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PEDIATRICS EXAM REVISION > Pediatric Oncology 2019 > Flashcards

Pediatric Oncology 2019 Flashcards

To prepare for College Exam

1
Q

6 MP

What is mechanism of action/excretion, uses, short and long term toxicity?

A
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2
Q

A Cancer What is the incidence of pediatric cancers by age group?

A
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3
Q

ALL Epedimiology?

A

Most common type of childhood cancer; 1/3 of childhood ca, of which ¾ is ALL

85% of all childhood leukemia; incidence 3/100,000

Peak 2-6yrs, boys>girls

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4
Q

ALL Etiology?

8 conditions, and 4 environmental associations?

A
  1. Unknown,
  2. genetic conditions

Ataxia-telangiectasia, Bloom, Down, Fanconi, Klinefelter, NF-1, SCID, Turner

  1. environmental associations -benzene, drugs (alkylating agents),3ionizing radiation, ,
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5
Q

ALL What are the High risk features?

what are the survival outcomes?

Name one bad translocation?

A
  1. NACI High risk (Age 10 - 13), WBC >50 = 75% survival
  2. Testicular disease AND
  3. SR but MRD+ at D29 +/- other features
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6
Q

ALL Rx What are the Induction drugs and maintainance Rx for Treatment of standard risk ALL?

What additional Rx for high risk?

A
  1. Induction with:

-Intrathecal chemo (Cytarabine + MTX)

  • P: Prednisone & PEG asparaginase
  • Vincristine, methotrexate

…and Possible if High risk: Daunorubicin

  1. Maintainence with Mtx and 6-MP
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7
Q

ALL What are flow cytometry + and -ve markers?

A

flow cytometry

Positive

CD19 B cell

CD10

Tdt

Negative

CD3

MPO

Myeloperoxidase

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8
Q

ALL What are the 4 phases of Rx?

A

1) Induction – goal is to attain remission (<5% blasts in bone marrow), attained in 95% of children
2) CNS prevention – eradicate subclinical CNS leukemia via intrathecal chemotx
3) Consolidation – goal is to decrease relapse
4) Maintenance

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9
Q

ALL What are the common types of ALL?

A
  1. Pre-B ALL

-“Common”

  • Infant
  • Philadelphia positive

2. T-ALL

  1. Mature B-cell

Commonest are in BOLD

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10
Q

ALL What are the flow cytometry markers?

A

Flow cytometry:

CD19 = B-cell marker

CD10 = Early B-cell marker

CD3 = T-cell marker

CD33 = myeloid marker

MPO = myeloid marker

Tdt = Terminal deoxynucleotidyl transferase

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11
Q

ALL What are the Sx?

A

Reflect degree of bone marrow infiltration by leukemic cells and extent of disease outside the marrow.

Common:

  1. Pancytopenia symptoms, bruising, palllor & Fatigue
  2. Other: Fever, Bone pain/limp, Hepatosplenomegaly, Lymphadenopathy

Less common:

  1. CNS: Cranial nerve palsies, signs of papilledema,
  2. Mucous/skin: Gum hypertrophy/ skin cutis

3. GUT:

a. Testicular enlargement
3. Nephro: Renal failure
4. MSK: Solid mass (chloroma in spine), vertebral #
5. Complications of SVC syndrome or airway compression 2º mediastinal mass (esp T-cell ALL)

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12
Q

ALL What are the very high risk features?

A
  1. Age : > 13 years
  2. CNS +, Hypodiploidy (<44), iAMP21, KMT2a rearrangement,

BCR-ABL,

  1. Induction failure, MRD + at D29 (SR or HR)

Are these essential?

Presence of t(9:22) (Philadelphia +), t(4:11),

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13
Q

ALL What are the 3 DDx categories for ALL?

A
  1. Hemato-oncology:
    a. Other malignancies (AML, NBL, Ewings Sarcoma, etc.),
    b. 1° bone marrow failure (aplastic anemia),
    c. Hematology: Transient erythroblastopenia of childhood (TEC), ITP
  2. Infections: EBV,
  3. Rheum: JIA.
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14
Q

ALL What does this slide show? -

List 5 slide features

A

Blasts visible with:

a. Large nuclei & Scant cytoplasm (High N:C ratio)
b. Fine chromatin

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15
Q

ALL What factors make a child standard risk?

Age & White cell count?

Trisomy, diploidy, translocation?

A
  1. Age >1 & <10 years, WBC < 50,000 = 85% survival
  2. No unfavourable features AND MRD negative at D29
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16
Q

ALL What Inv do you do?

A
  1. Blood:
    a. CBC shows abnormalities of ≥2 cell lines; lymphoblasts on smear
    b. Flow cytometry-Cytochemical analysis, Immunophenotyping, Cytogenetic analysis
  2. Bone marrow aspirate –blasts confirms diagnosis (>25%)
  3. CXR to screen for mediastinal adenopathy
  4. Lumbar puncture to screen for CNS disease (present <5% children at diagnosis)
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17
Q

ALL What is standard low risk?

A
  1. NCI Standard Risk (Age 1 – 10, WBC <50)
  2. Favourable cytogenetics
    a. ETV-RUNX1 (TEL-AML)
    b. Trisomy 4 and 10...96% :).

• AND

  1. MRD negative at D29
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18
Q

ALL What studies of tumor cells are useful for determining a patient’s prognosis?

A
  1. The cytogenetics and DNA index (ratio of DNA content in abnormal cells compared to normal reference cells) determines:
    a. The number and structure of chromosomes and

chromosomal material in tumor cells. > 50 chromosomes (hyperploidy) or a DNA index > 1.16 is favorable.

b. Certain chromosomal translocations are unfavorable.

2. Immunophenotyping is also useful and involves the determination of B- or T-cell lineage, with maturity or immaturity of cells

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19
Q

AML What are the Dx features?

A

Bone marrow aspirate –blasts confirm diagnosis (<25%)

Fab subtyping based on histology

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20
Q

AML What are the favourable Fx?

A

Favorable:

  1. chromosomes: t(8:21), inv(16), t(9:11), t(15:17),
  2. remission after 1 cycle,
  3. Fab-M4 w eosinophilia
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21
Q

AML What are the Rx phases?

