9: Peds Cancer Flashcards
What is the most common cancer in 10-14 yo (3)?
- Acute leukemia (21%)
- Brain and CNS (21%)
- Lymphoma (21%)
What meds are used during induction phase of ALL treatment (3)?
- Vincristine
- Prednisone
- L-asparaginase
Risk factors for cancer in children (6)?
- 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)
3 classification systems of non-Hodgkin lymphoma.
- Lymphoblastic lymphoma
- Small, noncleaved cell lymphoma (Burkitt, non-Burkitt, B-cell)
- Large cell lymphoma
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.
2
Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?
Localized tumor with complete gross excision, microscopic residual disease, or both.
1
Is this a sign of anemia, thrombocytopenia, or neutropenia?
Petechiae, bleeding, purpura.
Thrombocytopenia
What imaging is ordered with Wilms tumor (4)?
- CXR (4-field)
- Renal U/S
- Abd CT
- Abd MRI (caval patency)
Stains used of biochemical markers.
Cytochemistry
T/F The prognosis of children with HL is similar to that of adults with HL.
True
T/F With osteosarcoma, there can be a tender, warm, palpable mass. But signs are indistinguishable from those of osteomyelitis.
True
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.
Congenital rubella
Blueberry muffin baby
Maintenance therapy is targeted at eliminating _____ disease.
Residual
Which marker has the worst prognosis in neuroblastoma?
MYCN amplification tend to have rapid tumor progression and poor prognosis.
Treatment for Ewing sarcoma (3).
- Chemo
- Radiation
- Surgery
What is the most common site of origin in NHL?
Lymphoid structures of the intestinal tract.
What are B symptoms of prognostic significance in HL (3)?
- 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)
Why might there be weakness, limping, paralysis, and bladder/bowel dysfunction in neuroblastoma?
Symptoms arise from tumors of the paraspinal sympathetic that grow through the spinal foramina into the spinal canal and impinge on the spinal cord.
Labwork in neuroblastoma (5)?
- CBC
- CMP
- UA
- Coags
- LDH (Lactate dehydrogenase)
Why do pruritis, urticaria and fatigue occur in HL?
Cytokines produced by HL-RSC or supporting environment within the affected lymph nodes.
Is this a sign of anemia, thrombocytopenia, or neutropenia?
Fatigue, pallor.
Anemia
Is this a sign of anemia, thrombocytopenia, or neutropenia?
Fever, recurrent infections.
Neutropenia
What are the median age at diagnosis and peak age (unilaterla and bilateral) for Wilms tumor?
Median = 3.5 years Peak = 2-3 years unilateral Peak = <3 bilateral
How does EBV exposure increase risk of Non-Hodgkin Lymphoma?
Causes B-cell proliferation and in vitro immortalization.
What gene?
Normal role in cell division and growth. If mutated, they become carcinogenic.
Proto-Oncogenes
Adults or Peds?
Less difficulty with acute toxicities.
Peds
Common physical NHL findings:
Firm, fixed, and nontender cervical or _____ masses or adenopathy.
Supraclavicular
Stage 1, 2A, 2B, 3, 4, or 4S neuroblastoma?
Limited to infants.
4S
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
What gene?
“Parents” gene. When damaged or missing, cell ignores inhibitory signals and grows out of control.
Tumor Suppressor Genes
Labwork in osteosarcoma (4)?
- CBC
- CMP
- LDH/Alkaline Phosphatase (prognostic significance)
- UA
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.
Common physical NHL findings:
Thoracic dullness to percussion indicate _____.
Pleural effusion
T/F Combined modality (chemo + radiation) is the preferred approach for peds with HL.
True
What causes HTN in Wilms tumor?
Renin secretion by tumor cells or compression of renal vasculature by tumor.
Common physical NHL findings:
Dyspnea or stridor indicates _____.
Mediastinal mass
What test is used to r/o CNS involvement in ALL?
Lumbar Puncture
A large mediastinal shift indicates _____.
Superior vena cava syndrome
What are the 2 peaks for HL?
- 25
2. 50-60
Which site has an intermediate prognosis in osteosarcoma?
Distal femur
What are uncommon findings with NHL (4)?
- Nasopharyngeal mass
- Parotid enlargement
- Nephromegaly
- Testicular enlargement
Common physical NHL findings:
Abdominal distention or mass with or without tenderness, rebound tenderness, and/or shifting _____.
Dullness
When is NHL most common?
2nd decade of life. Peaks at 5-15 yo.
T/F Orbital inflammation implies extraocular extension in retinoblastoma.
False. Eyes have necrotic tumors, but not necessarily extraocular extension.
What is the most common cancer in 5-9 yo (2)?
- Acute leukemia (32%)
2. Brain and CNS (28%)
This is a HALLMARK finding of Hodgkin Lymphoma (HL).
Reed-Sternberg Cells
This is associated with Wilms tumor and includes aniridia, GU abnormalities, and mental retardation.
WAGR
What is the greatest predictor of death in retinoblastoma?
Extraocular extension either directly through the sclera or via extension along the optic nerve.
Treatment for osteosarcoma (3)?
- Resection (essential for cure)
- Presurgical chemo (shrinking of tumors and assessment of responsiveness of tumor)
- Postsurgical chemo
What gene?
Cause the cell cycle to go out of control.
Oncogenes
How does previous exposure to malaria increase risk of Non-Hodgkin Lymphoma?
Resultant T-cell suppression and EBV.
Where is the mutation in retinoblastoma?
13q14
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.
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.
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.
What gene?
Ensure DNA strands are copied correctly during cell division. Change causes accumulation of mutations in critical growth-regulating genes.
DNA Repair Genes
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
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)
Which cells are usually involved in non-Hodgkin lymphoma (3)?
- T cells
- B cells
- Indeterminate lymphocyte cells
Is Wilms tumor more common in AA or Caucasian children?
AA
In NHL, what labs would you get if the patient had a fever or evidence of sepsis (3)?
- PT/PTT
- Fibrinogen
- D-Dimer
Which site has the best prognosis in osteosarcoma?
Distal extremity
Why does treatment of Hodgkin Lymphoma increase risk for Non-Hodgkin Lymphoma?
Combined effects of chemo and radiation with immunosuppression.
Labwork for retinoblastoma (5).
- CBC
- CMP
- LDH
- UA
- Blood specimens for DNA analysis (patients, parents, siblings)
How is response to treatment measured in NHL?
PET/CT
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.
Common physical NHL findings:
Decreased breath sounds indicate _____ or _____.
Bronchial obstruction or pleural effusion
Where is the lymphadenopathy in HL?
Usually cervical (70-80%)
This is ordered for classification and staging of cancer.
Biopsy
Risk factors for non-Hodgkin lymphoma (8).
- Certain HLA types + certain blood types
- Pesticide exposure
- High birth weight
- Immunosuppression
- EBV
- Successful Hodgkin treatment
- Previous malaria exposure
- Genetics
What is MIBG?
Compound that accumulates in catecholaminergic cells. Provides specific way of identifying primary and metastatic disease if present in neuroblastoma.
This cancer is staged with the St. Jude system.
Non-Hodgkin Lymphoma (NHL)
What does urine show in neuroblastoma?
Elevated catecholamines detectable in urine. Specifically, HVA and VMA levels.
Adult cancer locations commonly include breast, colon, lung, prostate. Peds is _____, _____, and _____.
CNS
Muscle
Bone