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peds 2 > oncology > Flashcards

oncology Flashcards

1
Q

most common cancer in children, accounting for 30% of all childhood malignancies

A

acute leukemia (mostly ALL)

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2
Q
  • Second most common of all childhood malignancies
  • most common peds solid organ tumor
A

Primary malignant CNS tumors

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3
Q

most prevalent solid tumor in kids outside of the cranium

A

neuroblastoma

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4
Q

age group at risk for neuroblastoma, ALL, osteosarcoma, ewing sarcoma, hogdkins

A
  • 0-2 years: neuroblastoma
  • 2-5 years: ALL
  • 10-15 years: osteosarcoma, Ewing sarcoma
  • 15-19 years: Hodgkin lymphoma
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5
Q

top 3 most common cancers in kids

A
  1. leukemia
  2. CNS tumors
  3. lymphoma
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6
Q

trisomy 21 increases risk of ___

A

leukemia

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7
Q

fanconi anemia increases risk of ____ and ____

what two cancers

A

AML
solid tumors

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8
Q

what disorder is associated w/ increased risk of all these cancers?

  • Leukemia
  • Adrenocortical carcinoma
  • CNS tumors
  • Rhabdomyosarcoma
  • Osteosarcoma
A

Li fraumeni syndrome

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9
Q

Von hippel-lindau dz increases the risk of what 3 cancers

A
  • Retinal and CNS Hemangioblastoma
  • Clear cell renal cell carcinoma
  • Pheochromocytoma
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10
Q

The overall risk of malignancy is up to 4x higher than that of the general population with which predisposition disorder?

A

neurofibroma type 1 (NF1)

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11
Q

2 common tumors with NF1

A

neurofibroma; mostly benign
optic pathway glioma

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12
Q

3 common tumors associated with NF2

A
  • vestibular schwannoma
  • meningioma
  • spinal tumors
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13
Q

Consecutive genetic lesions results in arrest in development and abnormal proliferation; more in B cell
* Aberrant lymphoblast cell proliferation and survival are hallmarks

this describes the pathogenesis of what cancer

A

ALL

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14
Q
  • age 2-5
  • nonspecific sx
  • bone pain/refusal to bear weight
  • fever, pallor, bruising, wt loss, hepatosplenomegaly, etc
  • CNS & testicular involvement and mediastinal mass
A

ALL

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15
Q

procedure to evaluate the presence of leukemic blasts in the CSF; can give first dose of intrathecal chemotherapy this way too

A

lumbar puncture

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16
Q

gold standard for dx of ALL

A

bone marrow biopsy
over 25% blasts in marrow is leukemia

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17
Q

under 25% blasts in marrow is indicative of …

A

lymphoma

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18
Q

tx of ALL

A
  • multi-drug remien + CNS directed IT chemo (to prevent leukemic meningitis)

takes 2.5 yrs to complete

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19
Q

3 goals, post-induction for ALL

A
  • Prevention of leukemic regrowth
  • Reduction of residual tumor burden
  • Prevention of the emergence of drug-resistance in the remaining leukemic cells
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20
Q

genetic changes in the HPC that alter hematopoietic proliferation and differentiation so theres a lot of abnormal, immature myeloid cells in the bone marrow and peripheral blood

A

AML

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21
Q

which cancer has auer rods + blue ring outside

A

AML

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22
Q
  • nonspecific sx
  • Gingival hypertrophy, chloroma
  • bleeding/DIC
A

AML presentations

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23
Q

labs in AML vs ALL

A
  • AML: Elevated D-dimer, PTT, and PT/INR & Decreased fibrinogen
  • ALL: low ANC, high LDH & uric acid
  • BOTH: thrombocytopenia
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23
Q

between AML and ALL which one has scant cytoplasm

A

ALL

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24
Q

what post-remission therapy is offered to children with high-risk cytogenetics in first CR or patients with treatment-related AML