A

1) Induction – goal is to attain remission (<5% blasts in marrow)
2) CNS prevention
3) Consolidation – goal is to decrease relapse
4) Intensification & BMT – high risk of mucositis, infection, prolonged bone marrow suppression
5) Aggressive supportive care, esp w blood products, empiric abx, anti-fungal, nutritional

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22
Q

AML What are the Sx?

A
  1. Marrow failure symptoms as in ALL
  2. Other symptoms seen in AML
    - Subcutaneous nodules or blueberry muffin lesions,
    - gingival hypertrophy,
    - DIC,
    - chloromas or granulocytic sarcomas (discrete masses associated with AML-M2);
    - more often see CNS symptoms
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23
Q

AML What are the unfavourable Fx?

A

Unfavorable:

  1. chromosomes: monosomy 7,
  2. initial WBC > 100,000,
  3. secondary AML,
  4. myelodysplasia
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24
Q

AML What is the epidemiology and etiology?

A

Epidemiology: 11% of all childhood leukemia

Etiology:

  1. Unknown, but several associated chromosomal abn,
  2. Syndromes (Bloom, Diamond-Blackfan, Down, Fanconi anemia, , Kostmann, Li-Fraumeni , NF-1, Schwachman-Diamond,
  3. environmental RFs (ionizing radiation, chemotherapy, organic solvents, PNH)
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25
Q

AML What is the prognosis of AML?

What is the remission rate from chemo alone?

What is the remission rate after BMT?

Why do you BMT these guys and not ALL?

A
  1. Remission in 80% of patients from chemo alone
  2. matched-sibling BMT after remission = 60-70% long-term survival;
  3. graft-versus-leukemia effect from allogeneic transplantation (not seen in ALL)
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26
Q

Aneurysmal bone cyst What are the features?

A

Reactive lesion of bone

Xray = cavernous spaces filled with blood and solid aggregates of tissue, mostly femur, tibia & spine

Biopsy necessary to confirm diagnosis; treated with excision; recurrence rate 20-30%

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27
Q

Anthracycline (Doxo/Dauno)

What is mechanism of action/excretion, uses, short and long term toxicity?

A

Extra S/E of doxorubicin

Hair loss, Hand/foot syndrome, u Skin discolouration

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28
Q

Ara C

What is mechanism of action/excretion, uses, short and long term toxicity?

A
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29
Q

Asparaginase What is mechanism of action/excretion, uses, short and long term toxicity?

A
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30
Q

Bleomycin

What is mechanism of action/excretion, uses, short and long term toxicity?

A
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31
Q

Blood formation How are RBCs, white cells and platelets made?

A
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32
Q

Bone tumor

A
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33
Q

Brain tumor How do you manage acute hydrocephalus?

A
  1. Definitive = divert CSF, or remove block
  2. Emergent = dexamethasone to reduce oedema
    - Can “hold” until gets to a neurosurgeon
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34
Q

Brain tumor How does acute hydrocephalus and cerebral edema present?

A

Common with aggressive tumours

Tumour may invoke oedema

Blockage of drainage channels – acute hydrocephalus

-Severe hydrocephalus gives cerebral oedema too

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35
Q

Brain tumor medulloblastoma what is the classification?

A

Pathology

  1. Classic
  2. Large cell/anaplastic
  3. Nodular/desmoplastic

Molecular

Group 1 – WNT

Group 2 – SHH

Group 3

Group 4

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36
Q

Brain tumor What is the commonest malignant brain tumor in children?

How does it present?

A

Medulloblastoma

Presents:

  1. Headache, Vomiting, Ataxia
  2. Cranial nerve palsies & Developmental regression
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37
Q

Brain tumors Epidemiology?

A
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38
Q

Brain tumors Etiology?

A
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39
Q

Brain tumors How do you Dx them?

A

Emergency!!

  1. Ophthalmology,
  2. neuroimaging (head MRI),
  3. neuroendocrine evaluation,
  4. lumbar puncture
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40
Q

Brain tumors What are the Sx (3/6)

A

Increased ICP – lethargy, irritability, macrocephaly, headache, papilledema, vomiting

Supratentorial/cortical – subtle personality/speech changes, motor weakness, sensory changes, seizures, reflex abnormalities, infants can present with early handedness(<1yr is pathologic)

Infratentorial – abnormal gait, torticollis, blurred vision, diplopia, nystagmus

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41
Q

Brain tumors What are the Sx (2nd 3/9)

A

Brainstem – gaze palsy, multiple cranial nerve palsies, UMN defects (hemiparesis, hyperreflexia, clonus)

Optic pathway – decreased visual acuity, Marcus Gunn pupil (RAPD), visual field defect

Suprasellar – neuroendocrine deficits, e.g. DI, galactorrhea, abn puberty, hypothyroidism

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42
Q

Brain tumors What are the Sx (3rd 3/9)

A

Diencephalic syndrome: FTT, emaciation, increased appetite, euphoria

Pineal region: Parinaud syndrome: paresis of upward gaze, nystagmus, eyelid retraction

Spinal cord: long nerve tract motor and/or sensory deficits, bowel/bladder deficits, back pain

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43
Q

Brain tumors What 4 categories constitute 80% of all pediatric brain tumors?

A

astrocytoma (Juvenile pilocytic and Diffuse),

PNET (medulloblastoma),

Ependymoma,

Craniopharyngioma

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44
Q

Brain tumors What are the Sx, Rx and Progx of the 4 common types?

A
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45
Q

Brain tumors What is the epidemiology and origin of the 4 common types?

A
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46
Q

Cancer etiology What % of childhood cancer are associated with a genetic and/or congenital condition?

A

15%

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47
Q

Cancer etiology What is the 1st mechanism that genetic conditions predispose to cancer?

A

1. Gene alteration disrupts normal genetic repair:

Ataxia telangiectasia = lymphoma

Bloom syndrome = leukemia;

Xeroderma pigmentosa = skin cancer;

2. Dysfunctional cellular growth:

Beckwith-Weidemann = Wilms tumor, hepatoblastoma, rhabdo, adrenal tumor;

MEN = Hepatic tumors;

Neurofibromatosis = optic glioma, CNS tumors & rhabdomayosarcoma

3. Inactivation of tumor suppressor gene:

Familial retinoblastoma & Li Fraumeni = osteosarcoma

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48
Q

Cancer etiology What is the role of cancer stem cells?

