peds oncology Flashcards

1
Q

What is the most common pediatric CA

A
  • acute lymphoblastic leukemia
  • occurs more in kids (80%), adults (20%)
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2
Q

uncontrolled proliferation of malignant lymphoid precursor clone causes what type of CA

A
  • acute lymphoblastic leukemia
  • *lymphoid precursor clone -> unable to mature
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3
Q

acute lymphoblastic leukemia is stained with

A

TdT

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4
Q

What is the median age of people affected with acute myeloid leukemia

A
  • more common in adults, median age 65
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5
Q

uncontrolled proliferation of malignant myeloid precursor clone causes which type of CA

A

acute myeloid leukemia

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6
Q

acute myeloid leukemia is stained with

A

myeloperoxidase

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7
Q

presence of Auer rods is pathognomonic for what condition

A

acute myeloid leukemia

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8
Q

Name causes of leukemia

  • exposure
  • acquired disorders
  • genetics
  • infection
A
  • exposure: radiation, chemo
  • acquired disorders: MDS, myeloma
  • genetics: down’s syndrome, Neurofibromatosis, Klinefelter syndrome
  • infection: human T cells leukemia virus
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9
Q

acute leukemias will have what similar hallmark presentation on labs

A
  • pancytopenia with circulating blasts (immature cells)
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10
Q

what symptom can help differentiate between acute leukemia and aplastic anemia since both present with pancytopenia

A
  • bone pain
    • acute leukemias will have expansion of bone into bone marrow due to the presence of growing number of immature cells that will cause pain -> hyperplastic
    • aplastic anemia does not have blasts
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11
Q

Why can ALL present with thymic mass

A

T lymphocytes mature in the thymus

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12
Q

CNS involvement is more typical of which acute leukemia

A

ALL

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13
Q

what are the typical signs and symptoms of acute leukemia

A
  • anemia - pallor, tachycardia, SOB
  • thrombocytopenia - ecchymosis
  • neutropenia - fever and infection
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14
Q

What is leukostasis? treatment?

A
  • aka symptomatic hyperleukocytosis
    • WBC > 100,000/μL which causes hyperviscosity symptoms
      • dyspnea, HA, confsion
      • It is most often seen in leukemia patients
  • treatment: hydration and leukapharesis
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15
Q

how is acute leukemia diagnosed

A
  • bone marrow biopsy showing > 20% blasts
  • **Presence of t(8,21), inversion (16), t (16,16), and t (15,17) have AML regardless of blast percentage
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16
Q

why is it important to check a uric acid level

A
  • disease processes that cause a high proliferation of cells will cause uric acid dump which can destroy the kidneys
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17
Q

What can be given to patients with acute leukemia to reduce risk of tumor lysis syndrome: which is characterized by rapid development of hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia, and acute renal failure.

A
  • Allopurinol and Rasburicase will lower uric acid
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18
Q

what is tumor lysis syndrome

A
  • constellation of metabolic disturbances that may follow the initiation of cancer treatment
    • hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia
    • -> acute renal failure
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19
Q

myeloproliferative neoplasm caused by dysregulation and unregulated proliferation of mature granulocytes

A

chronic myeloid leukemia

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20
Q

median age of people affected with chronic myeloid leukemia

A
  • 45-55
    • 15-50% of all leukemias in adults
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21
Q

striking feature of chronic myeloid leukemia in CBC

A

WBC found to be strikingly elevated

  • pt may be asymptomatic
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22
Q

cause of chronic myeloid leukemia

A
  • translocation of chromosome 9 (ABL) and 22 (BCR)
    • creates an oncogene that leads to the proliferation of granulocytes (neutrophils, eosinophils, basophils, and commonly platelets)
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23
Q

how is CML diagnosed

A
  • bone marrow biopsy
  • peripheral blood work for BCR/ABL via FISh or PCR
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24
Q

