Grand Rounds · Hepatoblastoma with Dr. Greg Tiao
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Video·Published Mar 2022Older

Hepatoblastoma with Dr. Greg Tiao

With Dr. Dr. Greg Tiao · Grand Rounds
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What the experts said26 expert statements
Hepatoblastoma is the most common malignant liver tumor in the pediatric population
Epidemiological
Treatment strategies for hepatoblastoma have changed dramatically over the past 25 years
Clinical
There are around 250 new cases of hepatoblastoma per year
Epidemiological
Hepatoblastoma is nowhere near the most common liver tumor or the most common abdominal tumor in children
EpidemiologicalGreg Tiao
Neuroblastoma is the most common solid malignancy in the pediatric population
Epidemiological
Hemangioma is the most common liver lesion in children
Epidemiological
Hepatoblastoma can present as an asymptomatic abdominal mass, tumor rupture, or respiratory distress due to tumor size
ClinicalGreg Tiao
Hepatoblastoma can present as a paraneoplastic syndrome like precocious puberty
Clinical
An elevated alpha fetoprotein (AFP) is a key component in the workup and is critical for the diagnostic algorithm and treatment response
Clinical
Most experts rely on MRI to delineate anatomy and evaluate the extent of liver involvement in hepatoblastoma
ClinicalGreg Tiao
Eovist is an MRI contrast agent taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging
ClinicalGreg Tiao
To establish a diagnosis of hepatoblastoma, four things are needed: history, abdominal imaging, AFP check, and liver biopsy
Clinical
The most important intervention for survival is to achieve complete resection
Clinical
Aggressive resections include trisectionectomy, mesohepatectomy, resection with additional procedure in remnant liver, and liver transplantation
ClinicalGreg Tiao
Couinaud labeled liver segments in a counterclockwise fashion by injecting portal vessels, similar to how districts of Paris are labeled
Clinical
The right hepatic vein differentiates anterior and posterior sections of the right liver
ClinicalGreg Tiao
The right portal vein separates superior and inferior segments of the right liver
ClinicalGreg Tiao
The pre-text staging system classifies tumors based on the number of contiguous liver sections free of disease (minus four equals pre-text stage)
ClinicalGreg Tiao
Pre-text 1 has three contiguous sections free of disease, pre-text 2 has two, pre-text 3 has one, and pre-text 4 has no free sections or diffuse tumor
ClinicalGreg Tiao
Pre-text 1 and 2 tumors with greater than 1 cm margin from middle hepatic vein and portal bifurcation should be considered for resection at diagnosis
Clinical
Pre-text 3 and 4 tumors represent a big game changer in practice that improved survival from less than 30% in the 1970s-80s to 80-90% today
ClinicalGreg Tiao
Pre-text 3 tumors should generally be biopsied at diagnosis, started on neoadjuvant chemotherapy, then referred to transplant center or center with expertise in advanced liver resections
ClinicalGreg Tiao
Indications for transplant in pre-text 3 or 4 disease include unresectable disease, unsafe resection, or inadequate liver remnant
Clinical
It is important to consult a transplant center early for pre-text 3 and 4 tumors
ClinicalGreg Tiao
The PHITT (Pediatric Hepatic International Tumor Trial) is an ongoing multinational trial to identify high and low risk features for each pre-text stage
ClinicalGreg Tiao
The PHITT study has been ongoing for three years
ClinicalGreg Tiao