9: Peds Cancer Flashcards
(186 cards)
1
Q
What is the most common cancer in 10-14 yo (3)?
A
- Acute leukemia (21%)
- Brain and CNS (21%)
- Lymphoma (21%)
2
Q
What meds are used during induction phase of ALL treatment (3)?
A
- Vincristine
- Prednisone
- L-asparaginase
3
Q
Risk factors for cancer in children (6)?
A
- Prenatal X-ray
- DES exposure
- Transplacental transmission of maternal cancers
- Radiation and chemo (secondary cancers)
- Viral exposure (EBV)
- In utero expsoure: meds, pesticides, EMFs, motor vehicle exhaust (uncertain risk)
4
Q
3 classification systems of non-Hodgkin lymphoma.
A
- Lymphoblastic lymphoma
- Small, noncleaved cell lymphoma (Burkitt, non-Burkitt, B-cell)
- Large cell lymphoma
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
6
Q
Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?
Localized tumor with complete gross excision, microscopic residual disease, or both.
A
1
7
Q
Is this a sign of anemia, thrombocytopenia, or neutropenia?
Petechiae, bleeding, purpura.
A
Thrombocytopenia
8
Q
What imaging is ordered with Wilms tumor (4)?
A
- CXR (4-field)
- Renal U/S
- Abd CT
- Abd MRI (caval patency)
9
Q
Stains used of biochemical markers.
A
Cytochemistry
10
Q
T/F The prognosis of children with HL is similar to that of adults with HL.
A
True
11
Q
T/F With osteosarcoma, there can be a tender, warm, palpable mass. But signs are indistinguishable from those of osteomyelitis.
A
True
12
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
13
Q
Maintenance therapy is targeted at eliminating _____ disease.
A
Residual
14
Q
Which marker has the worst prognosis in neuroblastoma?
A
MYCN amplification tend to have rapid tumor progression and poor prognosis.
15
Q
Treatment for Ewing sarcoma (3).
A
- Chemo
- Radiation
- Surgery
16
Q
What is the most common site of origin in NHL?
A
Lymphoid structures of the intestinal tract.
17
Q
What are B symptoms of prognostic significance in HL (3)?
A
- Unexplained fever above 38 C for 3 days.
- Unexplained weight loss of 10% or more in previous 6 months.
- Drenching night sweats.
(Ann Arbor uses A and B with stages of 1-4 to indicate presence or absence of these symptoms)
18
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.
19
Q
Labwork in neuroblastoma (5)?
A
- CBC
- CMP
- UA
- Coags
- LDH (Lactate dehydrogenase)
20
Q
Why do pruritis, urticaria and fatigue occur in HL?
A
Cytokines produced by HL-RSC or supporting environment within the affected lymph nodes.
21
Q
Is this a sign of anemia, thrombocytopenia, or neutropenia?
Fatigue, pallor.
A
Anemia
22
Q
Is this a sign of anemia, thrombocytopenia, or neutropenia?
Fever, recurrent infections.
A
Neutropenia
23
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
24
Q
How does EBV exposure increase risk of Non-Hodgkin Lymphoma?
A
Causes B-cell proliferation and in vitro immortalization.
25
What gene?
| Normal role in cell division and growth. If mutated, they become carcinogenic.
Proto-Oncogenes
26
Adults or Peds?
| Less difficulty with acute toxicities.
Peds
27
Common physical NHL findings:
| Firm, fixed, and nontender cervical or _____ masses or adenopathy.
Supraclavicular
28
Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?
| Limited to infants.
4S
29
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.
True
30
What gene?
| "Parents" gene. When damaged or missing, cell ignores inhibitory signals and grows out of control.
Tumor Suppressor Genes
31
Labwork in osteosarcoma (4)?
1. CBC
2. CMP
3. LDH/Alkaline Phosphatase (prognostic significance)
4. UA
32
How is a thoracic neuroblastoma usually diagnosed?
Usually diagnosed by imaging studies obtained for other reasons. Symptoms of mild airway obstruction or cough lead to CXR.
33
Common physical NHL findings:
| Thoracic dullness to percussion indicate _____.
Pleural effusion
34
T/F Combined modality (chemo + radiation) is the preferred approach for peds with HL.
True
35
What causes HTN in Wilms tumor?
