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Pediatrics > BRS Oncology > Flashcards

BRS Oncology Flashcards

1
Q

B and T cell dysfunction, atopic dermatitis, thrombocytopenia, assoc with leukemia and lymphoma

A

wiskott-aldrich syndrome

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2
Q

X-linked lymphoproliferative disease is associated with ______ infection and may result in ______

A

EBV

lymphoma

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3
Q

Burkitt’s lymphoma associated with _____ infection
Kaposi’s sarcoma associated with _____ infection
Hodgkin’s lymphoma

A

EBV
HIV
EBV

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4
Q

_____ is the most common childhood cancer

A

ALL

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5
Q

peak incidence and most likely demographic of ALL

A

2-6 years, male caucasian

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6
Q

classification of ALL cell morphology

A

L1, L2, L3 with L1 being the most common in childhood (small with little cytoplasm)

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7
Q

immunophenotypes of ALL

A

pre-B-cell: 70% –> 70% CALLA +, 30% CALLA -
T cell: 25%
B cell: < 5%

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8
Q

clinical features of ALL

A

fever, bone/joint pain

pallor, bruising, HSM, LAD

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9
Q

what does CBC show in ALL

A

anemia
thrombocytopenia
WBC is variable- leukemic blasts are often seen

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10
Q

how to confirm dx of ALL

A

bone marrow biopsy demonstrates marrow replacement by lymphoblasts
-then do cytogenetics

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11
Q

good prognostic factors in ALL

A

1-9 years of age
female
white
<50,000 WBCs
hyperploidy (more than 53 chromosomes in leukemic cells)
CALLA +
no organ involvement or chromosomal translocation

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12
Q

3 stages of treatment for ALL

A
  1. induction with steroids, vincristine, L-asparaginase and also intrathecal methotrexate**
  2. consolidation with intrathecal methotrexate +/- cranial irradiation
  3. daily and periodic chemo for up to 3 years if no sxs
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13
Q

when to do BMT for ALL

A

very high risk children

those who have relapsed

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14
Q

some complications of ALL and the tx for ALL

A
  • infection 2/2 neutropenia: staph, e coli, pseudomonas
  • opportunistic infections: fungi, PCP
  • tumor lysis syndrome: hyperuricemia (renal probs), hyperkalemia (arrhythmias), hyperphosphatemia (hypocalcemia and tetany)
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15
Q

prognosis for ALL

A

generally good

85% long term survival

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16
Q

AML is often associated with ______

A

Down syndrome

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17
Q

classification of AML

A

M1-M7
M1, 2, 4, and 5 are most common
M3 (acute promyelocytic leukemia)- Auer rods
M7 (acute megakaryocytic leukemia)- associated with Down syndrome

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18
Q

which is CNS involvement more common?

AML vs. ALL

A

AML

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19
Q

clinical signs and sxs of AML

A

fever, HSM, bruising and bleeding, gingival hypertrophy, bone pain
LAD and testicular involvement are uncommon

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20
Q

lab findings of AML

A

pancytopenia or leukocytosis and DIC

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21
Q

how to dx AML

A
  • blood smear shows leukemic myeloblasts esp with *Auer rods

- bone marrow biopsy for confirmation

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22
Q

how to tx AML

A

AML (unlike ALL) requires intensive myeloablative therapy to induce remission –> then BMT is highly recommended

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23
Q

prognosis of AML

chemo, BMT, assoc with Down syndrome

A

chemotherapy- 50% respond
BMT- 70% respond
if assoc with Down syndrome, very responsive to tx

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24
Q

2 types of CML (least common leukemia in kids)

A 
adult-type: older children, teens, Philadelphia chromosome, t(9,22) BCR/ABL1
juvenile CML (JMML): infants and kids < 2 years, no Philadelphia chromosome
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25
Q

presentation of adult-type CML

A

nonspecific sxs:fatigue, weight loss, pain
massive splenomegaly
extremely high WBCs > 100,000

