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Hematology and Oncology > Oncology > Flashcards

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
Q

single most important prognostic factor in ALL

A

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

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26
Q

considered high risk in ALL

A

<1yr old and >10 years old
>50,000/uL
T-cell immunophenotype
slow response to therapy

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27
Q

initial therapy designed to eradicate leukemic cells from bone marrow

A

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
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28
Q

second phase of treatment of ALL

A

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
Q

maintenance phase of ALL

A

daily Mercaptopurine

weekly Methotrexate usually with intermittent doses of Vincristine and a corticosteroid

30
Q

leukemia common in adolescence

A

Acute myelogenous leukemia (AML)

31
Q

characteristic feature of AML

A

> 20% of bone marrow cells constitute a fairly homogenous population of blast cells

32
Q

most common classification of subtypes of AML

A

FAB system

*flow cytometry

33
Q

symptoms of AML that are uncommon in ALL

A

subcutaneous nodules or “blueberry muffin” lesions
infiltration of gingiva
signs and symptoms of DIC
chloroma or granulocytic sarcomas

34
Q

Philadelphia chromosome resulting in BCR-ABL fusion protein

A

Chronic myelogenous leukemia

  • spleen greatly enlarged –>right upper quadrant pain
  • Tx: Imatinib (Gleevec)
35
Q

diagnosis of CML

A
  • 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
Q

clonal proliferation of hematopoietic stem cells that typically affects children <2yrs old

A

Juvenile myelomonocytic leukemia

BM: myelodysplastic pattern with blasts accounting for <30% of cells

37
Q

Infant leukemia

A

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
Q

second most common malignancy in childhood and adolescence

A

Primary central nervous system tumors

39
Q

most common CNS tumor in 0-14 years old

A

Pilocytic astrocytomas and Medulloblastoma

40
Q

most common CNS tumor in 15-19 years old

A

pituitary tumors and pilocytic astrocytoma

41
Q

True or False.

there is slight predominance of infratentorial tumor lesion followed by supratentorial

A

True

42
Q

primary location of tumors in first year of life

A

Supratentorial

most commonly choroid plexus and teratomas

43
Q

primary location of tumors in children 1-10 years old

A

Infratentorial tumors predominate

juvenile PA and medulloblastoma

44
Q

primary location of tumors in >10 years old

A

supratentorial tumors

with diffuse astrocytomas

45
Q

classic triad associated with midline or infratentorial tumors

A

headache
nausea and vomiting
papilledema

46
Q

disorders of equilibrium, gait and coordination

A

Infratentorial tumors

47
Q

blurred vision, diplopia and nystagmus are associated with

A

Infratentorial tumors

48
Q

more commonly associated with focal disorders such as motor weakness, sensory changes, speech disorders, seizures and reflex abnormalities

A

Supratentorial tumors

49
Q

present with hand preference

A

Supratentorial tumors

50
Q

pineal region tumors; paresis of upward gaze, pupillary dilatation, relative to accomodation but not to light, nystagmus to convergences or retraction

A

Parinaud syndrome

51
Q

most common astrocytoma in children

A

Pilocytic astrocytoma

*classic site: cerebellum

52
Q

most commonly occur in hypothalamic/optic chiasmic region

A

Pilomyxoid astrocytoma

tx: surgery -primary
radiation therapy and chemotherapy

53
Q

most common group of malignat CNS tumors of childhood

A

Embryonal tumors or primitive neuroectodermal tumors (PNET)

54
Q

most common extracranial solid tumor in children and most commonly diagnosed malignancy in infancy

A

Neuroblastoma

55
Q

syndromes associated with Neuroblastoma

A

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
Q

location of neuroblastoma

A

half: adrenal glands
remainder: paraspinal sympathetic ganglia

57
Q

used to confirm diagnosis of neuroblastoma

A

homovanillic acid (HVA) and vanillylmandelic acid (VMA) in urine

58
Q

used to determine treatment risk group and estimated prognosis for each patient with neuroblastoma

A

patient’s age and tumor stage

59
Q

also known as nephroblastoma

A

Wilm’s tumor

60
Q

most common primary malignant renal tumor of childhood

A

Wilm’s tumor

61
Q

presents as asymptomatic abdominal mass and hypertension

A

Wilm’s tumor

62
Q

most common primary malignant bone tumor

A

Osteosarcoma

63
Q

common sarcoma in less than 10 years old

A

Ewing sarcoma

64
Q

differentiate Osteosarcoma from Ewing sarcoma

A

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
Q

pain, limp and swelling most common presentation

A

Osteosarcoma

*complete surgical resection

66
Q

most common benign tumors of infancy

A

Hemangiomas

*50% located in the head and neck region

67
Q

rapidly enlarging lesion, thrombocytopenia, microangiopathic hemolytic anemia and coagulopathy

A

Kasabach-Merritt Syndrome

68
Q

most common primary malignant intraocular tumor of childhood

A

Retinoblastoma

Hematology and Oncology (2 decks)

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