Renin secretion by tumor cells or compression of renal vasculature by tumor.
36
Common physical NHL findings:
| Dyspnea or stridor indicates _____.
Mediastinal mass
37
What test is used to r/o CNS involvement in ALL?
Lumbar Puncture
38
A large mediastinal shift indicates _____.
Superior vena cava syndrome
39
What are the 2 peaks for HL?
1. 25
| 2. 50-60
40
Which site has an intermediate prognosis in osteosarcoma?
Distal femur
41
What are uncommon findings with NHL (4)?
1. Nasopharyngeal mass
2. Parotid enlargement
3. Nephromegaly
4. Testicular enlargement
42
Common physical NHL findings:
| Abdominal distention or mass with or without tenderness, rebound tenderness, and/or shifting _____.
Dullness
43
When is NHL most common?
2nd decade of life. Peaks at 5-15 yo.
44
T/F Orbital inflammation implies extraocular extension in retinoblastoma.
False. Eyes have necrotic tumors, but not necessarily extraocular extension.
45
What is the most common cancer in 5-9 yo (2)?
1. Acute leukemia (32%)
| 2. Brain and CNS (28%)
46
This is a HALLMARK finding of Hodgkin Lymphoma (HL).
Reed-Sternberg Cells
47
This is associated with Wilms tumor and includes aniridia, GU abnormalities, and mental retardation.
WAGR
48
What is the greatest predictor of death in retinoblastoma?
Extraocular extension either directly through the sclera or via extension along the optic nerve.
49
Treatment for osteosarcoma (3)?
1. Resection (essential for cure)
2. Presurgical chemo (shrinking of tumors and assessment of responsiveness of tumor)
3. Postsurgical chemo
50
What gene?
| Cause the cell cycle to go out of control.
Oncogenes
51
How does previous exposure to malaria increase risk of Non-Hodgkin Lymphoma?
Resultant T-cell suppression and EBV.
52
Where is the mutation in retinoblastoma?
13q14
53
T/F Fundoscopic exam must be performed in all cases of childhood strabismus.
True. Strabismus can be the result of visual loss and could indicate retinoblastoma.
54
T/F Chemo is followed by radiation to the primary site of the lesion in osteosarcoma.
False. No radiation d/t high level of resistance.
55
T/F Treatment for NHL is similar to that for ALL.
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.
56
What gene?
Ensure DNA strands are copied correctly during cell division. Change causes accumulation of mutations in critical growth-regulating genes.
DNA Repair Genes
57
Favorable or unfavorable Shimada classification of neuroblastoma?
Older than 18 months with stroma-poor tumors, differentiated neuroblasts, and an MKI of 100-200/5000.
Unfavorable
58
Group of malignant hematologic diseases where normal bone marrow elements are replaced by abnormal, poorly differentiated lymphocytes (i.e., blast cells).
Acute Lymphoblastic Leukemia (ALL)
59
Which cells are usually involved in non-Hodgkin lymphoma (3)?
1. T cells
2. B cells
3. Indeterminate lymphocyte cells
60
Is Wilms tumor more common in AA or Caucasian children?
AA
61
In NHL, what labs would you get if the patient had a fever or evidence of sepsis (3)?
1. PT/PTT
2. Fibrinogen
3. D-Dimer
62
Which site has the best prognosis in osteosarcoma?
Distal extremity
63
Why does treatment of Hodgkin Lymphoma increase risk for Non-Hodgkin Lymphoma?
Combined effects of chemo and radiation with immunosuppression.
64
Labwork for retinoblastoma (5).
1. CBC
2. CMP
3. LDH
4. UA
5. Blood specimens for DNA analysis (patients, parents, siblings)
65
How is response to treatment measured in NHL?
PET/CT
66
What is the mainstay of therapy for osteosarcoma?
Removal of the lesion. Chemo is required to treat micrometastatic disease which is present but not detectable in most patients at diagnosis.
67
Common physical NHL findings:
| Decreased breath sounds indicate _____ or _____.
Bronchial obstruction or pleural effusion
68
Where is the lymphadenopathy in HL?
Usually cervical (70-80%)
69
This is ordered for classification and staging of cancer.
Biopsy
70
Risk factors for non-Hodgkin lymphoma (8).
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
71
What is MIBG?