treatment of CML

A
  • Gleevec
  • Hydrea- given initially to bring down WBC if too high
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25
diverse group of malignant lymphoproliferative neoplasms of lymphoid tissue derived from B-cell progenitor clone
non-hodgkins lymphoma
26
what is the 5th most common pediatric CA in children
non-hodgkins lymphoma * affects children \< 15yo
27
clonal lymphoproliferative disorder with presence of reed sternberg cells
Hodgkin's lymphoma
28
Hodgkin's lymphoma has bi-modal distribution and usually affects people ages 20-30s and 50s. If it does affect children, what age group is normally affected?
15-19 yo
29
exposure to what virus may increase risk of getting Hodgkin's lymphoma
ebstein barr virus
30
painful adenopathy after ETOH consumption is seen with which condition
Hodgkin's lymphoma
31
painless and persistent lymphadenopathy is seen in
lymphomas
32
supraclavicular nodes are commonly affected in which type of lymphoma?
Hodgkin's lymphoma
33
what classifies as Stage 1 lymphoma
* one area of nodes
34
what classifies as Stage II lymphoma
two areas of nodes on ONE side of the diaphragm
35
what classifies as Stage III lymphoma
nodal sites on BOTH sides of diaphram
36
what classifies as Stage IV lymphoma
involvement to extranodal sites (bone marrow)
37
Describe the A, B, E staging of lymphoma
* A: no night sweats, weight loss, or bulky nodes * B: night sweats, weight loss, or bulky nodes * E: extranodal site contagious with nearby node
38
steriod use can have what effect on presentation of lymphomas
will cause lymphadenopathy to go down for a period of time, but swelling will return
39
diagnosis of lymphomas
**excisional node biopsy**
40
workup if you suspect lymphoma
* CBC * CMP * LDH: monitor cell turnover * uric acid * beta 2 microglobulin: measure of tumor burden * sed rate * excisional node biopsy * bone marrow biopsy: part of staging
41
children with NHL usually have what grade
High grades * requires therapy right away * adults can vary from low to high grade
42
if you relapse or you follow a low grade lymphoma, why should you always re-biopsy
lymphomas can morph!
43
primary malignant tumor characterized by production of osteoid or immature bone by malignant cells
Osteosarcoma
44
what is the most common primary bone malignancy in children and adolescents
Osteosarcoma * peak age range 13-16 (growth spurt)
45
osteosarcoma has what typical clinical presentation
* pain and swelling * commonly long bones: femur or tibia * fever, weight loss, malaise
46
how is Osteosarcoma diagnosed
* biopsy * also get CT for mets
47
what is the 2nd most common primary bone malignancy? Most affected age range?
* ewing's sarcoma * 10-15 yo
48
12% of patients with ewing's sarcoma have
urogenital anomalies
49
ewing's sarcoma most commonly develops on which bones
flat or long bones
50
is ewings sarcoma usually a metastatic disease?
* approx 75% have localized disease * less likely will have nodal, meningeal, or CNS spread
51
brain tumors can be seperated into what two types
* glial: most common * nonglial * \*glial cells: give nerve cells nourishment
52
standarized testing to confirm brain tumors
MRI
53
this brain tumor arises from primitive sympathetic ganglian cells in the CNS
Neuroblastoma
54
What is the most common solid neoplasm in children
Neuroblastoma
55
Neuroblastoma most commonly affect what part of the body
* adrenal gland (40%) * abdomen (25%) * thoracic (15%)
56
treatment for Neuroblastoma
* low risk disease: watch and wait * higher risk: surgery
57
What is the most common renal malignancy
* Nephroblastoma (Wilms tumor)
58
are one or both kidneys usually affected in younger children with nephroblastoma
bilateral * unilateral in older children
59
what is the most common intraocular cancer of childhood
Retinoblastoma
60
leukocoria is associated with
Retinoblastoma * leukocoria: abnormal white reflection from the retina of the eye
61
bilat retinoblastoma is a result of
RB1 gene
62
What is the most common soft tissue sarcoma of childhood
Rhabdomyosarcoma
63
Rhabdomyosarcoma affects which gender more
boys
64
common causes of hepatic tumors
* majority are primary tumors * can be metastatic from * Wilms tumor * rhabodomyosarcoma
65
Which type of hepatic tumors is seen in children \< 5 yo and occurs in the right side of the liver
Hepatoblastoma