childhoodcancers

Question 1

what are the most common cancers of childhood

Answer 1

Leukemia 40%, CNS 27%, soft tissue 9%, non-hodgkin7&, kidney 7%, bone 6%, Hodgkin 4%

Question 2

what is the epidemiology of cancer

Answer 2

genetic mutations with an environmental exposure. Genetic risk include neurofibromatosis, familial polyposis, li-fraumeni syndrome, liked diseases include immune deficiencies, metabolic disorders, disorders of chromosome stability.

Question 3

tell me about retinoblastoma

Answer 3

Rare 1% of childhood cancers, 80% in children under 3, 30% are bilateral

Question 4

what is the epidemiology of retinoblatomas cancer

Answer 4

loss of both RB genes 60% spontanoues and 40% hereditary.

Question 5

what is the treatment for retinoblastoma

Answer 5

radiation chome and enucleation

Question 6

what is the prognosis of retinoblastoma

Answer 6

85% long term survival rate.

Question 7

what are the symptoms and signs for leukemia

Answer 7

fever for more than a week, petechiae, anemia, lymphadenopathy, hepatosplenomegaly, bone or joint pain without relief from meds.

Question 8

what are the CNS symptoms concerning brain tumors

Answer 8

seizures, weakness, difficulties, csf circulation problems causing pressure, headache and vomiting

Question 9

what does the location of lymphadenopathy tell us about the malignancy

Answer 9

if its generalized its more likely in the malignant except EBV, if its regional not involving the head and neck more likely malignant, if its hard/rubbery non tender matted node is more malignant, mediastinal or auricular or epitrochlear/supraclaviclicular are more malignant

Question 10

when do you biopsy in children

Answer 10

all masses, bone and joint pain,

Question 11

what is the most common malignancy in children

Answer 11

acute lymphoblastic leukemia, 2-5 year old boys 1/3 of all childhood malignancies more common in Caucasian . Down syndrome patients.

Question 12

what is the pathogenis of acute lymphoblastic leukemia or ALL

Answer 12

a lymphoid progenitor cell become genetically altered and undergoes unregulated clonal expansion, starts in the bone marow and the moves to everywhere like liver CNS testes lymph nodes,

Question 13

what are the signs and symptoms of ALL

Answer 13

Bone marrow infiltration: anemia low platelets, petechiae purpura murmur, bone pain fever, limp and fever, Extremeduallary gingival hyperthrophy facial nerve palsy. Enlarged liver mediastininal testical enlargement.

Question 14

what do you see in the CBC for ALL

Answer 14

50% will have increaased WBC 80% will have lymphoblasts on the peripheral smear, 95% will have more thn 2 cytopenias

Question 15

what percent of ALL are b cell precursors

Answer 15

80-85% primarily in the bone marrow presentations whereas 15% are t cell and are more mediastinal mass

Question 16

what is the treatment for ALL

Answer 16

chemotherapy: in blood and in the CSF if needed. 95% attain remission 75-85% survive 5 years without recurrence. Osteonecrossis osteopenia neuropathies, cardiace problems, obesity.

Question 17

tell me about AML

Answer 17

prognostic indicators are age and race with cytogenetics used to determine treatment options, treat with cytarabine, dauno lots of cardiace toxicity 2nd malignancies endocrinopathies.

Question 18

how common are brain tumors in children

Answer 18

Comprise 20% most common solid tumor in children

Question 19

tell me about low grade gliomas astrocytomas

Answer 19

is most common brain tumor

Question 20

what are the sings and symptoms of brain tumors

Answer 20

in the posterior fossa ataxia tremors stiff neck papilledema in the brainstem extremity weakness double vision facial weakness swallowing dysfunction , hemishperic tumors have hemiparesis hemianopsia aphasia and seizures.

Question 21

what is the work up for a brain tumor

Answer 21

ct but MRI better biopsy preferred by excision

Question 22

what is the treatment for brain tumors

Answer 22

surgery if we can remove most of it than we are doing well. Unless its a high grade fast dividing glioma then you might need radiation. Radation can be used in all brain tumors except choriod plexas tumors chemo therapy adjunctive.

Question 23

what is the most common epidemiology of astrocytomas

Answer 23

NF-1 mutation accounts for nearly 50-80% of all cases . Then li fraumenia syndrome and radation of the head and neck.

Question 24

what is the prognossis of astrocytomas =, medulloblastomas, ependymoma, and brain stem gliomas

Answer 24

astrocytomas, low 50-80 high 30%, medulloblastoma standard risk 80% high risk 50%, epndymoma neart to gross resection 50-75% less than NTR 0-30% brain stem gliomas more than 90% by 2 years.

Question 25

what is the leading cause of morbidity and mortality in pedaictric cancers

Answer 25

brain tumors

Question 26

tell me about wilms tumor

Answer 26

end most common abdominal malignancy, in children, caused by a muataion in the WT1 tumor suppressor gene needed for renal development.

Question 27

what is the incidence of wilms tumors

Answer 27

renal tumors represent 5-6% of pediatric cancers, higher in AA and lower in asains, peak at ages 2-3 rare in kids older than 5

Question 28

what are the anomilies and syndroms associated with wilms tumors

Answer 28

hemihypertrophy, aniridia, denys-drash syndrome GU anomiales and renal failure, bechwith wiedmann syndrome

Question 29

what ado you see with wilms tumors in the physical exam

Answer 29

large palpable abdominal mass blood in the urine. Hypertension fever, hypotension anemia and fever.

Question 30

what is the staging of wils tumors

Answer 30

1 limited to kidney but resection, 2 beyond kidney but complete resection, 3 residual tumor confined to abdomen,4 hematogenous mets lung liver bone brain or lymph nodes, 5 bilateral renal involvement at diagnosis. Tumor spill at time of surgery considered stage 3

Question 31

what is the treatment for wilms tumors

Answer 31

all stages have a nephrectormy and stage 1 only has nephrectomy. except bilateral, three and up get radiation and all get chemo with vincristine and dactinomycin, higher ups get doxorubicin ectoposide, carboplatin and cytoxan.

Question 32

what is the prognosis of wilms tumor

Answer 32

80-90% survival when bilateral 70-80% survival

Question 33

tell me about neuroblastomas

Answer 33

Ages 2-5 and neural crst tissue adrenal or paraspinal sympathetic tissue, often met at diagnosis to bone or bone marrow. Most commone extra crainial solid tumor in children most comon from adrenal gland

Question 34

what are the signs and symptoms of Neruoblastomas

Answer 34

abdominal mass, lower extremity weakness, spinal cord compression, cervial high throacic mass horners syndrome [miosis, ptosis, anhydrosis] in the met disease, irritability weight loss bone pain fever, proptosis bone lesion periorbital eccymoses raccon eye.

Question 35

what is the treatment fro neruoblastomas

Answer 35

surgery chomo stem cell transplant radiation

Question 36

what is the prognosis of neuroblastomas

Answer 36

by stage 1 90%, 2 75% 3 40-70%, 4 60% if less than 1 year 30% if 1-2 years old and 20% in more than 2 years old.

Question 37

what is the take home from ewings sarcomas

Answer 37

peak in adolescents , more common in males found after a sports injury.