Peds?

Tolerate lower doses of meds.

A

Adults

177
Q

Common physical NHL findings:

Focal pain or swelling in the extremities indicate _____.

A

Primary bone lymphoma

178
Q

This cancer is staged with the Ann Arbor system.

A

Hodgkin Lymphoma (HL)

179
Q

What is the treatment for Wilms tumor that is unilateral and how does it differ for bilateral?

A
  1. Complete nephrectomy
  2. Surgery
  3. Chemo
  4. Radiation
    For bilateral, nephrectomy of more involved site with excision of smaller lesion in remaining kidney. No surgery with radiation.
180
Q

Name 2 sanctuary sites.

A
  1. CNS

2. Testicles

181
Q

Stage 1, 2, 3, A, or B for osteosarcoma?

Local extramedullary spread.

A

B

182
Q

Adults or Peds?

Tumors more responsive to chemotherapy.

A

Peds

183
Q

Imaging studies for retinoblastoma (3).

A
  1. CT (cranial and orbital)
  2. U/S (distinguishes retinoblastoma from non-neoplastic conditions and useful for calcifications)
  3. MRI (degree of differentiation, not as specific as CT b/c of lack of sensitivity in detecting calcium)
184
Q

Imaging studies in osteosarcoma (5)?

A
  1. Plain x-ray
  2. CXR (metastases)
  3. CT (lesion and chest)
  4. MRI (lesion)
  5. Bone scan
185
Q

What is a sign of advanced neuroblastoma?

A

Periorbital ecchymosis (metastatic disease to the orbits).

186
Q

Performed when there is no chance of preserving useful vision in an eye with retinoblastoma.

A

Enucleation

187
Q

What are treatment options for neuroblastoma (4)?

A
  1. Surgery
  2. Radiation
  3. Chemo
  4. Bone marrow transplant (high risk patients)
188
Q

Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?

Representative ipsilateral nonadherent lymph nodes microscopically negative for tumor.

A

2A

189
Q

In NHL, maintenance therapy may be continued for _____.

A

6 months to 2 years

190
Q

S/Sx of paraneoplastic syndrome in neuroblastoma.

A

Opsoclonus (random eye movements and myoclonus)

191
Q

What is the most common sx with osteosarcoma?

A

Pain, particularly with activity. There can be swelling, depending on size and location.

192
Q

Children with down syndrome have a 20x greater chance of developing _____.

A

ALL

193
Q

Favorable or unfavorable Shimada classification of neuroblastoma?
Any age with stroma-rich tumors without a nodular pattern.

A

Favorable

194
Q

T/F Wilms tumor never crosses the midline of the abdomen.

A

False. That may be noted, but not always.

195
Q

What is the prognosis of NHL and HL in:
Stages 1-2?
Stages 3-4?

A 
1-2 = 90%
3-4 = 70%
196
Q

Adults or Peds?

Less responsive to chemo.

A

Adults

197
Q

_____ directed therapy is critical for improved survival rates in ALL and includes intrathecal chemo.

A

CNS

198
Q

What are the 2 most common loci for gene alteration in Wilms tumor (tumor suppressor genes)?

A
  1. 11p13 (WT1)

2. 11p15 (WT2)

199
Q

Is ALL more common in males or females?

A

Males (slightly)

200
Q

Bone marrow metastasis can produce _____ or _____.

A

Petechiae or purpura

201
Q

What is a red flag for anesthesia for diagnostic procedures in HL?

A

Mediastinal adenopathy (SOB, chest pain, cough) can put at risk for respiratory failure.

202
Q

This is ordered to determine the extent of involvement of malignant cells in marrow.

A

Bone Marrow Aspirate/Biopsy

203
Q

What is the major risk with the PE and Wilms tumor?

A

Palpating too vigorously can rupture the tumor and create stage 3 cancer.

204
Q

When is cranial irradiation or intrathecal chemo used in NHL?

A

CNS involvement

205
Q

This is associated with Wilms tumor and includes hemihypertrophy, macroglossia, omphalocele, and GU abnormalities.

A

Beckwith-Wiedemann Syndrome

206
Q

Risk factors for HL (2).

A
  1. Genetics

2. Immunodeficiencies (EBV, CMV)

207
Q

Cancer is classified by cell type involvement and by _____.

A

Cellular differentiation

Simmons NURP 503 Exam 3 (20 decks)

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