Clinical & Research Update: Pediatric Liver Tumors - A Case-Based Discussion with Drs. Katherine Somers & Alex Bondoc
With Dr. Dr. Alex Tobin & Dr. Dr. Alex Bondoc & Dr. Dr. Katherine Somers & Dr. Dr. Ranga (Ranganathan) · hosted by Dr. Dr. Sophia Schermerhorn · Live Event Content
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
MRI using a hepatobiliary contrast agent is the first-line imaging study for patients with a liver tumor, allowing better visualization of multifocal disease and ideal assessment of hepatic vasculature
Rib fractures or vertebral fractures are fairly common as a presentation factor with hepatic tumors in young children, particularly hepatoblastoma
Fractures will heal as children go through their therapy, and kids are remarkable in their resilience and tend to be rather unbothered by their fractures once cancer therapy is underway
Precocious puberty in a Tanner stage one patient is a sign that should prompt consideration of underlying malignancy on the differential
High-risk therapy for hepatoblastoma includes cisplatin and doxorubicin, with dexrazoxane cardiac protectant given institutionally with doxorubicin
Almost every young toddler with a large liver tumor requires aggressive nutritional support to get through intensive therapy and surgical procedures
Weight loss or sarcopenia during active cancer therapy is associated with poor outcomes across all pediatric cancer diagnoses
Tumor board presentation and multidisciplinary conference approach is associated with improved success and long-term outcomes for pediatric liver tumor patients
Maximum tumor shrinkage typically occurs after the first block or cycle of chemotherapy; subsequent blocks produce less shrinkage
ICG fluorescence is highly sensitive but not specific for tumor detection; it can help find multifocal disease and assess margins
In approximately 15% of lung resections, ICG fluorescence identifies lesions not visible on high-resolution axial imaging
The FIT trial (AHEP 1531) mandated liver biopsy for diagnosis before starting treatment
Post-chemotherapy tumor response is maximum in initial stages; once tumor is replaced by blood pools and fibrous tissue, size doesn't decrease much further
PRETEXT criteria define metastatic disease as more than 2 nodules greater than 3 millimeters in diameter or 1 nodule greater than 5 millimeters in diameter
Even when first-side chest metastases show no viable tumor, the other side is still cleared surgically because combined chemotherapy and surgical clearance is the best pathway to cure
Patients with hepatoblastoma are frequently born prematurely
End-stage renal disease early in life shows increased incidence of hepatoblastoma, not just autosomal recessive polycystic kidney disease
Cisplatin's primary toxicity is renal, and platinum excretion is fully dependent on a functioning renal system
Patients on peritoneal dialysis can receive platinum chemotherapy using advanced pharmacokinetic and pharmacodynamic modeling without typical mandated hyperhydration
For known predisposition patients, screening protocol starts with ultrasound and alpha-fetoprotein levels, accounting for different normal AFP ranges in first months of life
PRETEXT IV multifocal disease requires liver transplantation; patients should be referred early to transplant program
In the United States, children with hepatoblastoma automatically receive Status 1B categorization on deceased donor list, the second highest stratum for organ allocation
Conventional hepatoblastoma is genomically quiet with very low mutation burden and invariably has point mutation or small deletion in exon 3 of CTNNB1 gene
Hepatocellular neoplasm NOS shows more genomic instability with chromosomal gains and losses, characterized by CTNNB1 deletion, often large deletions or complete exon 3 skipping
Beta-catenin immunohistochemistry is frequently weak positive or negative in HCC-NOS, serving as a diagnostic clue along with pleomorphic appearance and macrotrabecular arrangement
HCC-NOS can be targeted with high-risk hepatoblastoma therapy to shrink tumors and make them amenable to surgery in most cases
Every child with new cancer diagnosis meets with genetic counseling oncology team for complete genetic testing including germline testing if something is identified
Radiomics can predict pure fetal histology hepatoblastoma with area under ROC curve of approximately 0.85
Pure fetal histology hepatoblastoma patients are cured with resection alone and don't need chemotherapy
Artificial intelligence can segment liver tumors very accurately at the level of an expert