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42 Cards in this Set

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what is the DDx of pediatric abdominal mass?
wilms tumor, neuroblastoma, hernia, intussusception, malrotation w/volvulus, mesenteric cyst, duplications cyst, liver tumor (hepatoblastoma/hemangioma), rhabdomyosarcoma, teratoma
what is wilms tumor?
embryonal tumor of renal origin
what is the incidence of wilms tumor? what's the average age at dx?
very rare: 500 new cases in the US per year. average age: b/w 1-5yrs
what are the sx of wilms tumor? signs?
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
what is the classic hx of wilms tumor?
found during dressing or bathing
what are the diagnostic radiologic tests for wilms tumor?
abdominal and chest CT
define stages I-III of wilms tumor
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
define stages IV and V of wilms tumor
IV: hematogenous metastases (lung, distal lymph nodes, brain). V: bilateral renal involvement
what are the best indicators of survival in wilms tumor?
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
what is the treatment of wilms tumor?
radical resection of affected kidney w/evaluation for staging, followed by chemotherapy (low stages) and radiation (higher stages)
what is neoadjuvant treatment for wilms tumor?
large tumors may be shrunk w/chemotx/XRT to allow for surgical resection
what are the associated abnormalities
ANIRIDIA, hemihypertrophy, beckwith-widemann syndrome, neurofibromatosis, horseshoe kidney
what is the beckwith-wiedemann syndrome
syndrome of: 1. umbilical defect 2. macroglossia 3. gigantism 4. visceromegaly (big organs)
what is neuroblastoma?
embryonal tumor of neural crest origin
what are the anatomic locations of neuroblastoma?
adrenal medulla - 50%, paraaortic abdominal paraspinal ganglia - 25%, posterior mediastinum - 20%, neck - 3%, pelvis - 3%
what is the incidence of neuroblastoma? what is the average age at dx?
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
with which types of neuroblastoma tumors does a patient w/horner's syndrome present?
neck, superior mediastinal tumors
what are the sx and signs of neuroblastoma?
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%)
labs for neuroblastoma?
24-hr urine to measure VMA, HVA, and metanephrines (elevated in >85%), neuron-specific enolase, n-myc oncogene, DNA ploidy
what are the diagnostic radiologic tests for neuroblastoma? what is the classic abdominal plain x-ray finding?
CT scan, MRI, I-MIBG, somatostatin receptor scan. classic abdominal x-ray finding is calcifications (~50%)
how do you assess bone marrow involvement for neuroblastoma?
bone marrow aspirate
what is the difference in position of tumors in neuroblastoma vs wilms tumors?
neuroblastoma may cross the midline, wilms tumors do so only rarely
tx of neuroblastoma
depends on staging
define stage I-III of neuroblastoma
I: tumor confined to organ of origin. II: tumor extends beyond organ of origin but not across midline. III: tumor extends across the midline
define stage IV and IVS of neuroblastoma
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
what is the treatment of neuroblastoma stages I-III?
I: surgical resection. II: resection and chemotx +/-XRT. III: resection and chemotx/XRT
what is the treatment of neuroblastoma stage IV and IVS?
IV: chemotx/XRT--> resection. IVS: in the infant w/small tumor and asymptomatic observe as many will regress spontaneously
what is the survival rate of each stage of neuroblastoma?
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)
what are the lab prognosticators for neuroblastoma?
aneuploidy is favorable! the lower the # of n-myc oncogene copies, the better the prognosis
which oncogene is assoc w/neuroblastoma?
n-myc oncogene
what is rhabdomyosarcoma? what is its claim to fame?
highly malignant striated muscle sarcoma. most common sarcoma in children
what is the age distribution of rhabdomyosarcoma?
bimodal: 2-5yrs, 15-19yrs
what are the most common sites of rhabdomyosarcoma? what are the signs/sx?
signs/sx: mass. sites: head and neck (40%), GU tract (20%), extremitites (20%)
how is the dx made of rhabdomyosarcoma?
tissue biopsy, CT scan, MRI, bone marrow
what is the treatment of rhabdomyosarcoma?
resectable: surgical excision +/- chemotherapy and radiation therapy. unresectable: neoadjuvant chemo/XRT, then surgical excision
what is hepatoblastoma?
malignant tumor of the liver (derived from embryonic stem cells)
what is the average age at dx of hepatoblastoma? what is the male to female ratio?
presents in 1st 3 yrs of life. male:female is 2:1.
how is the dx of hepatoblastoma made?
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
what % of hepatoblastoma patients will have an elevated alpha-fetoprotein level?
~90%
what is the tx of hepatoblastoma?
resection by lobectomy or trisegmentectomy is the tx of choice (+ postop chemotx); large tumors may require preop chemotx and subsequent hepatic resection
what is the overall survival rate of hepatoblastoma?
~50%
what is the major difference in age presentation b/w hepatoma and hepatoblastoma?
hepatoblastoma presents at younger than 3yo; hepatoma presents at older than 3yo and in adolescents