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Pediatrics > BRS- Onc > Flashcards

BRS- Onc Flashcards

1
Q

Leading cause of death from disease in childhood?
Most common childhood cancer?
Most common solid childhood cancer?

A

Cancer= leading cause of death from disease
Childhood cancer = ALL
Solid childhood cancer= brain tumors

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2
Q

Two immunodeficiencies that predispose kiddos to cancer

A
  • Wiskott Aldrich: lymphomas & leukemias

- X linked lymphoproliferative disease: EBV –> lymphoma

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3
Q

EBV assc cancer?

HIV assc cancer?

A

EBV –> Burkitts

HIV –> Kaposci’s

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4
Q

Downs syndrome assc cancers

A

ALL, AML

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5
Q

Syndrome assc with GI cancers

A

Blooms syndrome

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6
Q

NF II assc cancer

A

acoustic neuroma

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7
Q

Two abdominal mass types

A

Wilms Tumor

Neuroblastoma (crosses midline)

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8
Q

Soft mass on extremities

A

rhabdomyosarcoma

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9
Q

Early morning H/A + vomiting cause

A

brain mass

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10
Q

Tumor causing leukocoria

A

Retinoblastoma

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11
Q

Tumors causing HTN

A

Neuroblastoma
Wilms tumor
Pheochromocytoma

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12
Q

Peak incidence/ prototype patient with ALL

A

-white male age 2-6 years

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13
Q

Two classification types for ALL

A
  • cell morphology

- immunophenotype (+/- CALA)

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14
Q

Describe cell morphology types + which is most common in ALL?

A

L1: small with little cytoplasm
L2: between
L3: large lymphoblasts

*L1 more common

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15
Q

Describe immunophenotypes in ALL + which is most common?

A

T/B/PreB cell +/- CALLA

Most common Pre B cell + CALLA

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16
Q

What does CALA stand for?

A

common acute lymphocytic leukemia antigen

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17
Q

Most common ALL symptoms?

A

fever and bone pain

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18
Q

CBC Findings in ALL

A 
low platelets 
anemia 
variable WBC count 
**CBC does not rule out leukemia 
**Must confirm with marrow evaluation
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19
Q

Staging for ALL

A

no staging system

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20
Q

Favorable age, sex, race for ALL prognosis

A
  • 2-9
  • female
  • Caucasian
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21
Q

Favorable immunophenotype/genetic makeup for ALL:

A

+CALLA

-t(9,22)

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22
Q

Three stages of ALL management

A
  • induction
  • consolidation
  • maintenance
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23
Q

What drug is given to all ALL patients in the induction stage of treatment?

A

intrathecal MTX

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24
Q

What is the consolidation phase of ALL treatment and what is its purpose?

How long is maintenance phase of ALL tx?

A

intrathecal MTX
cranial irradiation, high risk 5+
prevents CNS involvement

-maintenance up to 3 years

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25
Q

Lab findings assc with tumor lysis syndrome

A

-^^uric acid, ^^potassium, ^^phospohate (=low Ca)

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26
Q

Three sequelae of tumor lysis syndrome

A
  • tetany
  • renal disease
  • cardiac disease
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27
Q

Overall ALL survival rate

A

85%

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28
Q

Four syndromes assc with AML

A
  • Fanconi
  • Downs
  • Kostmann
  • Neurofibromatosis
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29
Q

What are the 7 types of AML

A 
M1: acute myeloblastic, immature 
M2: acute myeloblastic, mature
M3: acute promyelocytic
M4: acute myelomonocytic 
M5: acute monocytic 
M6: erythroleukemia 
M7: acute megakaryotic
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30
Q

What are the 4 more common types of AML

A

M1,2,4,5

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31
Q

Which type of AML is assc with Auer Rods?

assc with Down Syndrome?

A 
Acute promyelocytic (M3)= auer rods 
Acute megakaryocytic (M7) = Downs
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32
Q

Organ systemic more commonly involved in AML than ALL? Common oral finding in AML?

A

CNS involvement + gingival hyperplasia common in AML

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33
Q

Which type of AML is most successfully cured?

A

AML assc with Downs= most responsive

50% cured with chemo; 70% cured with marrow transplant (in general cases)

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34
Q

Least common type of leukemia in kiddos is _____.

Two types are ____ & ______.

A

CML
Adult type
Juvenile CML

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35
Q

Chromosomal abnormality assc with adult type and juvenile type CML?

A

adult type: 9,22 BCR ABL

juvenile type: chromosome 7,8 involvement

36
Q

Age groups assc with adult type and juvenile type CML?

Which is more fatal

A

adult: older kiddos/teens
juvenile: younger than two, worst prognosis (often fatal, half relapse)

37
Q

WBC count and sign suggestive of adult CML? Juvenile?

A

Adult: massive splenomegaly; extremely high WBC
Juvenile: facial rash, moderate leukocytosis

38
Q

Hodgkins vs Non-Hodgkins lymphoma:

  • which is more common?
  • which grows more rapidly/is more aggressive?
  • which is commonly assc with systemic symptoms?
A
  • common: non
  • rapid: non
  • systemic: hodgkins
39
Q

Emergent sequelae assc with nonhodgkins lymphoma:

A
  • SVC syndrome

- airway compression

40
Q

Describe the staging assc with Hogdkins lymphoma

A 
Ann Arbor System:
I: single node 
II: two+ nodes, same side of diaphragm 
III: nodes on both sides of diaphragm 
IV: diffuse

A: lack of symptoms
B: symptoms present

41
Q

What predisposes kiddos to Hodgkins Lymphoma?

A

EBV mononucleosis

42
Q

Cell assc with Hodgkins Lymphoma?