A
  • May have a role in certain malignancies such as CML, AML, ALL, gliomas, and breast cancer.
  • These tumor-initiating cells have self renewal and proliferative properties similar to nonmalignant stem cells.”
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49
Q

Cancer etiology What other factors cause cancer?

A
  1. Viruses

EBV – Burkitt’s , Hodgkin + T-cell lymphoma

HBV – Hepatocellular carcinoma

HCV – Hepatocellular carcinoma, splenic lymphoma

HPV – Cervical cancer

HHV-8 – Kaposi sarcoma

  1. Genomic imprinting (selective inactivation of ½ gene alleles) à Wilms tumor – loss of normal inactivation of maternal gene
  2. Telomerase (enzyme that adds telomeres on xsomes = “immortal” cell lines
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50
Q

Cancer etiology What 2 major gene classes are implicated in development of cancer?

A

1) Mutation that converts Proto-oncogenes into oncogenes. These mutations can be:
a. Amplification, e.g. N-MYC in neuroblastoma
b. Point mutation, e.g. 10q RET in MEN2
c. Translocation, e.g. Philadalphia xsome t(9:22) BCR/ABL in ALL
2) Mutation that disables Tumor suppressor genes

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51
Q

Cancer etiology Alterations in which cellular processes can cause cancer?

A

Cancer is a complex of diseases arising from alterations that can occur in a wide variety of genes.

Alterations in the following normal cellular processes can result in a malignant phenotype:

  1. signal transduction,
  2. cell cycle control, DNA repair,
  3. cellular growth and differentiation,
  4. translational regulation, senescence and apoptosis (programmed cell death)
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52
Q

Cancer predisposition syndromes Features of Lynch syndrome?

A

MLH1, MSH2, MSH6, PMS2

non-polyposis Colorectal carcinoma, endometrial, gliomas, ovary, ureteric

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53
Q

Cancer predisposition syndromes Features of Neurofibromatosis type 1?

A

NF1 gene – complex mutations

Benign nerve sheath tumours, gliomas, MPNST, plexiform neurofibroma

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54
Q

Cancer predisposition syndromes Features of Li Fraumeni Syndrome

A

Germline p53 mutation

Adrenocortical carcinoma, sarcomas, breast cancer, brain tumours

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55
Q

Cancer predisposition syndrome features of Gorlin Syndrome?

A
  1. PTCH mutation – SHH pathway mutation *GROUP 2*
  2. Naevoid basal cell carcinoma syndrome

Basal cell carcinomas

Medulloblastoma

Odontogenic cysts

Skeletal and cutaneous abnormalities

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56
Q

Carbplatin

What is mechanism of action/excretion, uses, short and long term toxicity?

A
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57
Q

Chemotherapy side effects Which ones are the most concerning?

A
  1. leukoencephalopathy after high-dose methotrexate therapy; 2. infertility in male patients treated with alkylating agents (e.g., cyclophosphamide);
  2. myocardial damage caused by anthracyclines;
  3. pulmonary fibrosis caused by bleomycin;
  4. pancreatitis caused by asparaginase;
  5. renal dysfunction due to ifosfamide, nitrosourea, or platinum agents; and
  6. hearing loss from cisplatin
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58
Q

Chemotherapy side effects

What are the most important side effects from:

Mtx

Alkylating agent

Anthracycline

Bleomycin

Asparaginase

Ifosfamide

Cisplatin

A

Mtx

Alkylating agent

Anthracycline

Bleomycin

Asparaginase

Ifosfamide

Cisplatin

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59
Q

Chemotherapy What are the Late Effects Assessment and Management for lung, urologic and liver?

A
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60
Q

Chemotherapy What are the Late Effects Assessment and Management for Renal and gonadal?

A
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61
Q

Chemotherapy What are the Late Effects Assessment and Management for secondary malignancies?

A
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62
Q

Chemotherapy What are the Late Effects Assessment and Management for heart, lung and thyroid?

A
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63
Q

Chemotherapy What are the Late Effects Assessment and Management for gonadal?

A
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64
Q

Chemotherapy What are the Late Effects Assessment and Management for secondary malignancies and other organs?

A
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65
Q

Chemotherapy What are the Late Effects Assessment and Management for CNS, Cardiac and hearing?

A
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66
Q

Cisplatin

What is mechanism of action/excretion, uses, short and long term toxicity?

A

Cisplatin:

Immediate side effects

Nausea/vomiting

Acute renal tubular dysfunction + Hypomagnesaemia

Long-term side effects

Hearing loss

Renal impairment with low GFR

Electrolyte disturbances with low Mg, âK

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67
Q

CML What are:

  1. What is the incidence of all childhood leukemia?
  2. What chromosome and translocation is present?
  3. How long is the chronic phase and what features and what acute phase may happen?
  4. How is CML Treated?
A
  1. 2-3% of all childhood leukemia;
  2. 99% w Philadelphia chromosome t(9:22) BCR-ABL
  3. 3-4 yrs of chronic phase (high WBC, low Hb, high Plt, splenomegaly) leading to a “blast crisis” phase
  4. Treated with busulfan, hydorxyurea & interpheron-alpha; stem-cell transplant the only cure
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68
Q

Cyclophosphamide

What is mechanism of action/excretion, uses, short and long term toxicity?

A
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69
Q

Dactinomycin

What is mechanism of action/excretion, uses, short and long term toxicity?

A
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70
Q

Downs ALL and AML

How much commn is acute leukemia in Downs kids?

Which leukemia is more common?

What is the long-term survival?

Which (AML vs ALL has better prognosis?

In Downs kids What is the sensitivity to methotrexate & other metabolites and toxicity?

A

20x increased frequency of acute leukemia;

ALL>AML except first 3 years of life

prognosis >80% long-term survival.

ALL prognosis slightly worse than others,

AML prognosis slightly better.

More sensitive to methotrexate & other metabolites – higher risk of toxicity

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71
Q

Downs Kids Transient myeloproliferative disorder

  1. What is theincidence in kids with Down syndrome?
  2. What are the clinical and lab features?
  3. What is the Management?
  4. What is the Chance of leukemia?
A
  1. Occurs in 10% of neonates with Down syndrome ; lasts up to 1 year
  2. High WBC, blasts, anemia, thrombocytopenia, hepatosplenomegaly.
  3. Management: usually supportive w transfusions
  4. Chance of leukemia: 20-30% develop acute leukemia by 3 yrs old
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72
Q

Etoposide What is mechanism of action/excretion, uses, short and long term toxicity?