Compound that accumulates in catecholaminergic cells. Provides specific way of identifying primary and metastatic disease if present in neuroblastoma.
72
This cancer is staged with the St. Jude system.
Non-Hodgkin Lymphoma (NHL)
73
What does urine show in neuroblastoma?
Elevated catecholamines detectable in urine. Specifically, HVA and VMA levels.
74
Adult cancer locations commonly include breast, colon, lung, prostate. Peds is _____, _____, and _____.
CNS
Muscle
Bone
75
T/F There are links between viral exposure, such as EBV, and childhood ALL.
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.
76
Why are lumbar puncture and bone marrow aspiration and biopsy performed in retinoblastoma?
Useful in early diagnosis of distant metastasis.
77
Appearance of cells under microscope.
Morphology
78
Adults or Peds?
| Fewer long-term consequences.
Adults
79
Imaging studies in Ewing sarcoma (3).
1. X-rays
2. MRI (extent of disease)
3. CT scan (bony involvement)
80
Stage 1, 2, 3, A, or B for osteosarcoma?
| Intramedullary lesion.
A
81
In neuroblastoma, thoracic tumors extending to the neck are known as _____.
Horner syndrome
82
If ALL is T-lineage, what might you find?
Mediastinal mass that causes respiratory distress and stridor.
83
How common is it for both kidneys to be affected or for them to be affected one right after the other in Wilms tumor?
5-10%
84
What is the most common cancer in 15-19 yo (2)?
Lymphoma (26%)
| Carcinoma (21%)
85
Adults or Peds?
| More long-term consequences.
Peds
86
When would you get an echocardiogram in NHL?
To obtain baseline findings before chemotherapy with anthracyclines (cardiomyopathy).
87
Favorable or unfavorable Shimada classification of neuroblastoma?
Any age with stroma-poor tumors, undifferentiated or differentiated neuroblasts, and an MKI more than 200/5000.
Unfavorable
88
What is the most common way to diagnose Wilms tumor?
After incidental detection of asymptomatic abdominal mass.
89
What labs should be ordered for general diagnostic workup in cancer (4)?
1. CBC
2. Electrolytes
3. LFTs
4. Renal FTs
90
What is the classic triad in Horner syndrome?
1. Miosis (constricted pupils)
2. Partial ptosis
3. Loss of hemifacial sweating
91
T/F Lymphadenopathy is common in osteosarcoma.
False
92
Adults or Peds?
| Tolerate higher doses of meds.
Peds
93
What tests should be ordered for general diagnostic workup in cancer (2)?
1. Biopsy
| 2. Bone Marrow Aspirate/Biopsy
94
What are the 3 phases of treatment for ALL?
1. Induction Phase
2. Intensification (Consolidation) Phase
3. Maintenance (Continuation) Therapy
95
If the spermatic vein is obstructed in Wilms tumor, what may be noted?
Left varicocele
96
The histology of adult cancer is more epithelial/organ. Peds is _____.
Nonepithelial (leukemia, CNS, sarcomas)
97
If there is testicular involvement with ALL, what might you find?
Unilateral, painless testicular enlargement.
98
What imaging should be ordered for general diagnostic workup in cancer (4)?
1. X-rays
2. CT
3. MRI
4. Bone scan
99
ALL peaks in children aged _____ years old and subsequently decreases with age.
2-6
100
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.
2
101
General clinical manifestations of cancers in children (7).
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
102
_____ pain may indicate a paraspinal, retroperitoneal, or deep pelvic Ewing sarcoma.
Back
103
Favorable or unfavorable Shimada classification of neuroblastoma?
Older than 18 months with stroma-poor tumors, undifferentiated neuroblasts, and an MKI more than 100/5000.
Unfavorable
104
Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?
| Localized tumor, complete gross excision, or both with ipsilateral nonadherent lymph nodes positive for tumor.
2B
105
In NHL, small noncleaved cell involvement leads to _____ tumors.
Abdominal
106
When is radiation therapy used in NHL?
Mediastinal or CNS involvement
107
HTN in neuroblastoma is caused by _____, not catecholamine excess.
Renal artery compression
108
What is the duration of symptoms before diagnosis in NHL?
Generally 1 month or less.
109
Which version of ALL has the most pronounced male predominance?
