Peds: Tumors

Front 1

what is the DDx of pediatric abdominal mass?

Back 1

wilms tumor, neuroblastoma, hernia, intussusception, malrotation w/volvulus, mesenteric cyst, duplications cyst, liver tumor (hepatoblastoma/hemangioma), rhabdomyosarcoma, teratoma

Front 2

what is wilms tumor?

Back 2

embryonal tumor of renal origin

Front 3

what is the incidence of wilms tumor? what's the average age at dx?

Back 3

very rare: 500 new cases in the US per year. average age: b/w 1-5yrs

Front 4

what are the sx of wilms tumor? signs?

Back 4

sx: usually asymptomatic except for abd mass, 20% of patients present w/minimal blunt trauma to mass. signs: abd mass (most do not cross midline), hematuria (10-15%), HTN in 20% of cases (related to compression of juxtaglomerular appartus), signs of beckwith-widemann syndrome

Front 5

what is the classic hx of wilms tumor?

Back 5

found during dressing or bathing

Front 6

what are the diagnostic radiologic tests for wilms tumor?

Back 6

abdominal and chest CT

Front 7

define stages I-III of wilms tumor

Back 7

I: limited to kidney and completely resected. II: extends beyond kidney but completely resected, capsule invasion and perirenal tissues may be involved. III: residual nonhematogenous tumor after resection

Front 8

define stages IV and V of wilms tumor

Back 8

IV: hematogenous metastases (lung, distal lymph nodes, brain). V: bilateral renal involvement

Front 9

what are the best indicators of survival in wilms tumor?

Back 9

signs and histologic subtype of tumor; 85% of patients have favorable histology, 15% have unfavorable histology; overall survival for FH is 85% for all stages

Front 10

what is the treatment of wilms tumor?

Back 10

radical resection of affected kidney w/evaluation for staging, followed by chemotherapy (low stages) and radiation (higher stages)

Front 11

what is neoadjuvant treatment for wilms tumor?

Back 11

large tumors may be shrunk w/chemotx/XRT to allow for surgical resection

Front 12

what are the associated abnormalities

Back 12

ANIRIDIA, hemihypertrophy, beckwith-widemann syndrome, neurofibromatosis, horseshoe kidney

Front 13

what is the beckwith-wiedemann syndrome

Back 13

syndrome of: 1. umbilical defect 2. macroglossia 3. gigantism 4. visceromegaly (big organs)

Front 14

what is neuroblastoma?

Back 14

embryonal tumor of neural crest origin

Front 15

what are the anatomic locations of neuroblastoma?

Back 15

adrenal medulla - 50%, paraaortic abdominal paraspinal ganglia - 25%, posterior mediastinum - 20%, neck - 3%, pelvis - 3%

Front 16

what is the incidence of neuroblastoma? what is the average age at dx?

Back 16

1/7000-10,000 live births, most common solid malignant tumor of infancy, most common solid tumor in children outside the US. ~50% dxed by 2yo, ~90% dx by 8yo

Front 17

with which types of neuroblastoma tumors does a patient w/horner's syndrome present?

Back 17

neck, superior mediastinal tumors

Front 18

what are the sx and signs of neuroblastoma?

Back 18

symptoms vary by tumor locatio - anemia, failure to thrive, wt loss, poor nutritional status w/advanced disease. signs: asymptomatic abdominal mass (palpable in 50% of cases), respiratory distress (mediastinal tumors), horner's syndrome (upper chest or neck tumors), proptosis (w/orbital metastases), subcutaneous tumor nodules, HTN (20-35%)

Front 19

labs for neuroblastoma?

Back 19

24-hr urine to measure VMA, HVA, and metanephrines (elevated in >85%), neuron-specific enolase, n-myc oncogene, DNA ploidy

Front 20

what are the diagnostic radiologic tests for neuroblastoma? what is the classic abdominal plain x-ray finding?

Back 20

CT scan, MRI, I-MIBG, somatostatin receptor scan. classic abdominal x-ray finding is calcifications (~50%)

Front 21

how do you assess bone marrow involvement for neuroblastoma?

Back 21

bone marrow aspirate

Front 22

what is the difference in position of tumors in neuroblastoma vs wilms tumors?

Back 22

neuroblastoma may cross the midline, wilms tumors do so only rarely

Front 23

tx of neuroblastoma

Back 23

depends on staging

Front 24

define stage I-III of neuroblastoma

Back 24

I: tumor confined to organ of origin. II: tumor extends beyond organ of origin but not across midline. III: tumor extends across the midline

Front 25

define stage IV and IVS of neuroblastoma

Back 25

IV: metastatic disease. IVS: infants - localized primary tumor does not cross midline but remote disease is confined to the liver, subQ/skin, and bone marrow

Front 26

what is the treatment of neuroblastoma stages I-III?

Back 26

I: surgical resection. II: resection and chemotx +/-XRT. III: resection and chemotx/XRT

Front 27

what is the treatment of neuroblastoma stage IV and IVS?

Back 27

IV: chemotx/XRT--> resection. IVS: in the infant w/small tumor and asymptomatic observe as many will regress spontaneously

Front 28

what is the survival rate of each stage of neuroblastoma?

Back 28

I: ~90%. II: ~80%. III: ~40%. IV: ~15%. IVS: >80% (these tumors are basically stage I or II w/mets to liver, subQ tissue, or bone marrow; most of these patients if younger than 1yo have a spontaneous cure)

Front 29

what are the lab prognosticators for neuroblastoma?

Back 29

aneuploidy is favorable! the lower the # of n-myc oncogene copies, the better the prognosis

Front 30

which oncogene is assoc w/neuroblastoma?

Back 30

n-myc oncogene

Front 31

what is rhabdomyosarcoma? what is its claim to fame?

Back 31

highly malignant striated muscle sarcoma. most common sarcoma in children

Front 32

what is the age distribution of rhabdomyosarcoma?

Back 32

bimodal: 2-5yrs, 15-19yrs

Front 33

what are the most common sites of rhabdomyosarcoma? what are the signs/sx?

Back 33

signs/sx: mass. sites: head and neck (40%), GU tract (20%), extremitites (20%)

Front 34

how is the dx made of rhabdomyosarcoma?

Back 34

tissue biopsy, CT scan, MRI, bone marrow

Front 35

what is the treatment of rhabdomyosarcoma?

Back 35

resectable: surgical excision +/- chemotherapy and radiation therapy. unresectable: neoadjuvant chemo/XRT, then surgical excision

Front 36

what is hepatoblastoma?

Back 36

malignant tumor of the liver (derived from embryonic stem cells)

Front 37

what is the average age at dx of hepatoblastoma? what is the male to female ratio?

Back 37

presents in 1st 3 yrs of life. male:female is 2:1.

Front 38

how is the dx of hepatoblastoma made?

Back 38

physical exam - abdominal distention, RUQ MASS THAT MOVES W/RESPIRATION; elevated serum alpha-fetoprotein and ferritin (can be used as tumor markers); CT scan of abdomen, which often predicts resectability

Front 39

what % of hepatoblastoma patients will have an elevated alpha-fetoprotein level?

Back 39

~90%

Front 40

what is the tx of hepatoblastoma?

Back 40

resection by lobectomy or trisegmentectomy is the tx of choice (+ postop chemotx); large tumors may require preop chemotx and subsequent hepatic resection

Front 41

what is the overall survival rate of hepatoblastoma?

Back 41

~50%

Front 42

what is the major difference in age presentation b/w hepatoma and hepatoblastoma?

Back 42

hepatoblastoma presents at younger than 3yo; hepatoma presents at older than 3yo and in adolescents