A
73
Q

Ewing sarcoma Epidemiology and Etiology?

A

Second decade, Highest risk during adolescent growth spurt

Undifferentiated bone sarcoma that may arise from soft tissue

74
Q

Ewing sarcoma What are the DDx?

A

Other small round cell tumors –

neuroblastoma,

rhabdomyosarcoma,

Non-Hodgkin lymphoma

histiocytosis,

osteomyelitis

75
Q

Ewing sarcoma What are the Dx features on Xray?

A

Onion skin

76
Q

Ewing sarcoma What are the Rx options?

A

Chemotherapy

+/- surgery

+/- radiotherapy

77
Q

Ewing sarcoma What are the Sx?

A

Pain, swelling, limitation of motion, localized tenderness

Spinal cord compression if tumor is paraspinal or vertebral

Often have systemic symptoms – fever, weight loss

78
Q

Ewing sarcoma What is the progx?

A
79
Q

Ewings sarcoma Where does it metastasize to?

A

Lungs, Lymph nodes, bone marrow

80
Q

Ewings sarcoma Which are the most common bones involved?

A

Long bones – diaphysis

Axial skeleton & chest wall

81
Q

Febrile neutropenia What are the answers?

  1. Importance in emergency events for children with cancer?
  2. Importance in mortality cause?
  3. Why are cancer patients prone to infections?
A
  1. Commonest oncological emergency
  2. Commonest non-cancer cause of death
  3. Reason for increased risk: breakdown” of infective protection mechanisms
  • Low neutrophils/phagocytes
  • Impaired mucosal barriers
  • Indwelling catheters
  • Impaired immune response
82
Q

Febrile neutropenia What are the common organisms?

A

Gram positive (most common)

Staph aureus & epidermidis, Strep viridans

Gram negative (can be life threatening)

Pseudomonas

E coli

Klebsiella

Fungal

Candida

Cryptococcus

83
Q

Febrile neutropenia What is the critical ANC level?

How is the clincal presentation differnt from normal kids?

A
  1. Patients are at particular risk if the ANC is < 500 but < 1000 also incur some risk
  2. Lack of neutrophils leads to a loss of the inflammatory response and fever may be the only manifestation of infection and the need for empiric abx
84
Q

Febrile neutropenia When should you start thinking of an antifungal?

[Z1]

A

Patients without an identifiable source and persistent fever after > 5 days with progression of symptoms, the addition of an antifungal agent is generally warranted

85
Q

Fibroma What are the features?

A

Occurs in 40% of children > 2yrs old; represent ossification defect, usually discovered incidentally

Occasional pathologic fractures; xray = lucency, may be multilocular, long axis runs parallel to bone

Spontaneous regression after skeletal maturity; excision if >50% of bone diameter

86
Q

Germ cell tumors What are the answers to?

What are 2 syndromes and 4 signs that make germ cell tumours more common?

A
87
Q

How do you manage electrolytes problems in TLS?

A
  1. Px with HR K+ or HOCa+ -cardiac monit & lytes q 4hrly
    - K+ > 7-7.5 or or widening of QRS complex requires immediate intervention.9,10 Standard therapies to lower the potassium

Rx for HR K+:

a. loop diuretics, insulin and glucose, inhaled bagonists, polystyrene sulfate, and calcium gluconate for

symptomatic hyperkalemia or electrocardiographic changes.

b. hemodialysis.
2. Hyperphosphatemia is treated with phosphate

binders.

  1. The presence of secondary hypocalcemia can be

life-threatening and generally necessitates the use of

hemodialysis. Asymptomatic hypocalcemia should not be

treated with calcium administration because of the risk of

increasing calcium phosphate deposition in the rena

88
Q

Ifosphamide

What is mechanism of action/excretion, uses, short and long term toxicity?

A
89
Q

Infant leukemia What are the answers to the folowing?

  1. What % of childhood leukemia cases occur < 1 year?
  2. What is the ALL:AML ratio ?
  3. What are thePoor prognosis Fx?
  4. What is the Rx?
A
  1. 2% of childhood leukemia cases occur < 1 year
  2. ALL:AML ratio 2:1
  3. Poor prognosis – hyperleukocytosis, organomegaly, CNS disease, leukemia cutis (subcut nodules), diffuse pulmonary infiltration
  4. Intense chemotherapy + stem cell transplant
90
Q

How does Rasburicase work?

A

-Urate oxidase oxidizes uric acid to the water-soluble and

readily excreted metabolite allantoin.

  • Rasburicase (urate oxidase) reduces uric acid levels without

increasing the levels of uric acid precursors, and therefore

does not risk xanthine nephropathy.

91
Q

Lymphoma Epidemiology?

What is the % of childhood cancer;?

What are the risk factors?

A
  1. 3rd most common type of childhood cancer;
  2. 6% of childhood cancers; peak in adolescence; rare in children < 10 years
  3. Increased risk with immunodeficiency,
    - acquired (HIV) &
    - congenital (e.g. Ataxia-Telangiectasia)
92
Q

Lymphoma Hodgkins How do you Dx and rx SVC syndrome?

A
93
Q

Lymphoma What are the risk factors?

A
  1. Unknown
  2. genetic (risk 100-fold for monozygotic twin)
  3. infectious (EBV, CMV, HHV-6) factors
  4. Post chemo and radiotherapy
94
Q

Lymphoma What are the symptoms?

A
  1. Painless, non-tender, firm cervical lymphadenopathy;
  2. B symptoms (fever, wt loss, night sweats)

3. Mediastinal adenopathy common -

  • airway compromise,
  • SVC syndrome,
  • pleural/ pericardial effusion
95
Q

Lymphoma Hodgkins What is the characterisitc histology finding?

A

Reed Sternberg cells (owls eye cell)

96
Q

Lymphoma How do you Dx it?

A
  1. Chest x-ray
  2. excisional lymph node biopsy -Reed-Sternberg cell– clonal B cells
97
Q

Lymphoma staging Based on this PET scan, what Stage

would Melissa’s lymphoma be?

