Peds Oncology -Junkins

Question 1

What does metastasis imply? How does this happen?

Answer 1

Malignancy*

• seeding of body cavities and surfaces
• lymphatic spread
• hematogenous spread

Question 2

Case: 
A previously healthy 2 yo presents with prolonged unexplained fever and fussiness. On PE, he has pallor, diffuse LAD and a rash. WBC: 4000 (90% lymphocytes), ANC: 400, Hemoglobin: 4, Platelets: 80,000. Lots of small purple cells in the smear. 
What is the likely diagnosis?
A. AML
B. CML
C. ALL
D. Burkitt Lymphoma 
E. Non-Hodgkin's lymphoma

Answer 2

C. ALL

Question 3

Case: 
A previously healthy 2 yo presents with prolonged unexplained fever and fussiness. On PE, he has pallor, diffuse LAD and a rash. WBC: 4000 (90% lymphocytes), ANC: 400, Hemoglobin: 4, Platelets: 80,000. Lots of purple cells in the smear. 
What is the likely diagnosis?
A. AML
B. CML
C. ALL
D. Burkitt Lymphoma 
E. Non-Hodgkin's lymphoma

Answer 3

C. ALL

Question 4

What do mutations of stem cells or early blood cell precursors that impair and slow blood cell maturation give rise to?

Answer 4

Myelodysplastic disorders (MDS)

Question 5

What are some risk factors for leukemia?

Answer 5

• Downs syndrome
• identical twin with leukemia
• Fanconi’s anemia
• Li-Fraumani syndrome
• exposure to radiation, benzene, topoisomerase inhibitors and HTLV-1)

Question 6

What are the classifications of ALL based on morphology?

Answer 6

L1: uniformity of cell morphology
L2: variation in size and shape
L3: characteristic cytoplasmic granules (inclusion bodies)

Question 7

What are the markers associated with the different subtypes of ALL (pro-B cell, common, pre-B cell, B cell, T cell)?

Answer 7

• Pro B cell: CD19/CD20 +
• Common: 60%*, CD10 +
• Pre-B cel: cIgM +
• B cell: L3, sIgM +
• T cell: CD7/CD2 +

Question 8

What are the translocations associated with Burkitt’s (B cell) lymphoma?

Answer 8

t(8; 14)
t (8; 22)
t (2;8)

Question 9

True or false:

Children younger than 6 years old had significantly better Overall Survival than children aged ≥6 years

Answer 9

True

Question 10

Which ALL translocation has a better prognosis?

Answer 10

t (12, 21)

Question 11

Which cancer will have Reed-Sternberg cells? What markers are these + for?

Answer 11

Hodgkin’s lymphoma (Owl’s eyes)

CD15/CD30+ B cell origin

Question 12

What is the Ann Arbor Staging used for? What are the stages?

Answer 12

Hodgkin's lymphoma
I: lymph node above the diaphragm affected 
II: multiple above the diaphragm 
III: multiple above and 1 below
IV: multiple above and below

Question 13

Pathologic examination of a pt’s tumor shows blue, small, round cells with Homer-wright pseudo rosettes. What is the most likely diagnosis?

Answer 13

Medulloblastoma

Question 14

What are the most common peds solid organ tumors and the leading cause of death from childhood cancer?

Answer 14

Brain tumors (types=medulloblastoma and astrocytoma)

Question 15

What is the most common brain tumor of childhood and where does it present?

Answer 15

Medulloblastoma

in the cerebellum

Question 16

What does the pathology of Medulloblastoma look like and which mutations are associated with poor prognosis? What should you be aware of in treating children

Answer 16

• rosette arrangement of dark nuclei cells
• MYC=poor prognosis
• WNT=better prognosis

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Question 17

How does Retinoblastoma develop?

Answer 17

-Results from mutations of BOTH alleles of the Rb gene (chromosome 13q14)
-loss of Rb =deregulation
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Question 18

What is the “2-hit” hypothesis?

Answer 18

• “2-hit”: 2 separate mutations are required for tumorigenesis.
• heritable=1 inherited allele and a 2nd is acquired by mutation ==> binocular and multifocal disease
• Non-inherited=2 spontaneous mutations in a single retinal cell –> funicular unifocal disease

Question 19

How does Retinoblastoma develop? What is the most common finding?

Answer 19

• Results from mutations of BOTH alleles of the Rb gene (chromosome 13q14)
• loss of Rb =deregulation
• Leukocoria

Question 20

A 2 yo is broght in because of a large, unilateral abd mass noted by mother. While performing an US guided biopsy, the technician notes that the kidney calyces are highly distorted by the mass. Which of the following is most likely the origin of this lesion?
A. a mutation of the APDK1 gene on chromosome 16
B. Embryonic renal cells from the embryonic kidney
C. malignant transformation of renal tubular cells
D. Malignant transformation of uroepithelial cells
E. primitive neural crest cells

Answer 20

B. Embryonic renal cells from the embryonic kidney

Wilms tumor

Question 21

Where will an osteosarcoma present? An Ewing Sarcoma?

Answer 21

Osteosarcoma=metaphysis

Ewings=diaphysis (moth eaten or ratty )