A

allogenic BMT

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25
Q

tx of APML

A

all-trans retinoic acid (ATRA) to reduce risk of DIC; started if APML is suspected

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26
Q
  • Clonal transformation of cells of B-cell origin
  • Pathognomic bi-nucleated Reed-Sternberg cells
A

hodgkins lymphoma pathogenesis

bimodal age distribution and more in females

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27
Q
  • rubbery, firm lymphadenopathy
  • pruritis, fatigue, anorexia
  • B symptoms
  • mediastinal mass esp in kids over 12 yo w/ associated sx
A

Hodgkins lymphoma

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28
Q

what are the B symptoms

A
  • night sweats x 1 month
  • weight loss (over 10% in 6 mo)
  • persistent unexplained fevers (over 38 C) x 1 month
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29
Q

6 associated sx of mediastinal mass

A
  • SVC syndrome
  • dysphagia
  • dyspnea
  • orthopnea
  • cough
  • stridor
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30
Q

3 imaging modalities used in HL evaluation

A

CXR
CT w/ contrast
PDG-PET CT skull base to thigh

31
Q

gold standard for HL diagnosis

A

excisional biopsy bc needle doesnt give enough tissue
aspirates for select patients

32
Q

lugano staging of HL (1-4)

A
  1. localized; single node or organ
  2. 2+ nodes on same side of diaphram
  3. 2+ nodes above & below
  4. widespread
33
Q

low vs intermediate vs high risk chemo tx of HL

A
  • low: 2-4 cycles +/- radiation
  • intermediate: 2-6 cycles + response-based radiation
  • high: 3-5 cycles + response-based radiation
34
Q

5th most common diagnosis of pediatric cancer in children < 15 years

A

NHL

35
Q

Congenital and acquired immunodeficiency syndromes and DNA damage repair syndromes place affected patients at risk for this kind of cancer..

includes HIV, WAS, CVID

A

NHL

36
Q
  • painless lymphadenopathy (most common)
  • mediastinal mass

this descirbes what kind of cancer

A

NHL

37
Q
  • painless lymphadenopathy (most common)
  • mediastinal mass
    AND
  • belly pain similar to appendicitis
  • abdominal mass
  • sick
  • intussusception (part of intestine slides into another part)

what kind of cancer (specific)

A

Burkitt lymphoma

38
Q

3 imaging modalities to evaluate NHL

A
  • CT
  • PDG-PET scan
  • skeletal scintigraphy for bone involvement
39
Q

2 procedures to evaluate for NHL

A
  • bone marrow biopsy
  • lumbar puncture
40
Q

tx for burkitt lymphoma

A

multi-agent chemo +/- immunotherapy

NO RULE FOR SURGERY OR RADIATION

41
Q

tx of T-lymphoblastic leukemia

A

combo chemo based on ALL

42
Q

most common low grade glioma

A

pilocytic astrocytoma

43
Q

name a type of high grade glioma

A

DIPG

44
Q
  • Most cases are sporadic (random) but can be associated with various genetic conditions like Neurofibromatosis, Von Hippel Lindau, Tuberous Sclerosis
  • incresed risk in ppl exposed to ionizing radiation to head or spine
A

CNS pathogenesis

45
Q
  • early morning headache relieved w/ vomiting
  • clumsiness, worse handwriting
  • CN palsies
  • vision changes, seizures
  • endocrinopathies
A

CNS tumor presentation

46
Q

most common presenting sx of CNS tumors in infants

A

macrocephaly

47
Q

CNS tumor with N/V, HA, gair & coordination issues is likely located where?

A

posterior fossa

48
Q

CNS tumor with CN palsies, gait & coordination issues is likely located where?

A

brain stem

49
Q

CNS tumor with back pain, weakness, gait issues is likely located where?