A

Stage 3

98
Q

Lymphoma What are th types seen on biopsy?

A

Types:

  1. nodular,
  2. lymphocyte-rich,
  3. mixed cellularity,
  4. anaplastic large cell,
  5. lymphocyte-depleted
99
Q

Lymphoma What are the causes of mediastinal masses?

Anterior Mediastinal mass?

Middle Mediastinal mass?

Posterior Mediastinal mass?

A

Anterior Mediastinum:

  1. Lymphoma,
  2. Thymoma,
  3. Thyroid

Middle Mediastinum

  1. Lymphdenopathy –
  2. lymphoma, metastatic disease,
  3. Germ cell tumour

Posterior Mediastinum

  1. Neuroblastoma, neurofibroma,
  2. Ewings,
  3. Rhabdomyosarcoma
100
Q

Lymphoma What are the favourable and non favourable factors?

A

Unfavorable:

  1. tumor bulk,
  2. stage at diagnosis,
  3. B symptoms = 90% survival
  4. Relapse > 12 mos after therapy = 60-70% survival

Favorable prognostic factors

+ early-stage disease = 95% survival

101
Q

Lymphoma What is the Ann Arbor staging ?

A

Ann Arbor Staging - Lymphoma

Stage 1

Single nodal group

Stage 2

2 or more nodal groups on SAME side of diaphragm

Stage 3

2 or more nodal groups on BOTH sides of diaphragm

Stage 4

Involvement of extra-lymphoid tissues

B- Symptoms

Present vs Absent

102
Q

Lymphoma What is the Rx?

A
  1. Combined chemotherapy & radiotherapy

Chemo eg:

ABVD”

(A) Doxorubicin

Bleomycin

Vincristine

Dacarbazine

  1. Autologous stem cell transplant in those who never achieve remission or relapse after < 12mos
103
Q

Lymphoma Non-Hodgkin What is the Epidemiology and etiology?

A

Epidemiology

  • 60% of all lymphomas in children & adolescents; 10% of all malignancies
  • >70% present with advanced disease. RFs include immune deficiency, viral infection, syndromes.
  • Represents 50% of all pediatric malignancies in equatorial Africa (endemic Burkitt’s lymphoma)
  • Usually in younger children

Etiology:

EBV present in 95% of cases of endemic (African) Burkitt’s lymphoma

104
Q

Lymphoma Non-Hodgkin How do you Dx it? What are the 4 types?

A
  1. Burkitt 40% (small nocleaved B cell),
  2. Lymphoblastic 30% (TCell),
  3. Diffuse large B/T-cell 20%,
  4. Anaplastic large cell 10%
105
Q

Lymphoma Non-Hodgkin What are the Sx?

A

Masses: head & neck, intrathoracic, mediastinal, abdominal, cutaneous. Can spread to marrow.

Site specific findings: SVC syndrome, airway compression, intussusception, spinal cord compression, TLS

106
Q

Lymphoma Non-Hodgkin. What is the prognosis?

A

90-95% survival for localized disease;

60-90% with advanced disease

107
Q

Lymphoma Non-Hodgkin What is the Rx?

A

Systemic & intrathecal chemotherapy

Surgery for diagnostic biopsy; rarely for debulking large masses

108
Q

Methotrexate

What is mechanism of action/excretion, uses, short and long term toxicity?

A
109
Q

Monitoring blood tests?

A
  • Get baseline analyte values prior to Rx:

uric acid, electrolytes, LDH, and creatinine,

HIGH RISK: every 4 to 6 hours

INTERMEDIATE RISK: every 6-8 hours

LOW RISK: every 24 hours

N.B.

  1. uric acid levels may remain normal in patients with an emerging TLS ON ALLOPURINOL
  2. For rasburicase, laboratories must develop

protocols to collect uric acid samples in prechilled tubes that

are placed immediately on ice and kept on ice throughout

rapid transport to the laboratory for immediate run on the

instrument to prevent rapid in vitro uric acid breakdown,

and a spuriously low assay result.

110
Q

Neuroblastoma Epidemiology?

A

Epidemiology

3rd most common pediatric cancer ~8%; 90% diagnosed by 3yrs old

Most common neoplasm in infants - 1/3 of cases

111
Q

Neuroblastoma How do you Dx it?

A
  1. Abdo and chest CT
  2. BMA w/ biopsy (bilateral!)
  3. 95% have elevated urine HVA & VMA
  4. Bone scan/MIBG scan
  5. Mass biopsy
112
Q

Neuroblastoma What are the DDx?

A

Other small round cell tumors –

  1. rhabdomyosarcoma,
  2. Ewing sarcoma,
  3. Non-Hodgkin lymphoma
113
Q

Neuroblastoma What are the Sx and signs?

What are the symptoms that present with each location?

A

Can develop at any site of sympathetic nervous tissue, S&S reflect location & extent of tumor

  1. Thoracic: dyspnea/horner syndrome
  2. Abdominal: abdo pain, vomiting, palpable mass
  3. Pelvic: constipation and urinary retention
  4. Paraspinal: back pain, limp, leg weakness, hypotonia

Where can neuroblastoma develop?

114
Q

Neuroblastoma What are the Sx and signs?

Where does it like to spread and 2 examples?

What are 4 paraneoplastic syndromes?

A

Funny METS to bone marrow and skin:

  1. Periorbital bruising (raccoon eyes),
  2. blue berry muffin spots (skin nodules)

Paraneoplastic syndrome:

  1. Opsoclonus-myoclonus,
  2. intractable watery diarrhea,
  3. hypertension,
  4. sweating
115
Q

Neuroblastoma What is the etiology?

A
  1. Embryonal cancer of the peripheral sympathetic nervous system; variable neural differentiation from undifferentiated (neuroblastoma) to mature ganglion (ganglioneuroma)
  2. Amplification of n-MYC gene, 11q and 1p deletions associated with poor outcome
116
Q

Neuroblastoma What is the prognosis?

A

Factors include

  1. amount of stroma,
  2. degree of differentiation,
  3. presence of enlarged nucleoli,
  4. mitosis index

Depends on stage:

stage 1 > 90%;

stage 2 80%,

stage 3 50%;

stage 4 30%;

stage 4S (<1yr) >80%

117
Q

Neuroblastoma What is thr Rx?

