oncoped

Question 1

most common childhood malignancy; increased incidence in Down syndrome; ataxia-telangiectasia; Fanconi syndrome; NF1; risk is >70% if the first twin is diagnosed during the 1st yr of life and they shared the same placenta; patients who have undergone intense Tx for a solid tumor

Answer 1

ALL

Question 2

Diagnoses by a bone marrow evaluation that shows >25% of the BM cells as a homogenous population of lymphoblasts

Answer 2

ALL

Question 3

most important prognostic factor in ALL

Answer 3

treatment

Question 4

3 of the most important predictive factors in ALL

Answer 4

age at the time of diagnosis; intitial leukocyte count; speed of response to treatment

Question 5

Treatment of ALL Remission induction

Answer 5

Vincristine; prednisone; L-asparaginase x4wks + CNS prophylactic irradiation or chemotherapy to eradicate leukemic cells from BM

Question 6

Tx of ALL Maintenance phase

Answer 6

Mercaptopurine daily and Methothrexate weekly x2-3yrs

Question 7

complication of therapy in ALL

Answer 7

tumor lysis syndrome

Question 8

definition of remission in ALL

Answer 8

<5% blasts in the BM and a return of neutrophil and platelet count to near-normal levels after 4.5wks of tx

Question 9

site of metastasis of ALL

Answer 9

liver; spleen; lymph nodes

Question 10

poor prognostic factors in ALL

Answer 10

10 yrs; male; WBC>100k U/L on presentation; presence of CNS leukemia; presence of mediastinal mass

Question 11

Ann Arbor staging of lymphomas

Answer 11

Stage 1-one LN group involved; Stage II-two LN groups involved on the same side of the diaphragm; Stage III-two LN groups involved on both sides of the diaphragm or extralymphatic involvement; Stage IV-diffuse extralymphatic site involvement (usually BM CNS or liver)

Question 12

B symptoms in lymphoma

Answer 12

weight loss >10% of TBW over 3mos; unexplained high-grade fever; drenching night sweats)

Question 13

Chemotherapeutic agenets for Hodgkin’s lymphoma

Answer 13

COPAD Cyclophosphamide; Vincristine; prednisine; Doxorubicin) or COMP (Cylophosphamide; vincristine; methotrexate; prednisone; 6mercaptopurine)

Question 14

treatment of hodgkin’s in patients with refractory disease or relapse

Answer 14

myeloblative autologous stem cell transplant

Question 15

3rd most common posterior fossa tumor of childhood infiltrates the pons and spreads throughout the brainstem

Answer 15

brain stem glioma

Question 16

common supratentorial tumor in children with solid and cystic areas that tend to calcify; shor stature; hypothyroidism; DI; adrenal insufficiency

Answer 16

craniopharyngioma

Question 17

most common malignant abdominal tumor

Answer 17

Wilm’s tumor?

Question 18

Wilm’s tumor is associated with what dse entities

Answer 18

neurofibromatosis; Beckwith-Wiedemann syndrome; WAGR syndrome

Question 19

hemihypertophy; visceromegaly; macroglossia

Answer 19

Becwith-Weidememann syndrome

Question 20

WAGR syndrome

Answer 20

Wilm’s tumor; aniridia; genito-urinary abnormalities; mental Retardation

Question 21

painless abdominal mass with flank mass that DOES NOT cross the midline

Answer 21

Wilm’s tumor

Question 22

3rd most common pediatric cancer

Answer 22

neuroblastoma

Question 23

pluripotent stem cells invaginate and migrate along the neuraxis where neural crest cells are present (sympathetic ganglia; adrenal glands)

Answer 23

neuroblastoma

Question 24

most common extracranial tumors in infants

Answer 24

neuroblastoma

Question 25

nontender abdominal mass which may cross the midline; Horner syndrome; HTN; cord compression(from a paraspinal tumor)

Answer 25

neuroblastoma

Question 26

most common sites of metastasis in neuroblastoma

Answer 26

long bones; skull; bone marow; liver; lymph nodes; skin

Question 27

Tx for St1(confined to structure of orgin) and St2(tumor beyond structure of origin but not cross the midline with 2B or without 2A LN involvement

Answer 27

surgery

Question 28

a tumor of striated muscle; most common soft tissue sarcoma in children

Answer 28

rhabdomyosarcoma

Question 29

histologic type of rhabdomyosarcoma with the worst prognosis

Answer 29

alveolar

Question 30

most common histologic type of rhabdomyosarcoma

Answer 30

embryonal

Question 31

vaginal grape-like mass type of rhabdomyosarcoma

Answer 31

sarcoma botyroides

Question 32

highest risk period for developing osteosarcoma

Answer 32

adolescent growth spurt

Question 33

risk factor in developing osteosarcoma

Answer 33

hereditary retinoblastoma

Question 34

most common SSx in osteosarcoma

Answer 34

pain; limp; swelling

Question 35

px with deep bone pain often nighttime awakening; palpable mass; xray demonstrating the lesion

Answer 35

osteosarcoma

Question 36

Xray finding in osteosarcoma

Answer 36

sunburst pattern (due to mineralization and formation of periosteal spicules in the surrounding tissues

Question 37

small; round cell undifferentiated tumor with small uniform nuclei and scant cytoplasm thought to be of neural crest origin; with systemic manifestations like fever and weight loss unlike osteosarcoma

Answer 37

Ewing’s sarcoma

Question 38

most likely chromosomal aberration in Ewing’s sarcoma

Answer 38

t(11;22) translocation

Question 39

Xray finding in Ewing’s sarcoma

Answer 39

onion-skinning or moth-eaten appearance (lytic bone lesion with periosteal reaction)

Question 40

what part of the bone is the most common location of osteosarcoma

Answer 40

metaphysis

Question 41

what part of the bone is the most common location of Ewing’s sarcoma

Answer 41

diaphysis