Hepatoblastoma

Question 1

Typical Age at diagnosis of hepatoblastoma?

Answer 1

3yrs with almost all diagnosed prior to 4.

Question 2

Risk factors associated with hepatoblastoma?

Answer 2

Exposure to metals/paints/petroleum products
FAS
Maternal smoking
OCP during pregnancy
Infertility treatment
Immunosuppressive meds during pregnancy

Question 3

Genetic syndromes associated with HB?

Answer 3

BWS

FAP

Question 4

Is there a relationship between prematurity and HB?

Answer 4

Yes.

15 x risk in infants weighing less than 1000g.

Question 5

Pretext staging system with annotations?

Answer 5

Pretext group correlates to the number of contiguous segments free of tumor.

Question 6

Pretext annotations:

Answer 6

C - caudate lobe
E - extrahepatic disease
F - multifocal
R - tumor rupture
M - mets
P - portal vein (1 branch or 2 main portal V)
N - Nodes (1 intra abdo or 2 extra abdo)
V - Vena cava ( 1 vein, 2 veins or 3 IVC/all 3)

Question 7

How does HB present?

Answer 7

Asymptomatic mass in infant or toddler.
Late presentation may have wight loss, fever, and resp distress.

Rare* precocious puberty

90% of malignant tumors under 4 yrs is HB.

Question 8

DDx by age?

Answer 8

Infant/Toddler:
Malignant - HB, rhabdoid, teratoma, biliary rhabdo.
Benign - hemangioma, mesenchymal hamartoma, other

School age:
Malignant - HCC (FL-HCCa, HC-NOSb), embryonal sarcoma of the liver
Benign - FNH

Adolescents:
Malignant - HCC, sarcoma
Benign - adenoma

Question 9

Work up for liver tumor?

Answer 9

Labs: CBC, lytes, Liver panel, AFP, CEA (for suspected HCC), HCG for suspected germ cell, chatecholamines for suspected neuroblastoma mets.

Question 10

What is the utility of AFP in HB?

Answer 10

Elevated in 80-90% of all HB

• may also be elevated in HCC, or germ cell tumors.
• may not be elevated above normal levels in neonatal tumors when charted on nomogram.

AFP normalizes by 6 months

Question 11

Imaging characteristics on US, CT and MRI for HB?

Answer 11

US - solitary hyperechoic masses, calcifications with areas of necrosis or bleeding.
-Doppler will give information on relationship to vessels and thrombus.

CT- arterial and venous phase. Usually have decreased enhancement relative to liver with heterogenous pattern.

MRI - hyper intense on T2

Question 12

What imaging is required for staging?

Answer 12

CT chest

PET?? Maybe

Question 13

Histologic subtypes of hepatoblastoma?

Answer 13

• Well differentiated fetal (formerly pure fetal)
• Small cell undifferentiated
• Embryonal
• Macrotrabecular
• Mixed epithelial and mesenchymal

Question 14

When do you biopsy HB vs. upfront resection?

Answer 14

Pretext 1 and 2 should be respected upfront unless annotation factors preclude complete resection.

Everything else is biopsies upfront - 3-5 cores should be obtained and 1 core of normal liver.

Question 15

Standard chemo for HB?

Answer 15

Cisplatin - most effective

C5V- cisplatin, 5FU, vincrisitne

C5VD - C5V+ doxorubicin

Question 16

Risk stratification for HB?

Answer 16

Very low risk: Pretext 1/2 with pure fetal and fully resected. AFP > 100 Ng/ml

Low risk: Pretext 1/2 with histology other than pure fetal (but not small cell). AFP > 100

Intermediate risk: Pretext 2/3/4 that are unresectable up front. Or have small cell on upfront resection.
Resectability is assessed after 2-4 cycles of C5VD.

High risk: all patients with AFP < 100 or mets at diagnosis. Chemo upfront with surgery considered after 6 cycles.

Question 17

How is relapse managed in HB?

Answer 17

Rise in AFP is usually first sign of relapse.

40% of relapse patients can have second complete response with combined surgery and chemo including resection of local recurrence and pulmonary disease.

Question 18

Surgical plan in HB:

Answer 18

All tumors with 1 cm margin achievable in the middle hepatic vein and resectable with hemihepatectomy should be resected up front.

If neo adjucent chemo is given then resectability is assessed post cycle 2. Post text 3/4 unresectable tumors should be referred for transplant.

Resection should take place post cycle two or 4 for tumors that become resectable.

Lung mets persisting after chemo should be resected.

Question 19

5 year Survival per risk group for HB?

Answer 19

Very low: 100%

Low: 90-98%

Intermediate: 40-70%

High: 20-60%