Oncology Flashcards

1
Q

major tumor suppressor gene; “guardian of the genome”

A

p53

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2
Q

phases/ stages of treatment in chemotherapy

A

Initial therapy: Remission Induction
given for 4 weeks, Vincristine weekly, corticosteroid, single dose of long acting pegylated Asparaginase
*Intrathecal always given

second phase: Consolidation
focus on intensive CNS therapy
13-28 weeks; Cytarabine and Vincristine

Maintenance: 2-3 years
daily Mercaptopurine and weekly oral Methotrexate
intermittent dose of Vincristine and corticosteroid

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3
Q

more than 20% blasts; marrow failure

A

AML

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4
Q

what are the clinical findings in patients with AML?

A

subcutaneous nodules (“blueberry muffin lesions”)
infiltration of gingiva
chloromas

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5
Q

what are the BMA findings in patients with AML?

A

hyper cellular marrow; monotonous pattern

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6
Q

What are the B symptoms

A

weight loss >10%, unexplained fever >38C, drenching night sweats

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7
Q

painless, tender, firm, rubbery cervical or supraclavicular LAD

A

Hodgkin lymphoma

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8
Q

pathognomonic of Hodgkin lymphoma

A

Reed Sternberg cells

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9
Q

treatment for Hodgkin Lymphoma

A

chemo and radiation therapy

tx: Cyclophosphamide, Procarbazine, Vincristine or Vinblastine, Prednisone (COPP)

ABVD: Doxorubicin, Bleomycin, Vinblastine, Dacarbazin

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10
Q

60% of lymphomas in children; aggressive and high grade

A

Non-Hodgkin Lymphoma

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11
Q

What are the subtypes of Hodgkin Lymphoma?

A

Lymphoblastic-for immature B & T lymphocytes
Burkitt lymphoma
diffuse large B cell
Anaplastic large cell

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12
Q

3rd most common malignancy in childhood

A

Lymphoma

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13
Q

types of lymphoma

A

Hodgkins

Non-Hodgkin

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14
Q

types of Non-Hodgkin lymphoma

A

B cell
T cell
Large cell (either B or T)

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15
Q

type of Non-Hodgkin lymphoma with translocation between chromosome 8 and Ig locus 2, 14, 22

A

Burkitt lymphoma

c-myc-oncogene –> malignant B cell lymphoma

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16
Q

most common presentation of Hodgkin Disease

A

painless, firm lymphadenopathy usually in supraclavicular and cervical nodes

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17
Q

what are the B symptoms

A

fever >38C for 3 consecutive days
drenching night sweats
unintentional weight loss 10% within 6 months

