Liver tumors

Question 1

5 congenital risk factors for hepatoblastoma

Answer 1

familial polyposis, Gardners, BWS, hemihypertrophy, glycogen storage disease

Question 2

hepatoblastoma= environmental risk factors

Answer 2

Fetal alcohol syndrome
* Prematurity and low birth weight
(15-20 x < 1,000g)
* Oral Contraceptives
* Oral Gonadotropins
* Parental exposure to metal,
petroleum products, paint

Question 3

congenital RF for hepatocellular carcinoma

Answer 3

Hereditary Thyrosenemia
* Biliary cirrhosis
* Glycogen storage diseases
* α-1 antitrypsin deficiency
* Hemochromatosis

Question 4

3 histological subtypes of hepatoblasoma

Answer 4

epithelial (fetal and embryonal), pure fetal (great prognosis) , and small cell undifferentiated (very bad)

Question 5

association- genetics, small cell undifferentiated histology

Answer 5

22q11 abberations

Question 6

3 subtypes of hepatocellular carcinoma

Answer 6

conventional, fibrolamellar (more protracted presentation, lower AFP) vs hepatocellular NOS

Question 7

what type of peds hepatoblastoma can be surgery only

Answer 7

stage 1 pure fetal

Question 8

only curative therapy for hepatocellular carcinoma

Answer 8

full resection or transplant

Question 9

most common molecular changes in thyroid cancer for peds

Answer 9

BRAFs

Question 10

syndrome to associate with medullary thyroid carcinoma

Answer 10

men2 syndrome

Question 11

who gets ppx thyroidectomy

Answer 11

MEN2b, 2A

Question 12

most common histology for nasopharyngeal carcinoma in children

Answer 12

type 3- undifferentiated carcinoma