Liver

Question 1

Compare HB and HCC epidemiology and RF

Answer 1

HB 90% <5yo
HCC 95% >15yo, 3M:F

HB: BWS, hemihypertrophy, FAP, Glycogen storage disorders, FAS, prem/LBW

HCC: glycogen storage disorders, A1-AT, hepatitis B/C, hereditary tyrosinemia, Alagille, NF-1, AT, FA

Question 2

HB and HCC histologic subtypes with low/N AFP

Answer 2

HB small cell undifferentiated
HCC fibrolamellar

Question 3

HB histologic subtypes and genetics

Answer 3

Epithelial (85-90%)
Fetal (10%): less aggressive
small cell undifferentiated (5%): aggressive, SMARCB1 mutation, low/N AFP

WNT
CTNNB1 activation (somatic) 70%

Question 4

HCC histologic subtypes

Answer 4

Conventional (80%)
Fibrolamellar (20%): AYA, low/N AFP, DNAJB1-PRKACA fusion

WNT
EPHB2
TGFB1/MTOR

Question 5

Transplant for HB and HCC?

Answer 5

HB: 10%; PRETEXT 4 or V+/P+
HCC: uncommon; multifocal confined to liver

Question 6

Liver COG vs PRETEXT staging

Answer 6

COG postsurgical
I: GTR
II: microscopic residual disease (+ margins)
III: gross residual disease and/or nodal involvement
IV: metastatic

PRETEXT pre-surgical
Group + (# nonadjacent sections free of disease) = 4
I: 3 adjacent unaffected
II: <3 adjacent unaffected
III: 3 sections, or 2 nonadjacent sections unaffected
IV: all 4 sections

V: 3 hepatic veins/intrahepatic IVC
P: both portal veins
E: extrahepatic extension
F: multifocal
R: rupture
M: distant mets

Question 7

HB risk stratification COG vs SIOP

Answer 7

COG
VLR: PRETEXT I-II fetal histo resected
LR: PRETEXT I-II resected
IR: PRETEXT II-IV unresectable; V/P/E; SCU histo
HR: M+, AFP <100

SIOP
SR: PRETEXT I-III
HR: V/P/E/M; SCU; AFP<100; rupture

Question 8

HB Tx

Answer 8

COG: resection –> adj chemo (not pure fetal)

SIOP: neoadj chemo –> resection

chemo: cisplat +- doxo (SIOP HR neoadj)

AHEP1531
- LR: cisplat
- IR: cisplat, 5FU, VCR, doxo
- HR: carbo/doxo, carbo/etop, VCR/irenotecan

Question 9

HB Px

Answer 9

VLR: OS 100%
LR: OS/EFS >90%
IR: OS/EFS >80%
HR: OS/EFS 40-60%

Worse: higher PRETEXT, PRETEXT annotation, AFP <100 or >1M, 8+yo

Question 10

HCC Tx

Answer 10

complete resection
unresectable: neoadj

No RT

AHEP1531
- resected: cisplat/doxo (PLADO)
- unresectable/mets: cisplat/doxo/sorafenib vs addition of gemcitabine/oxaliplatin/sorafenib

Question 11

HCC Px

Answer 11

All 5yOS 42%

localized 5yEFS <50%
distant mets 5yEFS <20%

Question 12

Liver tumor - AFP prognostic?

Answer 12

<100 or >1M worse

Question 13

HB criteria for no chemo

Answer 13

Pure fetal histology
PRETEXT I
complete resection

Question 14

HB/HCC labs

Answer 14

AFP elevation
abnormal LFTs
thrombocytosis (IL-6 secretion)
rare hyperphos, Cushing