Hepatic tumors

Question 1

Risk factors for Hepatoblastoma

Answer 1

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

Question 2

Hepato prog factors

Answer 2

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

Question 3

Why is pure fetal histology important

Answer 3

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

Question 4

Why is small cell variant of hepato important

Answer 4

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

Question 5

Risk factors for HCC

Answer 5

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

Question 6

Hepato outcomes

Answer 6

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

Question 7

Common presenting signs/symptoms of Hepatoblastoma

Answer 7

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

Question 8

Workup of liver mass

Answer 8

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

Question 9

Which hepato/HCC has normal AFP?

Answer 9

SCU hepatoblastoma

fibrolamellar HCC

Question 10

Describe PRETEXT staging

Answer 10

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

Question 11

What is the half-life of AFP

Answer 11

5-7 days

Question 12

What are the additional letters with SIOPEL staging

Answer 12

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

Question 13

What are the additional letters with SIOPEL staging

Answer 13

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

Question 14

What are SIOPEL risk groups?

Answer 14

Standard risk: Pretext 1,2,3

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

Question 15

What are the COG risk groups?

Answer 15

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

Question 16

Prognostic factors for HCC

Answer 16

Stage

GTR (necessary for cure)

Question 17

Pros of SIOPEL vs COG approaches

Answer 17

COG - can catch pure fetal histology and avoid chemo

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

Question 18

Which chemotherapy is active in hepatoblastoma

Answer 18

Cisplatin
Carboplatin
5-FU
VCR
Doxo
Ifos

Question 19

Long-term toxicity for HR hepato treatment

Answer 19

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