Oncology Slide Review
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Transcript of Oncology Slide Review
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Oncology Slide Review
LaJuan Chambers, MD
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•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?
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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
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•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?
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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
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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
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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
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FISH for t(15;17)
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• 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?
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M7 AML
• Megakaryocytic leukemia
• 5-10% of AML
• Associated with:– Downs syndrome– Klinefelter’s
• Difficult to treat (chemo, stem cell transplant)
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• 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?
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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
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•2 yo child presents with abdominal mass and painless hematuria
•No other symptoms
•What’s in the differential? What’s the diagnosis?
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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
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• 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?
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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
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• 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
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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
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•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?
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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
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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