Oncology Slide Review

1. Oncology Slide Review LaJuan Chambers, MD

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

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

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

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

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 <1yr or >9.99yr WBC >50K CNS leukemia Hypodiploidy (DI<1) t(9;22) t(4;11) MLL rearrangement