T-cell ALL
110
Why is there abdominal pain, fever, anemia, and hypotension in Wilms tumor?
Hemorrhage into the tumor.
111
Common physical NHL findings:
| Obtundation, agitation, and meningismus indicate _____.
CNS involvement
112
What is the most common cancer in <5 yo?
Acute leukemia (35%)
113
Does ALL affect black or white children more?
White
114
```
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.
```
4
115
T/F Blood studies provide pathognomic or suggestive results to diagnose Ewing sarcoma.
False. Blood tests may be helpful in evaluating other diagnoses (Blood cultures, CRP, CBC, LDH, ESR).
116
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.
1
117
What is considered elevated with VMA/HVA in neuroblastoma?
3 standard deviations higher than age-related reference range levels.
118
T/F With ALL, specific translocation of chromosomes on cells can affect prognosis.
True
119
Adults or Peds?
| More difficulty with acute toxicities.
Adults
120
T/F Clinically significant cytopenias are common in NHL at diagnosis.
False. Clinically significant cytopenias are uncommon in NHL.
121
Expression of cell surface antigens.
Immunophenotype
122
S/Sx of ALL (7).
1. Anemia (fatigue/pallor)
2. Thrombocytopenia (petechiae, bleeding, purpura)
3. Neutropenia (fever, recurrent infections)
4. Bone pain
5. Limp
6. Lymphadenopathy
7. Hepatosplenomegaly
123
Children with more than _____ chromosomes (hyperdiploid) on cells have the best prognosis in ALL.
50
124
Undifferentiated neuroblastomas histologically present as small, round, blue cell tumors with dense nests of cells in a fibrovascular matrix and _____.
Home-Wright pseudorosettes
125
This is associated with Wilms tumor and includes unusual facies, islet cell hypertrophy, macrosomia, and hamartomas.
Perlman Syndrome
126
What kind of CXR is needed in Wilms tumor?
4-field
127
Labwork for Wilms tumor (5)?
1. CBC
2. CMP
3. UA
4. Coags
5. Cytogenetics (1p and 16 q deletion may reveal 11p13 and 11p15 issues)
128
Where is the most common site for osteosarcoma?
Femur (distal) followed by tibia (proximal) and humerus (proximal).
129
In retinoblastoma, glaucoma, retinal detachment, and inflammation are signs of _____.
Tumor necrosis
130
Common physical NHL findings:
| Distended neck veins and plethora indicate _____.
Superior vena cava syndrome
131
What labs do you order in HL (6)?
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)
132
An area where it is difficult to get enough concentration of chemo to kill leukemia cells.
Sanctuary sites
133
Why is ALL treatment primarily based on chemo?
Systemic disease
134
Genetic abnormalities in number or structure of chromosomes.
Chomosomal Analysis (Cytogenetics)
135
The pathogenesis of adult cancer is environmental and lifestyle factors. Peds has a _____.
Genetic role
136
T/F It is important to carefully evaluate all lymph node stations and for involvement of liver, spleen, and tonsillar tissue in HL.
True. Important in evaluation of response to treatment.
137
Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?
| Localized unilateral tumor with contralateral regional lymph node involvement.
3
138
In NHL, lymphoblastic involvement leads to _____ tumors.
Intrathoracic
139
What are important features of neuroblastoma (5)?
1. Degree of neuroblast differentiation.
2. +/- Schwannian stromal development (stromal rich or poor).
3. Index of cellular proliferation (MKI).
4. Nodular pattern.
5. Age.
140
Chronic diarrhea in neuroblastoma is secondary to tumor secretion of _____.
Vasoactive intestinal peptide
141
What are survival rates for retinoblastoma?
86-92%. Rates drop with each decade of life for patients with the genomic mutation.
142
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.
1
143
T/F Those with the genomic mutation for retinoblastoma are more likely to develop secondary cancers later in life.
True. They also present with either bilateral disease or unilateral multi-focal disease in retinoblastoma.
144
If there is CNS involvement with ALL, what might you find (6)?
1. Headache
2. Vomiting
3. Lethargy
4. Papilledema
5. Nuchal rigidity
6. Cranial nerve deficits
145
Who is most often affected by Hodgkin Lymphoma (HL)?
Young adults. Occurs in all age groups, but is rare in children younger than 5 yo.