A

Binucleated reed Sternberg cell

43
Q

Complications of Hodgkins Lymphoma therapy:

A

1) hypothyroidism
2) secondary malignancies
3) male sterility
4) growth retardation

44
Q

Non-Hodgkins Lymphoma:

  • age group/sex
  • predisposing factor
A

-5+
-male
immunodeficiency predisposes

45
Q

Three major categories of Non-Hodgkins lymphoms

A

1) lymphoblastic lymphoma (T cell)
2) small non-cleaved (B cell)
3) large cell (B cell)

46
Q

Of the three non-hodgkins lymphomas, what type includes burkitts?

A

small non cleaved B cell

47
Q

Most common presenting feature of non-hodgkins lymphoma

A

painless lymphadenopathy

48
Q

Common presentation lymphoblastic lymphoma

A

anterior mediastinal mass w/ airway or SVC obstruction

49
Q

Endemic Burkitts:

  • location in the world
  • location in the body
A
  • Africa

- Jaw

50
Q

Intussusception in a kiddo older than 3 requires workup for?

A

lymphoma (especially small cell)

51
Q

Common location of large

A
  • tonsils
  • adenoids
  • peyers patch
52
Q

Three diseases assc with brain tumors

A
  • VHL
  • TS
  • NF
53
Q

Most and second most common peds brain tumors

A

-glial #1
-PNETs #2
ependymomas & craniopharygiomas also common

54
Q

High grade brain tumor common location?

Low grade?

A 
High= supratentorial 
Low= infratentorial
55
Q

Most common PNET tumor?

A

-medulloblastoma in the cerebellum

56
Q

MC infratentorial tumor? supra?

A 
supra= astrocytoma 
infra= medulloblastoma
57
Q

Ataxia is assc with what brain tumors?

Seizures?

A 
ataxia= infratentorial (cerebellum)
seizures= supratentorial
58
Q

Principal treatment of brain tumors?

A

-resection or debulking

59
Q

Peds brain tumor with worst prognosis

A

brainstem glioma

60
Q

Neuroblastoma:

  • most common location
  • most common age
  • diagnosis
A
  • 75% in abdomen or pelvis, can be anywhere on the sympathetic ganglia chain
  • first 5 years of life
  • marrow biopsy + urine HVA/VMA
61
Q

Neuro complication assc with 2% of neuroblastomas

A

-acute cerebellar atrophy
“dancing feet and eyes”
=ataxia, opsoclonus, myoclonus

62
Q

Staging of neuroblastoma:

A
  • I: localized
  • II: spread but not crossing midline
  • III: crosses midline
  • IV: crosses midline + mets
  • IVS: stage I-II + mets
63
Q

What type of neuroblastoma may spontaneously regress?

A

-Stage IVs in a baby

64
Q

What marker is significant for poor prognosis in neuroblastoma?

A

n-myc

65
Q

Most common childhood renal tumor

A

Wilms, usually kiddos under 5

66
Q

Describe Beckwith Wiedemann syndrome

A
  • hemihypertrophy
  • macroglossia
  • visceromegaly
67
Q

Describe WAGR syndrome + assc chromosome

A

chromosome 11

  • wilms tumor
  • aniridia
  • genitourinary abnormalities
  • retardation
68
Q

Two symptoms assc with Wilms tumor

A

Hematuria

HTN (pressure on renal artery)

69
Q

Staging Wilms tumor

A
  • I: limited to kidney
  • II: local spread, completely excised
  • III: residual tumor after resection
  • IV: distant mets
  • V: bilateral renal involvement
70
Q

Prognosis Wilms Timor:

A

90% survival

71
Q

Condition predisposing to Rhabdo

A

neurofibromatosis

72
Q

Most common sites for rhabdo

A

head and neck including the orbit

73
Q

Most common and second most common bone tumors in kiddos? Prognosis of each?

A
  • osteosarcoma (#1), 60% survive

- ewings sarcoma, 80% survive

74
Q

Osteosarcoma:

  • peak incidence
  • common location
  • radiographic findings
  • presentation
A
  • adolescent males during growth spurt
  • metaphysis of long bones, esp common at knee
  • sunburst pattern
  • poor systemic involvement
75
Q

Ewings Sarcoma:

  • most common location
  • symptoms
  • radiographic findings
A
  • diaphysis of long bones
  • fever, weight loss, leukocytosis, ^ESR
  • onion skin pattern
76
Q

Ewings Sarcoma chromosomal abnormalities

A

t(11,21)

77
Q

Most common type of liver tumor?
Most common age group?
Assc syndrome?

A

hepatoblastoma
under 3
Beckwith Wiedemann syndrome

78
Q

Hepatocellular carcinoma:

most common assc disease

A

chronic hepatitis B

79
Q

Prognosis for hepatoblastoma/ hepatocellular carcinoma

A

-both= poor, hepatocellular carcinoma worst

80
Q

Six germ cell tumors seen in kiddos

A
  • teratoma**
  • seminoma (Male)
  • dysgerminoma (Female)
  • Yolk Sac
  • Embryonal
  • Choriocarcinoma
81
Q

Most common germ cell tumor seen in kiddos

A

sacrococcygeal teratoma, 95% benign

82
Q

Elevation of what protein is assc with yolk sac tumors?

A

AFP

83
Q

How commonly are ovarian tumors malignant?

A

1/3rd younger the child= more malignant

84
Q

Most common site for Langerhans Cell Histiocytosis

A

skull

painful bony lesions +/- pathologic fractures

85
Q

Skin finding in LCH

A

mimics cradle cap

Pediatrics (12 decks)

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