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
• Whats his diagnosis?

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Chronic Myelocytic Leukemia

• Accounts for
• Three phases
• Chronic (
• 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 weeks
  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(1517) PML-RARA gene rearrangement found in
  nearly all cases

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FISH for t(1517)
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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
• Whats the diagnosis?

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M7 AML

• Megakaryocytic leukemia
• 5-10 of AML
• Associated with
• Downs syndrome
• Klinefelters
• 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
• Whats in the differential? Whats the diagnosis?

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Wilms 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
• Whats in your differential?

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Neuroblastoma

• Characteristics
• Neural crest origin
• 1 case per 7,000 births
• Median age _at_ 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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Burkitts Lymphoma

• Usually presents as abdominal mass
• Change in bowel habits
• Intussusception
• Nausea/vomiting
• Doubling time
• 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
• Whats 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
• WBC
• No CNS leukemia
• Hyperdiploidy (DI1)
• Trisomies 4,10 17
• t(1221) TEL/AML1

• Poor Prognosis
• Age 9.99yr
• WBC 50K
• CNS leukemia
• Hypodiploidy (DI
• t(922)
• t(411) MLL rearrangement