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26
Q

presentation of JMML (juvenile CML)

A 
fever
chronic eczema-like facial rash
suppurative LAD
petechiae and purpura
moderate luekocytosis WBC <100,000; anemia, thrombocytopenia
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27
Q

how to tx CML

A

BMT and gleevec (imatinib)

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28
Q

prognosis of CML

A

adult type- biphasic course

JMML- often fatal; relapse in at least 50%

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29
Q

cancer of the antigen-processing cells found in the lymph nodes or spleen

A

Hodgkins lymphoma

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30
Q

Hodgkin’s lymphoma most common presentation

A

slow and indolent
painless LAD in the supraclavicular/cervical regions
relatively common to see systemic symptoms

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31
Q

reed stern berg cell is seen in _____

A

Hodgkin’s lymphoma

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32
Q

how to dx Hodgkin’s lymphoma

A

lymph node bx where you see reed stern berg cell

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33
Q

staging of Hodgkin’s lymphoma

A

stage I: single lymph node
stage II: on same side of diaphragm
stage III: both sides of diaphragm
stage IV: disseminated to one or more extra-lymphatic organs

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34
Q

how to tx hodgkin’s lymphoma

what are some complications of tx

A

chemo and XRT

  • growth retardation
  • secondary malignancies
  • hypothyroidism
  • male sterility (very common)
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35
Q

prognosis of Hodgkin’s lymphoma

A

stage I and II: >80% long term survival

60-70% for more advanced disease

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36
Q

non-hodgkin’s lymphoma is ______ (indolent/aggressive)
more male or female?
associated with _______ states

A

aggressive
male
immunodeficiency states (HIV, wiskott-aldrich, ataxia telengiectasia, prior EBV)

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37
Q

3 categories of non-Hodgkin’s lymphoma

A
  1. lymphoblastic lymphoma- T cell, histologically similar to ALL, anterior mediastinal mass, SVC syndrome or airway obstruction
  2. small non cleaved cell lymphoma such as burkitt’s- B cell, intussusception, jaw mass in burkitt’s
  3. large cell lymphoma- B cell, enlargement of lymphoid tissue in tonsils, adenoids, peyer’s patches
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38
Q

most common lymphoma in childhood

A

burkitt’s

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39
Q

most common presenting feature in non-Hodgkin’s lymphoma

A

painless LAD

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40
Q

suspect _______ in any child > 3 years presenting with intussusception

A

lymphoma- esp small non cleaved cell lymphoma

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41
Q

how to dx non-Hodgkin’s lymphoma

A

LN biopsy and imaging of various sorts for staging

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42
Q

how to tx non-Hodgkin’s lymphoma

A

treatment must be rapid

  • surgery
  • chemo
  • ppx for CNS disease
  • tx tumor lysis syndrome
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43
Q

after leukemia, _____ are the second most common childhood cancer
they are also the most common solid tumors in kids

A

brain tumors

44
Q

4 most common brain cancers by histology

A
  1. glial cell tumors: astrocytomas
  2. primitive neuroectodermal tumors (PNET): medulloblastomas in the cerebellum
  3. ependymomas
  4. craniopharyngiomas
45
Q

in kids, _______ (infratentorial/supratentorial) tumors are more common

A

infratentorial

46
Q

MC infratentorial tumor

MC supratentorial tumor

A

medulloblastoma

astrocytoma

47
Q

brain tumor: diminished vision, visual field deficits, strabismus

A

optic glioma

48
Q

brain tumor: growth retardation, delayed puberty, visual changes, DI, other hormonal problems

A

craniopharyngioma

49
Q

how to dx brain tumors

A

MRI

CSF analysis at the time of surgery

50
Q

how to tx brain tumors

A
  • surgery
  • chemo
  • try not to do XRT if under age 5 years
51
Q

poorest prognosis with brain cancer

A

brainstem gliomas
not resectable
chemo doesn’t work

52
Q

malignant tumor of neural crest cells arising anywhere along the sympathetic ganglia chain and within the adrenal medulla

