Grand Rounds · Hepatoblastoma with Dr. Greg Tiao
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Podcast10 min·Published Feb 2022Older

Hepatoblastoma with Dr. Greg Tiao

With Dr. Greg Tiao · Grand Rounds
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What the experts said11 expert statements · 13 host summaries
There are around 250 new cases of hepatoblastoma per year.
EpidemiologicalGreg Tiao
Hemangioma is the most common liver lesion in children.
EpidemiologicalGreg Tiao
EOVIST is an MRI contrast agent that is taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging and distinguishing biliary anatomy.
ClinicalGreg Tiao
Couinaud labeled the liver segments in a counterclockwise fashion after the districts of Paris, by injecting portal vessels.
ClinicalGreg Tiao
The right hepatic vein differentiates the anterior sections (segments 5 and 8) from the posterior sections (segments 6 and 7) of the right liver.
ClinicalGreg Tiao
The right portal vein separates superior segments (7 and 8) from inferior segments (5 and 6) in the right liver.
ClinicalGreg Tiao
The change in practice for PRETEXT 3 and 4 tumors improved survival rates for patients with hepatoblastoma from less than 30% in the 1970s and 1980s to between 80 and 90% today.
EpidemiologicalGreg Tiao
PRETEXT 3 tumors should generally be biopsied at diagnosis and then started on neoadjuvant chemotherapy, with readiness to send patients to a transplant center or a center with expertise in advanced liver resections depending on response.
GuidelineGreg Tiao
For PRETEXT 3 or 4 liver disease, specific indications for transplant include unresectable disease, unsafe resection, or resection that would leave the patient with an inadequate liver remnant.
GuidelineGreg Tiao
Teams are now trained to recognize hepatoblastoma tumors and send them to centers that offer transplant or can do aggressive resection, resulting in more patient referrals than 15 years ago.
ClinicalGreg Tiao
The FIT study has been ongoing for three years.
ClinicalGreg Tiao
Hepatoblastoma is the most common malignant liver tumor in the pediatric population.
Host summary
Neuroblastoma is the most common solid malignancy in the pediatric population.
Host summary
The differential diagnosis for a liver mass should include hepatoblastoma, hemangioma, hematomas, focal nodular hyperplasia, and hepatocellular carcinoma.
Host summary
Hepatoblastoma can present as an asymptomatic abdominal mass, tumor rupture, respiratory distress due to tumor size, or as a paraneoplastic syndrome like precocious puberty.
Host summary
An elevated alpha-fetoprotein (AFP) is a key component in the workup of hepatoblastoma and is critical for the diagnostic algorithm and as a guide for treatment response.
Host summary
MRI is relied upon by most experts to delineate the anatomy and evaluate the extent of liver involvement in hepatoblastoma.
Host summary
To establish a diagnosis of hepatoblastoma, four things are needed: history, abdominal imaging, AFP check, and liver biopsy.
Host summary
The most important intervention for survival in hepatoblastoma is to achieve complete resection.
Host summary
Aggressive resections for hepatoblastoma include trisectionectomy, mesohepatectomy, resection with an additional procedure in the remnant liver, and liver transplantation.
Host summary
In the PRETEXT staging system, PRETEXT 1 has 3 contiguous sections free of disease, PRETEXT 2 has 2 contiguous sections free, PRETEXT 3 has 1 free section, and PRETEXT 4 has no free sections or diffuse tumor burden in all sections.
Host summary
PRETEXT 1 and 2 tumors with greater than a centimeter margin from the middle hepatic vein and the portal bifurcation should be considered for resection at diagnosis.
Host summary
It is important to consult a transplant center early for PRETEXT 3 and 4 tumors.
Host summary
The Pediatric Hepatic International Tumor Trial (FIT) is an ongoing multinational trial aimed at identifying high and low risk features for each of the PRETEXT stages of hepatoblastoma.
Host summary