A

spinal cord

50
Q

most common sx of supratentorial and central CNS tumors

A

HA

51
Q

tx of CNS tumors (2)

A
  • near total surgical resection
  • chemo therapy to delay or replace radiation
52
Q

Abnormal growth of embryonic neural crest cells that normally make up the sympathetic ganglia and adrenal medulla

A

neuroblastoma (NBL)

53
Q

median age and gender of NBL diagnosis

A

17 months; males

54
Q
  • metastasis to marrow, liver, skin causing constitutional sx, blueberry muffin rash
  • spinal cord compression– pain, numbness limb weakness
  • horners syndrome– ptosis, miosis, anhydrosis
  • opsoclonus myoclonus syndrome
  • raccoon eyes
A

NBL clinical presentation

55
Q

opsoclonus myoclonus syndrome (3)

A

Rapid multidirectional eye movements
involuntary muscle spasms
irritability

55
Q

Urine HVA and VMA elevated in almost all patients with this cancer

A

NBL

56
Q

2 imaging for NBL

A
  • MRI > CT (can still do CT)
  • I-123 MBIG scan (follow / PET if neg.)
57
Q

biopsy shows Homer-Wright rosettes (small clusters of small round blue cells separated by a fibrillar matrix)

what cancer?

A

NBL

58
Q

management of NBL (2)

A
  • observation– can resolve; can do US or urine test
  • if over 12 months– intense multimodal therapy including radiation & surgery & (MBIG therapy?)
59
Q
  • Thought to arise from a mesenchymal stem cell that differentiates towards fibrous tissue, cartilage, or bone
  • more in Black males
  • more in rapid bone growth or in prior cancer tx
A

osteosarcoma (OSt)

60
Q

most common primary malignant bone tumor

A

OSt

61
Q
  • localized pain esp after an injury
  • generally no systemic sx
  • large, TENDER to palpation, soft tissue mass
A

OSt presentation

62
Q

4 imaging for OSt eval

A
  • plain films– sunburst, Codman’s triange, pathologic fracture
  • MRI of long bone
  • CT for chest
  • PET/radionuclide scan for whole body

biopsy is for definitive diagnosis

63
Q

tx of OSt (3)

A
  • surgery is standard
  • chemo
  • radiation if incomplete resection or unresectable
64
Q
  • mesenchymal progenitor cell origin
  • Nonrandom chromosomal translocations that involve breakpoints within the EWSR1 gene
  • Can develop in any bony or soft tissue but tend to develop in the diaphysis of long bones and the axial skeleton
A

ewing sarcoma (EWS)

65
Q
  • 2nd most common primary bone malignancy
  • more in white males
A

EWS

66
Q
  • localized pain or swelling; often worse at night or w/ exercise
  • minor trauma may initiate it
  • constitutional sx
A

EWS presentation

67
Q

first image obtained when evaluating for EWS

A

plain film xray

68
Q

XRAY findings below match up with what cancer

  • “moth eaten” bony lesion
  • codman’s triangle– displaced periosteum and cortex expansion
  • onion skin apperance
A

EWS

69
Q

other imaging used in EWS evaluation

A
  • MRI or CT of primary site
  • CT for pulmonary metastases
  • radionuclide bone scan
70
Q

way to evaluate for bone marrow metastases with EWS

A
  • bone marrow biopsy
  • tumor biopsy w/ CT
71
Q

EWS tx (3)

A
  • chemo
  • surgery > radiation
  • adjuvant radiation
72
Q

effects of cancer survival

A
  • neurologic– memory, coordination, academic performance, etc issues
  • endocrine– impaired growth, obesity, thyroid dysfunction
  • effect on fertility depends on age of therapy and what kind (radiation, alkylating agents); gonadal function and pre puberty ok
  • AML tx with anthracyclines can cause cardiomyopathies
  • higher risk of secondary malignancies if tx with radiation
73
Q

family centered care

A
  • respect for family preferences
  • flexibility and custamization
  • partnership an dcommunication
  • inforamtion sharing

peds 2 (10 decks)

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