A

neuroblastoma

53
Q

peak incidence of neuroblastoma

A

age 2 years

54
Q

most common presentation of neuroblastoma

A

firm abdominal mass that crosses the midline

55
Q

how to dx neuroblastoma

A

-definitive dx by bone marrow bx + elevated urine catecholamines (VMA, HVA)
OR
-tissue biopsy

also CT or MRI for assessing tumor spread and then bone scan/skeletal survey to assess for mets to the bone

56
Q

staging of neuroblastoma

A

I: tumor confined to structure of origin
II: extends beyond structure of origin but doesn’t cross midline
III: tumor goes past midline
IV: distant mets
IVS: localized tumor at stage I or II but distant mets to any organ but bone

57
Q

how to tx neuroblastoma

A

surgery alone for stage I and II
chemo for metastatic disease and locally advanced dz
XRT for advanced disease

58
Q

prognosis of neuroblastoma

A 
good prognosis
-children < 1 year
-stage I and II disease
-spontaneous regression can happen in young infants with stage IVS disease
bad prognosis
-stage III and IV disease
-amplification of oncogene N-myc 
-high levels of ferritin, LDH, and neuron-speicfic enolase
59
Q

______ is a childhood tumor of the kidneys

A

Wilm’s tumor (nephroblastoma)

60
Q

peak incidence of wilm’s tumor

A

age 3 ears

61
Q

hemihypertrophy, macroglossia, visceromegaly

A

beckwith-wiedemann syndrome

62
Q

wilms tumor, aniridia, GU abnormalities, MR

A

WAGR syndrome

63
Q

most common presentation of wilm’s tumor

A

abdominal mass (smooth, firm, rarely crosses midline)
abdominal pain
hematuria
HTN

64
Q

how to dx wilm’s tumor

A

CT or MRI

bx and histology

65
Q

how many stages of wilm’s tumor

A

I-V

V is bilateral renal involvement

66
Q

how to tx wilm’s tumor

A

prompt surgery
chemo
XRT for advanced dz

67
Q

prognosis of wilm’s tumor

A

excellent in most cases

68
Q

most common soft tissue sarcoma in childhood

A

rhabdomyosarcoma- malignant tumor of the same embryonic mesenchyme that gives rise to skeletal muscle

69
Q

common presentation rhabdomyosarcoma

A

painless soft tissue mass

70
Q

most common areas involved in rhabdomyosarcoma

A
  1. head and neck
    - orbital tumors- proptosis, chemosis, eyelid swelling, CN palsies
    - nasopharyngeal tumors- epistaxis, airway obstruction, chronic sinusitis
    - laryngeal tumors- hoarseness
  2. GU tract- hematuria, urinary tract obstruction, abd mass
  3. extremities- painless growing mass
71
Q

how to dx rhabdomyosarcoma

A

CT or MRI

bx and histology

72
Q

how to tx rhabdomyosarcoma

A

surgical resection

+/- chemo and XRT

73
Q

prognosis of rhabdomyosarcoma

which sites have the best prognosis

A

head and neck and GU tumors have the best prognosis

74
Q

most common malignant bone tumor

peak incidence and gender

A

osteogenic sarcoma

male teens

75
Q

most likely location for osteogenic sarcoma

A

near the knee

*metaphyses of tubular long bones

76
Q

systemic sxs are _______ common/uncommon in osteogenic sarcoma
how about ewing’s sarcoma

A

uncommon with osteogenic sarcoma

common in ewing’s sarcoma (fever, malaise, weight loss)

77
Q

X-ray finding in osteogenic sarcoma and ewing’s sarcoma

A

osteogenic- sunburst

ewing’s- onion skin

78
Q

most common locations for ewing’s sarcoma

A

flat bones and diaphysis of tubular bones; most commonly in axial skeleton (esp pelvis)

