1. Oncology Slide Review LaJuan Chambers, MD

2. 16 yo young man with fatigue, pallor and low-grade fever for 2 weeks On exam, spleen palpated 8cm below left costal margin HPD reveals WBC 200,000, Hgb 5g/dl, platelet 700,000 Bone marrow to the left… What’s his diagnosis?

3. Chronic Myelocytic Leukemia Accounts for <5% of leukemias in children Three phases: –Chronic (<5%) –Accelerated (5-30%) –Blast (>30%) Therapy: –Chemotherapy (Imantinib) –Stem cell transplant

4. 4 yo boy with known Wiskott- Aldrich syndrome Presents with right-sided neck mass of 2 week’s duration Two maternal uncles have had similar condition FNA findings to the left… What is his diagnosis?

5. Malignant Lymphoma Usually Non-Hodgkins lymphoma –Large cell immunoblastic (typically) Occasionally presents in extranodal locations and CNS Difficult to treat (most die within a year of diagnosis) Many have c-myc gene rearrangements

6. Case #3 2 yo child with fever, fatigue, epistaxis and pallor Exam reveals moderate splenomegaly and petechiae WBC 2,000; Hgb 6g/dl and platelet count 17K PT 17, PTT 45, INR 3.5

8. M3 AML 5-10% of childhood AML Blasts have granules and Auer rods Increased risk of bleeding diathesis Overall good prognosis (chemo and all- trans retinonic acid alone) t(15;17) PML-RARA gene rearrangement found in nearly all cases

9. FISH for t(15;17)

10. 3 yo boy with Downs syndrome presents with extensive bruising, epistaxis and pallor WBC 0.5K, Hgb 7.7g/dl and platelets 4K Bone marrow aspirate revealed these cells What’s the diagnosis?

11. M7 AML Megakaryocytic leukemia 5-10% of AML Associated with: –Downs syndrome –Klinefelter’s Difficult to treat (chemo, stem cell transplant)

12. 20 month old child presents to PCP for well child checkup Grandparents noticed that his eyes looked “different” on Christmas photos What is this “finding” called?

13. Retinoblastoma Often present at birth 1 in 18,000 live births in the US Bilateral disease present in 20-30% May be inherited or sporadic 13q14 mutation may be found

15. 2 yo child presents with abdominal mass and painless hematuria No other symptoms What’s in the differential? What’s the diagnosis?

16. Wilm’s Tumor Presentation: Abdominal mass Hematuria Hypertension May be associated with: –WAGR –Beckwith-Wiedemann –Denys-Drash May be associated with: –WT1 (11p13) gene –WT2 (11p15) gene Amenable to: –Surgery –Chemotherapy –Radiation to mets

17. 3 yo child with hypertension, watery diarrhea and the CT scan findings to the left… Biopsy of mass reveals the findings shown… What’s in your differential?

18. Neuroblastoma Characteristics: –Neural crest origin –1 case per 7,000 births –Median age @ diagnosis – 22 months –Catecholamine excess –Elevated urine VMA/HVA Poor prognostic factors: –Age >1yr –Elevated ferritin/LDH –Extensive disease –Amplified n-MYC –Persistent bone marrow involvement –Poor histological differentiation

19. 2yo with rapidly enlarging abdomen Normal HPD, but metabolic panel reveals potassium 7mmol/L, creatinine 3mg/dl, uric acid 15mg/dl, calcium 6mg/dl and phosphorus 6mg/dl

20. Burkitt’s Lymphoma Usually presents as abdominal mass: –Change in bowel habits –Intussusception –Nausea/vomiting Doubling time <24 hours May be complicated by tumor lysis syndrome May have bone marrow involvement Treatment consists of: –Chemo (systemic and intrathecal) Overall good response to therapy and good prognosis

21. 2 yo girl with lymphadenopathy, fever, fatigue, bruising and pallor WBC 46K (with 90% “atypical lymphocytes”), Hgb 5g/dl and platelets 5K What’s her diagnosis?

22. Acute Lymphoblastic Leukemia Most common type of leukemia in childhood (80%) Peak incidence age 4y Pts. stratified according to risk: –Low –Standard –High –Very-high

23. Prognostic Factors - ALL Good Prognosis –Age >1yr or <9.99yr –WBC <50K –No CNS leukemia –Hyperdiploidy (DI>1) –Trisomies 4,10 & 17 –t(12;21) TEL/AML1 Poor Prognosis –Age 9.99yr –WBC >50K –CNS leukemia –Hypodiploidy (DI<1) –t(9;22) –t(4;11) MLL rearrangement