146
Why does pseudouveitis occur in retinoblastoma?
Tumor cells invade the retina diffusely without forming a discrete tumor mass.
147
Stage 1, 2, 3, A, or B for osteosarcoma?
| High-grade lesions.
2
148
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.
3
149
What test is used to r/o testicular infiltration if the testes are enlarged?
Ultrasound
150
What are you looking for with the CBC in ALL (3)?
1. Peripheral smear for lymphoblasts
2. Elevated leukocyte count
3. Pancytopenia
151
T/F A large number of patients with retinoblastoma (95%) have no previous family hx, even those with the bilateral, hereditary form.
True
152
Which site has the worst prognosis in osteosarcoma?
Axial skeleton
153
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.
2
154
What is the more common presenting symptom of retinoblastoma in underdeveloped countries.
Proptosis
155
Adults or Peds?
| Tolerate lower doses of meds.
Adults
156
Common physical NHL findings:
| Focal pain or swelling in the extremities indicate _____.
Primary bone lymphoma
157
This cancer is staged with the Ann Arbor system.
Hodgkin Lymphoma (HL)
158
What is the treatment for Wilms tumor that is unilateral and how does it differ for bilateral?
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.
159
Name 2 sanctuary sites.
1. CNS
| 2. Testicles
160
Stage 1, 2, 3, A, or B for osteosarcoma?
| Local extramedullary spread.
B
161
Adults or Peds?
| Tumors more responsive to chemotherapy.
Peds
162
Imaging studies for retinoblastoma (3).
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)
163
Imaging studies in osteosarcoma (5)?
1. Plain x-ray
2. CXR (metastases)
3. CT (lesion and chest)
4. MRI (lesion)
5. Bone scan
164
What is a sign of advanced neuroblastoma?
Periorbital ecchymosis (metastatic disease to the orbits).
165
Performed when there is no chance of preserving useful vision in an eye with retinoblastoma.
Enucleation
166
What are treatment options for neuroblastoma (4)?
1. Surgery
2. Radiation
3. Chemo
4. Bone marrow transplant (high risk patients)
167
Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?
| Representative ipsilateral nonadherent lymph nodes microscopically negative for tumor.
2A
168
In NHL, maintenance therapy may be continued for _____.
6 months to 2 years
169
S/Sx of paraneoplastic syndrome in neuroblastoma.
Opsoclonus (random eye movements and myoclonus)
170
What is the most common sx with osteosarcoma?
Pain, particularly with activity. There can be swelling, depending on size and location.
171
Children with down syndrome have a 20x greater chance of developing _____.
ALL
172
Favorable or unfavorable Shimada classification of neuroblastoma?
Any age with stroma-rich tumors without a nodular pattern.
Favorable
173
T/F Wilms tumor never crosses the midline of the abdomen.
False. That may be noted, but not always.
174
What is the prognosis of NHL and HL in:
Stages 1-2?
Stages 3-4?
```
1-2 = 90%
3-4 = 70%
```
175
Adults or Peds?
| Less responsive to chemo.
Adults
176
_____ directed therapy is critical for improved survival rates in ALL and includes intrathecal chemo.
CNS
177
What are the 2 most common loci for gene alteration in Wilms tumor (tumor suppressor genes)?
1. 11p13 (WT1)
| 2. 11p15 (WT2)
178
Is ALL more common in males or females?
Males (slightly)
179
Bone marrow metastasis can produce _____ or _____.
Petechiae or purpura
180
What is a red flag for anesthesia for diagnostic procedures in HL?
Mediastinal adenopathy (SOB, chest pain, cough) can put at risk for respiratory failure.
181
This is ordered to determine the extent of involvement of malignant cells in marrow.
Bone Marrow Aspirate/Biopsy
182
What is the major risk with the PE and Wilms tumor?
Palpating too vigorously can rupture the tumor and create stage 3 cancer.
183
When is cranial irradiation or intrathecal chemo used in NHL?
CNS involvement
184
This is associated with Wilms tumor and includes hemihypertrophy, macroglossia, omphalocele, and GU abnormalities.
Beckwith-Wiedemann Syndrome
185
Risk factors for HL (2).
1. Genetics
| 2. Immunodeficiencies (EBV, CMV)
186
Cancer is classified by cell type involvement and by _____.
Cellular differentiation
