Hepatic tumours, ASPHO Flashcards
liver tumours = what percent of tumours in kids?
1%
median age of liver tumours in kids?
19 mos…80% under 15 yrs
hepatoblastoma = ___ most common intra-abdo tumour in kids
3rd
incidence of hepatoblastoma?
11.2 per million
91% of hepatoblastoma cases occur before what age?
5
liver malig in adolescents?
hepatocellular carcinoma; >95% of liver tumours in people over age 15
hepatocellular ca is more common where? why?
asia and africa; endemic hep b
DDX for benign liver tumours in infancy? (3)
hemangioendothelioma, mesenchymal hamartoma, teratoma
ddx for malig liver tumours in infants? (4)
HEPATOBLASTOMA, rhabdoid tumour, yolk sac tumour, LCH, M7 AML, disseminated NBL
ddx of benign liver tumours age 1-3?
hemangioendothelioma, mesenchymal harmatoma
ddx for malig liver tumours age 1-3?
HBL, rhabdomyosarcoma, inflammatory myofibroblastic tumour
ddx for benign liver tumour age 3-10? (1)
perivascular epithelioid cell tumours (PE-comas)
ddx for malig liver tumours age 3-10? (4)
hepatocellular carcinoma, embryonal sarcoma, angiosarcoma, cholangiocarcinoma, endocrine (gastrin) carcinoma
ddx for benign liver tumours age 10-16? (3)
adenoma, focal nodular hyperplasia, biliary cystadenoma
ddx for malig liver tumours in age 10-16? (3)
conventional and fibrolamellar hepatocellular carcinoma, hodgkin lymphoma, leiomyosarcoma
describe bio of HBL
- activation of WNT pathway, including activating mutations of CTNNB1 (or APC) in >90% of cases; upregulation of Wnt signaling pathway genes, like DKK1, APCDD1, AXIN2, CCND1
- NFE2L2 (NRF2) mutation in 5%
- Other alterations= mutations in epigenetic modifiers like MLL2, ARID1a or variable expression of Lin-28, AFP, Notch
give 5 congenital risk factors for HBL
“Genetics For Hepatic Glitches at Birth”
- familial polyposis
- Gardner syndrome
- Beckwith-Wiedemann Syndrome
- Hemihypertrophy
- Glycogen storage disease
Give 5 enviornemntal RFs for HBL
“Other Features Predisposing GI Malignant Conditions”
- Fetal alcohol syndrome
- Prematurity and LBW
- Oral contraceptives
- Oral gonadotropins
- Parental exposure to metal/petroleum/paint
BWS: 1) involves what chrom? 2)incidence?
3) give 5 features
1) 11p15.5
2) 1 in 10k
3) abdo wall defects, macroglossia, GU malformations, organomegaly, hyperinsulinemic hypoglycemia
BWS: malig risk in first decade of life?
8-10%
Give 5 tumours associated with BWS and the incidence of the 2 most common
Wilms: 43% HBL: 20% Adrenocortical tumours (7%) NBL RMS Pancreatoblastoma Leukemia
Hemihypertrophy incidence?
1 in 86k
Hemihypertrophy can be ISOLATED or associated with which syndromes (give 3)?
BWS
Klippel-Trenaunay-Weber
McCune-Albright Syndrome
Malignancy risk with hemihypertrophy?…give 3 tumours that are associated
5%
Wilms, HBL, adrenocortical tumours