Hepatoblastoma Flashcards
Typical Age at diagnosis of hepatoblastoma?
3yrs with almost all diagnosed prior to 4.
Risk factors associated with hepatoblastoma?
Exposure to metals/paints/petroleum products FAS Maternal smoking OCP during pregnancy Infertility treatment Immunosuppressive meds during pregnancy
Genetic syndromes associated with HB?
BWS
FAP
Is there a relationship between prematurity and HB?
Yes.
15 x risk in infants weighing less than 1000g.
Pretext staging system with annotations?
Pretext group correlates to the number of contiguous segments free of tumor.
Pretext annotations:
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)
How does HB present?
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.
DDx by age?
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
Work up for liver tumor?
Labs: CBC, lytes, Liver panel, AFP, CEA (for suspected HCC), HCG for suspected germ cell, chatecholamines for suspected neuroblastoma mets.
What is the utility of AFP in HB?
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
Imaging characteristics on US, CT and MRI for HB?
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
What imaging is required for staging?
CT chest
PET?? Maybe
Histologic subtypes of hepatoblastoma?
- Well differentiated fetal (formerly pure fetal)
- Small cell undifferentiated
- Embryonal
- Macrotrabecular
- Mixed epithelial and mesenchymal
When do you biopsy HB vs. upfront resection?
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.
Standard chemo for HB?
Cisplatin - most effective
C5V- cisplatin, 5FU, vincrisitne
C5VD - C5V+ doxorubicin
Risk stratification for HB?
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.
How is relapse managed in HB?
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.
Surgical plan in HB:
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.
5 year Survival per risk group for HB?
Very low: 100%
Low: 90-98%
Intermediate: 40-70%
High: 20-60%