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18
Q

pathologic hallmark of Hodgkin lymphoma

A

Reed-Sternberg cells

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19
Q

treatment for childhood Hodgkin DIsease

A

chemotherapy plus low dose involved field radiation treatment

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20
Q

peak incidence age of ALL

A

2-3 years old; more common in boys

association between EBV and B cell ALL

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21
Q

most common immunophenotype of leukemia ALL with onset 1-10 years old

A

Precursor B-cell ALL

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22
Q

diagnosis of ALL strongly suggested by peripheral blood smear showing

A

bone marrow failure

*anemia and thrombocytopenia

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23
Q

> 25% of bone marrow cells are lymphoblasts

A

ALL

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24
Q

Staging of ALL is based on

A

CSF findings

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25
single most important prognostic factor in ALL
treatment 3 of the most important predictive factors Age of the patient at time of diagnosis (1-10yrs) initial leukocyte count (<50,000/uL) speed of response to treatment
26
considered high risk in ALL
<1yr old and >10 years old >50,000/uL T-cell immunophenotype slow response to therapy
27
initial therapy designed to eradicate leukemic cells from bone marrow
Remission induction * given for 4 weeks * consists of Vincristine weekly * corticosteroids such as Dexa or Pred * either repeated dose of native L-asparaginase or single dose of long-acting, pegylated asparanginase * pxs at higher risk also receive Daunomycin at weekly intervals
28
second phase of treatment of ALL
focuses on CNS therapy Intrathecal chemo given by lumbar puncture *after remission, many regimens provide 14-28 weeks of multiagent therapy --> Consolidation and intensification
29
maintenance phase of ALL
daily Mercaptopurine | weekly Methotrexate usually with intermittent doses of Vincristine and a corticosteroid
30
leukemia common in adolescence
Acute myelogenous leukemia (AML)
31
characteristic feature of AML
>20% of bone marrow cells constitute a fairly homogenous population of blast cells
32
most common classification of subtypes of AML
FAB system *flow cytometry
33
symptoms of AML that are uncommon in ALL
subcutaneous nodules or "blueberry muffin" lesions infiltration of gingiva signs and symptoms of DIC chloroma or granulocytic sarcomas
34
Philadelphia chromosome resulting in BCR-ABL fusion protein
Chronic myelogenous leukemia * spleen greatly enlarged -->right upper quadrant pain * Tx: Imatinib (Gleevec)
35
diagnosis of CML
* high WBC count with myeloid cells at all stages of differentiation in peripheral blood and bone marrow * confirmed by cytogenic and molecular studies that demonstrate presence of Philadelphia chromosome and BCR-ABL gene
36
clonal proliferation of hematopoietic stem cells that typically affects children <2yrs old
Juvenile myelomonocytic leukemia BM: myelodysplastic pattern with blasts accounting for <30% of cells
37
Infant leukemia
poor prognosis often present with CNS or skin involvement hyperleukocytosis and extensive tissue infiltration --> organomegaly including CNS disease subcutaneous nodules known as leukemic cutis and tachypnea due to diffuse pulmonary infiltration by leukemic cells *very intensive chemotherapy including stem cell transplantation
38
second most common malignancy in childhood and adolescence
Primary central nervous system tumors
39
most common CNS tumor in 0-14 years old
Pilocytic astrocytomas and Medulloblastoma
40
most common CNS tumor in 15-19 years old
pituitary tumors and pilocytic astrocytoma
41
True or False. | there is slight predominance of infratentorial tumor lesion followed by supratentorial
True
42
primary location of tumors in first year of life
Supratentorial | most commonly choroid plexus and teratomas
43
primary location of tumors in children 1-10 years old
Infratentorial tumors predominate | juvenile PA and medulloblastoma
44
primary location of tumors in >10 years old
supratentorial tumors | with diffuse astrocytomas
45
classic triad associated with midline or infratentorial tumors
headache nausea and vomiting papilledema
46
disorders of equilibrium, gait and coordination
Infratentorial tumors
47
blurred vision, diplopia and nystagmus are associated with
Infratentorial tumors
48
more commonly associated with focal disorders such as motor weakness, sensory changes, speech disorders, seizures and reflex abnormalities
Supratentorial tumors
49
present with hand preference
Supratentorial tumors
50
pineal region tumors; paresis of upward gaze, pupillary dilatation, relative to accomodation but not to light, nystagmus to convergences or retraction
Parinaud syndrome
51
most common astrocytoma in children
Pilocytic astrocytoma *classic site: cerebellum
52
most commonly occur in hypothalamic/optic chiasmic region
Pilomyxoid astrocytoma tx: surgery -primary radiation therapy and chemotherapy
53
most common group of malignat CNS tumors of childhood
Embryonal tumors or primitive neuroectodermal tumors (PNET)
54
most common extracranial solid tumor in children and most commonly diagnosed malignancy in infancy
Neuroblastoma
55
syndromes associated with Neuroblastoma
Pepper syndrome: massive involvement of liver with metastatic disease with or without respiratory distress Horner syndrome: unilateral ptosis, myosis and anhidrosis Hutchinson syndrome: limping and irritability in young child associated with bone marrow metastases Opsoclonus: myoclonic jerking and random eye movement with or without cerebellar ataxia Myoclonus-ataxia syndrome: Kerner-Morrison syndrome: intractable secretory diarrhea due to tumor secretion of vasointestinal peptides Neurocristopathy: neuroblastoma assoc with other neural crest disorders
56
location of neuroblastoma
half: adrenal glands remainder: paraspinal sympathetic ganglia
57
used to confirm diagnosis of neuroblastoma
homovanillic acid (HVA) and vanillylmandelic acid (VMA) in urine
58
used to determine treatment risk group and estimated prognosis for each patient with neuroblastoma
patient's age and tumor stage
59
also known as nephroblastoma
Wilm's tumor
60
most common primary malignant renal tumor of childhood
Wilm's tumor
61
presents as asymptomatic abdominal mass and hypertension
Wilm's tumor
62
most common primary malignant bone tumor
Osteosarcoma
63
common sarcoma in less than 10 years old
Ewing sarcoma
64
differentiate Osteosarcoma from Ewing sarcoma
osteosarcoma and Ewing sarcoma are both prevalent on 2nd decade Ewing sarcoma: small round cell; common in diaphysis of long bones, flat bones; local pain and swelling, fever; "onion skinning" --> radiotherapy and/or surgery of primary tumor Osteosarcoma: common in metaphysis of long bones; local pain and swelling; sunburst pattern; --> chemotherapy, ablative surgery of primary tumors
65
pain, limp and swelling most common presentation
Osteosarcoma *complete surgical resection
66
most common benign tumors of infancy
Hemangiomas *50% located in the head and neck region
67
rapidly enlarging lesion, thrombocytopenia, microangiopathic hemolytic anemia and coagulopathy
Kasabach-Merritt Syndrome
68
most common primary malignant intraocular tumor of childhood
Retinoblastoma