A

Surgery, chemotherapy, radiotherapy

4S = special!

  1. <1year of age,
  2. localized primary tumour w/ spreading limited to liver, skin, bone marrow.

Very good prognosis!

118
Q

Osteoblastoma What are the features?

A

Locally destructive, progressively growing bone lesion, prefers vertebrae

Insidious onset of dull, aching pain; may have neurological deficits at presentation

Biopsy necessary to confirm diagnosis; treated with excision

119
Q

Osteochondroma (exostosis)

What does osteochondroma look like on xray? (mushroom according to Amanda)

A

Arise in metaphysis of long bone, esp distal femur, proximal humerus, proximal tibia

Bony, painful mass; Xray = stalks or broad-based projections from bone surface

Malignant degeneration in 1% of adults, surgical resection if symptomatic

120
Q

Osteoid osteoma What are the features?

A

Unremitting, gradually increasing pain that is worse at night, relieved by analgesic

Most common sites proximal femur & tibia; vertebral lesion = scoliosis or neurological deficits

Xray = round or oval lucency surrounded by ring of sclerosis

Surgical resection; some resolve spontaneously with skeletal maturity

121
Q

Osteosarcoma Epidemiology?

What 2 syndromes are asociated with it?

A

Malignant spindle cell tumour.

Second decade, Highest risk during adolescent growth spurt

Etiology:

hereditary retinoblastoma,

Li-Fraumeni

122
Q

Osteosarcoma How do you Dx it?

What are the characteristic X-ray findings in osteosarcoma

where does osteosarcoma metastasize to?

A
  1. Radiology:
    a. Xray = sunburst pattern (ossification of soft tissue) & Codman triangle (cortical elevation) - primary site

b. MRI primary site & CT chest

u Bone scan

  1. Biopsy of tumor & metastatic workup to lung + bone marrow
  2. Blood work up: Normal CBC and chemistry, but may have elvated LDH & ALP
123
Q

Osteosarcoma What are the DDx?

What is your differential for a lytic bone lesion?

A

Lytic bone lesion ddx =

histiocytosis,

Ewing sarcoma,

lymphoma,

bone cyst

124
Q

Osteosarcoma What are the metastatic spread patterns?

A

Indicative of highly aggressive or advanced tumour

  1. Local invasion
  2. Lymphoid spread -Locoregional, distant
  3. Haematogenoous spread

u Distant sites (e.g. lungs, brain, bone marrow)

  1. CNS tumours & CSF dissemination
125
Q

Osteosarcoma What are the Sx?

A
  1. Pain, limp, swelling most common. May be attributed to sports injury or growing pains
  2. Deep bone pain that causes nighttime awakenings; May also see decreased ROM, joint effusion, tenderness, warmth

Normal CBC and chemistry, but may have elvated LDH & ALP

Locations: long bone metaphysis – 50-80% are in proximal tibial / distal femur

126
Q

Osteosarcoma What is the progx?

A

75% survival in non-metastatic disease;

25% survival in metastatic (lung) disease

127
Q

Osteosarcoma What is the Rx?

A

1. Neo-adjuvant chemo (Cisplatin, Doxorubicin, Methotrexate (high dose)..then

  1. complete surgical resection (ESSENTIAL) (may need amputation) ….then more chemo

Note: this tumour is more chemo resistant

**NOT radiosensitive

128
Q

Osteosarcoma Which are the most common bones involved?

A

Ends of long bones (usually solitary)

Pelvis, others

129
Q

Prednisone What is mechanism of action/excretion, uses, short and long term toxicity?

A
130
Q

Radiation Rx What are the long-term side effects?

A
  1. Growth delay (cranial or vertebral irradiation)
  2. Endocrine (midbrain irradiation)
  3. Pulmonary or cardiac insufficiency (chest)
  4. Strictures and adhesions (GI)
  5. Infertility (pelvic
131
Q

Radiation Rx What are the Short-term side effects?

A
  1. Dermatitis of adjacent tissues
  2. Mucositis anywhere from mouth to anus
  3. Somnolence (Cranial irradiation)
  4. Alopecia
132
Q

Radiotherapy What are the Late Effects Assessment and Management for CNS and eye?

A
133
Q

Retinoblastoma Diagnosis?

What 2 investigations must you do?

A

Opthalmology,

MRI to r/o intracranial tumor

134
Q

Retinoblastoma Epidemiology?

A

BILATERAL:

Rare, bilateralinvolvement highest if presents <1yr old

25% bilateral/hereditary

UNILATERAL:

60% unilateral/non-hereditary;

15% unilateral/hereditary;

135
Q

Retinoblastoma Etiology

A

Hereditary form due to 2-hit hypothesis:

1st inherited germ cell mutation then

2nd somatic mutation

Increased risk in Li-Fraumeni

136
Q

Retinoblastoma How is it managed?

What are the Primary + secondary goals?

What are your treatment options?

What do you need to do for first degree relatives?

A

Primary goal = cure; secondary goal = preserving vision

  1. Enucleation (eyeball resection),
  2. chemotherapy + focal therapy (laser or cryo),
  3. radiation
  4. All 1st-degree relatives need to be screened
137
Q

Retinoblastoma Progx?

A

95% cure rate; poor prognosis if metastatic

138
Q

Retinoblastoma Sx ?

A

Leukocoria– white papillary reflex

Strabismus;

with advanced disease = orbital inflammation, hyphema, pupil irregularity

139
Q

Retinoblastoma What are the DDx?

What other 4 things can give you leukoria?

A

Cataract,

retinopathy of prematurity,

visceral larva migrans,

coloboma

140
Q

Retinoblastoma What is the relevance of the RB1 gene carrier?

A
  1. If RB1 gene carrier – depending on the mutation 10-100% risk of retinoblastoma, and
  2. increased risk for other tumours (osteosarcoma, glioma, glioblastom multiforme, melaninoma, lung, bladder) and need to have optho exams until 9 yo
141
Q

Rhabdomyosarcoma How do you Dx it?

A

Requires high index of suspicion

Biopsy required, cytogenetics to determine prognosis

142
Q

Rhabdomyosarcoma How do you Rx them?

