Cancer

Question 1

What is neuroblastoma?

Answer 1

Cancer arising in the adrenal medullary or less often sympathetic nervous system

Question 2

at what age is neuroblastoma typically diagnosed?

Answer 2

17 months

Question 3

where does neuroblastoma typically arise from?

Answer 3

abdomen (65%), thorax (15-20%)

Question 4

how does neuroblastoma present?

Answer 4

abd pain, discomfort, sense of fullness due to abdominal mass

Question 5

what are some paraneoplastic syndromes with neuroblastoma?

Answer 5

cerebellar ataxia, opsoclonus-myoclonus, watery diarrhea, or htn

Question 6

how do you dx neuroblastoma?

Answer 6

confirmed by bx

Question 7

how do you treat neurblastoma?

Answer 7

surgical resection, chemo, radiation therapy

Question 8

What is 4S disease

Answer 8

unique stage of neuroblastoma, small primary tumor origination location, limited mets to skin, liver, bone marrow, common to have spontaneous remission

Question 9

what is prognosis for 4S disease

Answer 9

depends on age at dx, state and biological factors, Low risk and intermediate - 90%, High risk: >50%, Good if limited stage, younger age

Question 10

What is wilms tumor

Answer 10

Nephroblastoma, embryonal cancer of kidney composed of blastemal, stromal and epithelial elements

Question 11

What is most common kindey cancer in children <15 years

Answer 11

Wilms tumor

Question 12

what is associated with wilms tumor?

Answer 12

genetic syndromes, most solitary

Question 13

how does wilms tumor present?

Answer 13

painless, palpable abdominal mass (80%)

Question 14

how do you diagnose wilms tumor

Answer 14

us, abdominal CT/MRI

Question 15

why would you rather do a neprectomy than biopsy for wilms tumor?

Answer 15

risk of peritoneal contaminiation by tumor cells

Question 16

how do you treat wilms tumor

Answer 16

surgical resection, chemo, radiation therapy

Question 17

what is prognosis for wilms tumor

Answer 17

overall outcome is excellent, possible recurrence

Question 18

what is the 2nd most common malignancy of children

Answer 18

CNS malignancies

Question 19

what are some risk factors for CNS malignancies

Answer 19

cranial irradiation, genetic/familiar sundromes, neurofibromatosis, tuberus sclerosis, li-fraumenia sundrome, von hippel-lindaue, turcot syndrome

Question 20

How do CNS malignancies present?

Answer 20

macrocephaly, bulging fontanelle and or splayed sutures, seizures, headaches

Question 21

what is best imaging for cns malignancies

Answer 21

MRI

Question 22

who gets an MRI to screen for CNS malignancies

Answer 22

persisitent vomiting upon wkaing, persistent headache >4 weeks, visual findings, motor findings

Question 23

what is initially done for imaging of CNS malignancies

Answer 23

CT

Question 24

what is retinoblastoma

Answer 24

cancer arising from immature retina

Question 25

how does retinoblastoma present

Answer 25

common leukocoria (white reflex in pupil), strabismus, inflammation, impaired vision

Question 26

how do you exam fundi for retinoblastoma

Answer 26

with pupils widely dilated, children usually need anesthesia

Question 27

how do you dx retinoblastoma

Answer 27

fundoscopic

Question 28

what do you find in a fundoscopic exam with retinoblastoma

Answer 28

cancers appear as single or multiple gray white elevations in retina, cancer seeds may be visible in vitreous

Question 29

if there is fx of retinoblastoma when does screening occur

Answer 29

shortly after birth then every 4 months until age 4 years old

Question 30

how do you treat retinoblastoma?

Answer 30

small cancers, bilateral dx, photocoagulation, cryotherapy, radiation therapy Advanced, larger: enucleation Chemotherapy sometimes used to reduce

Question 31

what is prognosis for retinoblastoma

Answer 31

overall 5 year - 95% metastasis is poor

Question 32

what is rhabdomyosarcoma

Answer 32

Arising from embryonic mesenchymal cells that have potential to differential into skeletal muscle cells Can arise from alone ant type of muscle tissue in any location, resulting in highly variable clinical manifestations Categorized as soft tissue sarcoma, most common cancer in this group, cancer typically detected by CT or MRI

Question 33

which is the 3rd most common solid canacer in children

Answer 33

rhabdomyosarcoma

Question 34

how does rhabdomyosarcoma present

Answer 34

head and neck region (35%), usu, in orbit or nasopharyngeal passages

Question 35

how do you diagnose rhabdomyosarcoma

Answer 35

biopsy

Question 36

how are head and neck lesions best imaged

Answer 36

MRI

Question 37

how are masses imagined

Answer 37

CT

Question 38

how do you treat rhamdbomyosarcoma

Answer 38

surgery, radiation, chemo

Question 39

what is prognosis for rhabdomyosarcoma

Answer 39

overall survival ranges from >90% in low risk dx, <50% in high risk dz

Question 40

what is ewing sarcoma

Answer 40

Believed to be neural crest cells in origin Mostly occur in bone, by can be extra osseous

Question 41

What is seen on XR for ewing sarcoma?

Answer 41

moth eaten, onion skinning

Question 41

where does ewing sarcoma aris

Answer 41

axial skeleton (48%), pelvis (26%), femur (20%)

Question 42

what is seen on XR with osteosarcoma

Answer 42

sunburst pattern, sclerotic

Question 43

how does osteosarcoma present

Answer 43

pain, limp, swelling

Question 44

where do you mostly see osteosarcoma

Answer 44

metaphysis of long boned

Question 45

what are risk factors for osteosarcoma

Answer 45

prior cancer or cancer tx, retinoblastoma, prior radiation, alkylating agents

Question 46

What is ALL

Answer 46

Most common pediatric cancer but can strike all ages, peak incidence 2-5 years - second peak after 50

Question 47

What are phases of ALL

Answer 47

Induction, consolidation, maintenance

Question 48

what is the goal for induction treatment for ALL

Answer 48

to complete remission: <5% blast cells in bone marrow, absolute neutrophil count >1000/mcL, a platelet count >100,000/mcL an dno need for blood transfusion

Question 49

what is sx of ALL

Answer 49

fatigue, pallor, infection, malaise, bone pain, CNS sx, easy bruising and bleeding