Hepatoblastoma Flashcards
What is the ddx for a liver mass in an infant?
Most likely:
hepatoblastoma vs mesenchymal hamartoma
Malignant:
Hepatoblastoma
rhabdoid tumor
Biliary tract rhabdomyosarcoma
Benign: teratoma
What is the workup for suspected HB?
AFP BhCG (ddx malignant germ cell of liver) LFT, coags, cbc Initial imaging is US, then MRI liver / ct liver CT chest Growing use of PET but not standard of care as per now.
no need for bone scan.
Anemia often present in infants with large liver mass, regardless of etiology, with throbocytosis as a compensatory mechanism
How to interpret a low AFP level when hepatoblastoma is suspected
AFP is usually in the thousands.
3 options
1) not a hepatoblastoma
2) false negative - ask lab to remeasure with a dilution (level may have been too high for standard detection)
3) A low AFP may be a marker of poor prognosis in 10% of hepatoblastoma
What is the principle of PRETEXT staging?
Pretreatment extent of disease
# of contiguous segment of liver, free of tumor. Out of a maximum of 4 segments. Pretext 1 better than 4.
Pretext 1: 3 contiguous seg
Pretext 2: 2 contiguous seg
Pretext 3: 1 free segment
Pretext 4: 0 free segment
Also, add annotation factor
What is an annotation factor?
V vascular
Circumferential involvement of 50% or more of 3 hepatic veins and/OR retrohepatic vena cava, and/OR evidence of thrombus in those vessels
P: circumferential involvement of > 50% L+R portal vein and/or of main portal vein, and/or venous thrombus
E Extrahepatic contiguous involvment
M Metastatic disease
C Caudate lobe involvment
F Multifocal tumor
R evidence of preoperative tumor rupture or hemorrhage
N perihepatic N+
What is the desired surgical margin for hepatoblastoma?
1 cm. if cannot obtain consider neoadjuvant chemo, then upfront transplant if remains impossible
Is a biopsy necessary for neoadjuvant treatment?
Yes.
need healthy liver and tumor.
Can use core biosy (US guided or lap assisted), lap, or open.
if core, need 2 healthy liver specimen, contiguous to tumor + 7 tumoral cores.
What are the different liver segments as per couinaud
Left Lateral 2-3
Left medial 4a, b
Right anterior: 5,8
Right Posterior: 6,7
Intraop landmark:
Falciform separates 2-3 / 4
Gallbladder is at junction of 4 & 5-8
What is the classic nuclear staining for hepatic rhabdoid tumors?
loss of INI-1 (negative)
What are the surgical guidelines for upfront resection of hepatoblastoma?
Proceed to resection w/o bx for :
PRETEXT 1
PRETEXT 2
w/o V-P-E-F-R
if you can obtain a 1 cm margin (vascular anatomy is the limiting fx)
What is POSTEXT?
Post chemotherapy extent of tumor. Basically, reassess extent of tumor using PRETEXT framework.
What is the management of lungs mets for hepatoblastoma?
In patient who can undergo standard liver resection, stage metastatectomy at the discretion of the treating team.
In patient who require liver transplant, perform metastatectomy soon after induction therapy as transplant team may need to demonstrate a metastasis free period before transplant.
which PRETEXT should be removed upfront?
PRETEXT 1-2 with neg annotation marker (V-P-E-F-R) in which a 1 cm margin can be obtained.
Which PRETEXT should be biopsied and refered for transplant assessment?
PRETEXT 4
Complex PRETEXT 3 resections
PRETEXT 3 with annotation factor (V-P-E-F-R)
Which PRETEXT should undergo bx + chemo (and differ referal to transplant)?
PRETEXT 1-2 with annotation factor or uncertain surgical margin
PRETEXT 3 w/o annotation factors and straightforward surgery