A
  1. Neo-adjuvant chemotherapy then
  2. complete resection if possible,
  3. followed by XRT & chemo
143
Q

Rhabdomyosarcoma What are the DDx?

What 3 other small round tumors are DDx?

A

Other small round cell tumors –

neuroblastoma,

Ewing sarcoma,

Non-Hodgkin lymphoma

144
Q

Rhabdomyosarcoma What are the Sx?

A
  1. retroperitoneal & other 35%
  2. Head & neck 25%,
  3. genitourinary tract 22%,
  4. extremities 18%,

Mass that causes displacement or obstruction of normal structures – symptoms are specific to site

Orbit = proptosis,

middle ear = chronic otitis media,

larynx = hoarseness,

bladder = hematuria

145
Q

Rhabdomyosarcoma What is the prognosis?

A

80-90% survival with complete resection;

70% with incomplete resection,

25% with disseminated

146
Q

Rhabdomyosarcoma Where do these tumors arise from?

A

Arise from striated skeletal muscle

147
Q

Rhabdomyosarcoma What syndrome is associated with this? Epidemiology?

(Soft tissue sarcomas)

A

3.5% of childhood cancers, increased risk in neurofibromatosis

148
Q

Role of high K+ in TLS?

A

K+ is an ICF electrolyte regulated through renal excretion.

TLS HR K+ …cardiac dysrhythmias and sudden death.

Ca+ stabilizes the cardiac membrane, Low Ca+ exacerbates HR Ca+- induced cardiotoxicity and cardiac dysrhythmias.

HR Ca+ > 7.0 mEq/L(hyperkalemic emergency) and can be potentiated by ongoing TLS, Low Ca+, and/or renal failure

149
Q

Role of hydration in TLS?

A
  1. Maintaining a high urine output with adequate hydration
    - most important aspect of TLS prevention and

management, because this improves renal perfusion and

glomerular filtration, and minimizes acidosis, all of which

serve to prevent the precipitation of uric acid and calcium

phosphate crystals in the renal tubules.

  1. intermediate-to-high risk & for established TLS: hyperhydration with NS to maintain equal

fluid balance and a high urine output.

  1. K+, PO4, and Ca+ should NOT be added to the hydration solution.
  2. Monitored Volume status to prevent volume overload, particularly in patients with renal failure or cardiac dysfunction, and diuretics may be necessary to maintain urine output.
150
Q

Role of Rasburicase in TLS?

What are the benefits and limitations of rasburicase?

A

Preferred prophylactic agent for patients at high risk for TLS and the treatment of choice for established TLS.

Benefits:

  1. rapidly reduces uric acid levels and breaks down deposits of uric
  2. Reduces PO4 + serum creat levels
  3. does not require renal dosing;

however, there is a risk for

Problems:

  1. severe hypersensitivity reactions (anaphylaxis)

particularly with repeat dosing

  1. contraindicated in pregnant/lactating F & G6PD Def’y , (severe hemolytic anemia) - Screen for G6PD
151
Q

Role of Acute Renal Injury in TLS?

A

AKI - independent predictor of short + long-term TLS mortality

Mecnms:

Crystal-dependent and crystal-independent mechanisms.

  1. Crystal-dependent mechanisms
    - obstructive uropathy.
    - Decreased urinary outputcan result in volume overload and cardiac failure, which can exacerbate crystal precipitation.

Urine alkalinization to > pH 6.5 not in use now

2. Crystal-independent mechanisms

-loss of autoregulation, renal vasoconstriction,

and local inflammation

-Tumor lysis–induced hypercytokinemia can result in hypotension, systemic inflammation, and multiorgan failure

152
Q

Secondary malignancyWhat is the incidence and how should you screen for it?

A
  1. The risk appears to be cumulative, increasing by about 0.5% per year, resulting in approximately a 12% incidence at 25 yr after treatment.
  2. Patients should be examined annually, with particular attention to second malignancies.”
153
Q

Supportive care How do you manage the immunocompromised host?

A
154
Q

Supportive care Miscelleneous problems:

A
155
Q

Supportive care WHow do you treat each of the following oncological emergencies?

  1. Increased ICP

Spinal cord compression

Hyperleukocytosis

Tracheal compression

SVC syndrome

Tumor lysis syndrome

SIADH

GVHD

A
156
Q

Supportive care What organ toxicities are of concern?

A
157
Q

Techniques for safe administration of chemotherapy What are 4 methods?

[Z1]List 6 techniques for safe administration of chemotherapy.

A
  1. Safe storage and labelling
  2. Appropriate handling and protective equipment
  3. Careful identification of patient and dosage (double checked) AND ROUTE
  4. Appropriate administration
158
Q

How does Allopurinol work?

A

Allopurinol blocks uric acid synthesis leading

to accumulation of the uric acid precursors xanthine and

hypoxanthine, of which hypoxanthine is more soluble and

more easily excreted than uric acid.

159
Q

How do Allopurinol and Rasburicase work?

A
160
Q

Pathogenesis- Hyperuricemia

A

Hyperuricemia with hyperphosphatemia, can lead to acute kidney injury.

Mecanism:

-Purine containing nucleic acids released

into the serum are catabolized to uric acid and, when

present in excess, surpass the renal tubular capacity for uric

acid excretion.

-Uric acid is poorly soluble in water, and the solubility is markedly reduced in the physiologically acidic environment of the distal tubules and collecting system. Thus, uric acid crystals readily precipitate when concentrated in the renal circulation, leading to renal tubule obstruction and obstructive uropathy, with compromised glomerular filtration and reduced

urine output.

161
Q

TLS What are the CLINICAL FEATURES of TLS?

Timing?

Symptoms (4)?

Cplxns (3)?

A

Timing:

  1. Within 12 to 72 hr > initiation of cytotoxic therapy
    - Can occur 72 hrs
  2. Can occur before Rx

Symptoms include:

  1. nausea, vomiting, diarrhea, anorexia,
  2. weakness, lethargy and syncope.
  3. hematuria,
  4. muscle cramps, tetany,

Cplxns:

  1. Cardiac dysrhythmias,
  2. seizures,
  3. sudden death
162
Q

What is the Diagnostic Criteria for TLS

(Cairo Bishop grading for Lab & Clinical TLS)

A
163
Q

What is Tumor lysis syndrome (TLS)?

What are the lab findings and Cplxns?

