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Flashcards in Hepatic tumors Deck (19):
1

Risk factors for Hepatoblastoma

Prematurity
Familial adenomatous polyposis
Gardner syndrome
Beckwith-Wiedemann syndrome
Hemihyperplasia syndromes (formerly hemihypertrophy)
Glycogen storage diseases
Other associated congenital anomalies: Meckel’s diverticulum, congenital absence of adrenal gland, congenital absence of kidney, umbilical hernia

2

Hepato prog factors

1)Higher pretext group
- Gross total resection
- Response to chemo (30% by RECIST criteria) or 90% decrease in AFP levels - also a marker of resectability of tumor if it shrinks well.

2) Positive pretext annotations (i.e. VPEFRM)

3) Low AFP (associated with small cell undifferentiated variant)

4) Older age (>8 y.o.)

5) Pure fetal histology

6) Decrease of AFP as predicted by half life

3

Why is pure fetal histology important

COG study showed pure fetal histology with complete resection (COG stage1) can be treated with surgery alone OS 100%

4

Why is small cell variant of hepato important

associated with low AFP and younger age (6-10m)

associated with 22q11 and INI negative staining

has poor prognosis - dismal if unable to achieve GTR

Automatically upgrade to at least IR therapy

5

Risk factors for HCC

-tyrosinemia
-biliary cirrhosis
- Glycogen storage disease
- alpha-1-antitrypsin deficiency
- hemochromatosis
- Hep B & C
- Alcohol
- anabolic steroids

6

Hepato outcomes

LR - 90%+
IR - 70-90%
HR - 40-60%

7

Common presenting signs/symptoms of Hepatoblastoma

Asymptomatic abdo mass
Pain
Elevated AFP (90%)
Thrombocytosis
Rarely hypertension and precocious puberty

8

Workup of liver mass

Imaging:
- CT/MRI of primary
- U/S with dopper of primary to assess patency of vessels
- CT chest/abdo/pelvis for mets

Labs:
- CBC
- liver enzymes and function
- AFP

Tissue:
- Biopsy

Other:
- liver transplant consult
- audiogram/echo in anticipation of therapy

9

Which hepato/HCC has normal AFP?

SCU hepatoblastoma
fibrolamellar HCC

10

Describe PRETEXT staging

PRETEXT staging is determined by the number of contiguous UNINVOLVED sections of the liver.

PRETEXT stage = the number of contiguous uninvolved segments subtracted from 4

11

What is the half-life of AFP

5-7 days

12

What are the additional letters with SIOPEL staging

V - extension in IVC or all 3 hepatic veins

P - extension into both branches or main portal vein

E- extra hepatic disease (biopsy proven)

M - distant mets

13

What are the additional letters with SIOPEL staging

V - extension in IVC or all 3 hepatic veins

P - extension into both branches or main portal vein

E- extra hepatic disease (biopsy proven)

F - multifocal

R - tumor rupture

M - distant mets

14

What are SIOPEL risk groups?

Standard risk: Pretext 1,2,3

High risk: Pretext 4, V+, E+, P+, M+, AFP < 100, tumor rupture

15

What are the COG risk groups?

Very Low: PXT 1/2 with FH

Low: PXT 1/2

IR: PXT 2,3,4 unresectable, V+, E+, P+, SCU

HR: any pretext with M+, low AFP

16

Prognostic factors for HCC

Stage
GTR (necessary for cure)

17

Pros of SIOPEL vs COG approaches

COG - can catch pure fetal histology and avoid chemo

SIOPEL - more time for surgical planning and easier resection post neo-adjuvant chemo

18

Which chemotherapy is active in hepatoblastoma

Cisplatin
Carboplatin
5-FU
VCR
Doxo
Ifos

19

Long-term toxicity for HR hepato treatment

Hearing loss
Therapy-related AML/PTLD
Cardiotoxicity
Renal toxicity
Fertility
Growth and learning
Immunosuppression, infection