79
Q

how to dx primary bone cancer (osteogenic and ewing’s sarcoma)

A

MRI –> tissue biopsy –> bone scan, chest CT and other stuff to look for mets

80
Q

how to tx osteogenic sarcoma

A

surgery for primary lesion and pulmonary mets

chemo

81
Q

prognosis of osteogenic sarcoma

A

survival > 60%

82
Q

bone cancer with small, ground, blue cell tumor

A

ewing’s sarcoma

83
Q

second most common malignant bone tumor occurring in male adolescents

A

ewing’s sarcoma

84
Q

chromosomal translocation in ewing’s sarcoma

A

t (11,21)

85
Q

how to tx ewing’s sarcoma

prognosis

A

multiagent chemo followed by surgical excision
XRT if complete excision is not possible
-prognosis good for local dz, poor if mets are present

86
Q

most common liver tumor in childhood
what age group
what is it assoc with

A

hepatoblastoma
< 3 years
beckwith-wiedemann syndrome

87
Q

hepatocellular carcinoma in childhood is most likely 2/2 _____

A

chronic active hep B infection

also biliary atresia, glucogen storage disease type I, alpha-1-antitrypsin deficiency, hereditary tyrosinemia

88
Q

presentation of liver tumors in childhood

A

RUQ mass, loss of appetite and weight

jaundice is usually absent

89
Q

how to dx liver tumors in childhood

A

CT or MRI

look for elevated alpha-fetoprotein in the serum

90
Q

how to tx liver tumors in childhood

what’s the prognosis

A

chemo and surgical resection

overall, prognosis is quite poor and often end up with mets

91
Q 
\_\_\_\_\_\_ is the most common teratoma during first year of life
female vs. male
what's the presentation
benign or malignant 
how to tx
A 
sacrococcygeal
female predominant 
soft tissue mass growing from coccyx 
benign
tx with surgical excision
92
Q

anterior mediastinal teratomas are generally _______ (benign/malignant) and can present with ______

A

benign

airway obstruction

93
Q

ovarian teratomas are the most common ovarian tumor and are generally _____ (benign/malignant)
you may see ______ on abdominal xray

A

benign

calcium in the tumor

94
Q

most common testicular tumors

A

yolk sac tumor > teratoma

95
Q

peak ages of testicualr tumor

A

< 5 years and during teen years

96
Q

in kids, chances of testicular tumor being benign

what about adults

A

in kids, 1/3 are benign

in adults, all are malignant

97
Q

elevated alpha fetoprotein is seen in which germ cell tumor

A

yolk sac tumor

98
Q

for ovarian tumors, 1/3 are malignant

the younger the child is, the more likely that the tumor is ____ benign/malignant

A

malignant

99
Q

eosinophilic granuloma
Hand-Schuller-Christian disease
Letterer-Siwe disease

A

all are part of Langerhans Cell Histiocytosis (LCH)

-severe immune dysregulation disease that may be cancer-like

100
Q

common clinical involvement with langerhans cell histiocytosis (LCH)

A
  • skeletal involvement- skull, bony lesions, chronic draining ears indicates mastoid LCH
  • skin involvement- seborrheic dermatitis of the diaper area and scalp
  • pituitary or hypothalamic involvement
101
Q

how to dx LCH

A

histologic features on skin or bone bx

102
Q

how to tx LCH

prognosis

A

if single lesion or organ –> local curettage, low dose XRT, steroids, or single agent chemo
if multiple lesions –> multi agent chemo
prognosis is variable based on extent of disease

103
Q

Down syndrome puts you at risk for these cancers

A

ALL and AML

104
Q

beckwith-wiedemann syndrome predisposes to these tumors

A

wilms, rhabdomyosarcoma, hepatoblastoma

105
Q

neurofibromastosis type I predisposes to these tumors

A

brain tumors and lymphoma

Pediatrics (14 decks)

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