A

Deftn: Acute, life-threatening disease among children that is associated with the initiation of cytoreductive therapy in the treatment of malignancy.

Characteristic findings:

Raised: uric acid, phosphate, K+, urea, creatinine

Low: Ca+,

Cplxns: cardiac arrhythmia, seizures, renal failure,

and sudden death.

164
Q

Tumor markers What tumors produce the following tumor markers?

  1. Alpha-fetoprotein
  2. Beta-HCG
  3. Urine catecholamines
A
  1. Alpha-fetoprotein: Hepatoblastoma, germ cell tumours
  2. Beta-HCG: Germ cell tumours
  3. Urine catecholamines: Neuroblastoma, Phaeochromocytoma
165
Q

Unicameral bone cyst What are the features?

A

Usually occur b/w 3-12 yrs, fluid-filled lesion, mostly asymptomatic until pathologic fracture

Xray = solitary, centrally located lucency, proximal humerus or femur

Treatment with aspiration and injection of bone marrow or steroids

166
Q

Vinblastine What is mechanism of action/excretion, uses, short and long term toxicity?

A
167
Q

Vincristine

What is mechanism of action/excretion, uses, short and long term toxicity?

What are the toxic effects?

A

Special side effects: (vincristine, vinblastine, vindesine)

Vincristine

Vinca alkaloid

Severely neurotoxic

Severe vesicant – extravasation damage

Intrathecal administration ( WILL DIE….)

Severe demyelination

Severe encephalopathy

Severe pain..extravasation burn..

Death, usually

168
Q

What are the classes of chemotherapy?

A
  1. Antimetabolites: MTX, 6 MP, Ara C
  2. Alkylating agents: cyclophosphamide, ifosphamide, cisplatin, carboplatin
  3. Antibiotics: Anthracyclines (Doxorubicin, daunomycin), Dactinomycin, Bleomycin

4. Plant alkaloids; Vincristine, vinblastine, etoposide VP 16

5. Miscellaneous: Prednisone, PAG Asparaginase

Alkylating agents

u e.g.

u Anthracyclines

u e.g. doxorubicin

u e.g. Methotrexate

u Antibiotics

u e.g. bleomycin

u Enzymes

u e.g. asparaginase

u Microtubule inhibitors

u e.g. vincristine

u Nucleoside analogues

u e.g. 6-mercaptopurine

u Platinum agents

u e.g.

u Small-molecule inhibitors

u e.g. imatinib

u Topoisomerase inhibitors

u e.g. etoposide

169
Q

What are the indication for hemodialysis in TLS?

A
  1. refractory volume overload
  2. AKI: oliguria or anuria,
  3. persistent HR K+, HR uric acid despite above measures
  4. hyperphosphatemia-induced symptomatic hypocalcemia, and a calcium phosphate product of greater than 70 mg2/dL

N.B. Prophylactic hemodialysis in patients at risk for TLS may be appropriate in the setting of preexisting renal disease or acute renal injury at presentation, and further studies are needed

170
Q

What are the Risk factors in TLS?

A
171
Q

What is the role of Allopurinol in TLS?

A

“Preferred prophylactic in low-to-intermediate risk patients”

  • *Limitations:**
    1. As it it does not break down preexisting uric acid, urate nephropathy can develop in the 3 days it takes to have a therapeutic effect. SO is NOT the preferred agent in the presence of hyperuricemia.

Furthermore, xanthine nephropathy must be considered in patients who develop TLS while receiving appropriate prophylactic therapy with allopurinol.

  1. allopurinol is excreted by the kidneys and must be dose reduced or discontinued in patients with renal insufficiency.
  2. hypersensitivity reactions have occurred. Asian populations have a higher frequency of the HLAallele, which is associated

with severe adverse cutaneous reactions with allopurinol

use.

  1. because allopurinol reduces purine degradation,

chemotherapeutic agents, such as 6-mercaptopurine

and azathioprine, must be dose reduced by 50% to 70%

when concurrently administered.

N.B. Any patient who develops TLS while receiving allopurinol prophylaxis should be switched to rasburicase

172
Q

What is the role of Hyperphosphatemia and Hypocalcemia in TLS?

A
  • Tumor cells have higher (PO4) than their normal cells
  • In TLS renal tubular excretion of (PO4) becomes saturated. - excess serum phosphate
  • Excess (PO4) binds to Ca+– low (Ca) + Ca PO4 deposition - - Low (Ca) Sx:
  • Arrythmias, seizures, Nephrocalcinosis -renal tubulular nephrolithiasis and cause or further provoke an obstructive

uropathy.

173
Q

What is the Cairo-Bishop Grading of Clinical Tumor Lysis Syndrome (TLS)?

A
174
Q

Wilms tumor Epidemiology ?

How often is unilat vs bilat?

A

Epidemiology

6% of pediatric cancers, usually between 2-5 years; unilateral 90%

Etiology:

Most sporadic; 1-2% AD;

increased risk in syndromes

  1. Beckwith-Wiedemann
  2. Denys-Drash – Glomerulopathy/Pseudohermaphrodism
  3. Neurofibromatosis
  4. Sotos
  5. WAGR- (W-ilms, A-niridia, G- GU anomalies, R- MR,
175
Q

Wilms tumor Etiology?

A

Complex mixed embryonal tumor

176
Q

Wilms tumor How do you Dx it?

A
  1. CT scan: Diagnostic imaging to confirm intrarenal origin & determine local & m_etastatic spread_ ( claw sign)
  2. Biopsy if diagnosis uncertain (Look at both kidneys!) - risk of biopsy!!
177
Q

Wilms tumor How do you Rx it?

A

Nephrectomy, chemotherapy, radiotherapy

178
Q

Wilms tumor Sx and Signs?

A

1. Renal specific Sx:

a. Hematuria 25%, hypertension (renal ischemia),
b. Mass effect:
- Abdominal mass, generally discovered fortuitously while parent bathing child
- rapid abdominal enlargement (hemorrhage into renal pelvis),
2. develop acquired bleeding diasthesis (VWD)
3. polycythemia

179
Q

Wilms tumor What is the progx?

A
  1. Depends on DNA content;
  2. Staging:

>60% survival across all stages;

>90% survival if stages 1-3

PEDIATRICS EXAM REVISION (56 decks)

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