Hepatoblastoma: Update Course 2014
PRETEXT staging stands for pre-treatment extent of disease and is based on segmental liver anatomy, performed prior to chemotherapy.
guideline2:02 ↗
PRETEXT staging has a tendency to overstage because it is based purely on imaging, and bulky tumors make it difficult to assess true vascular invasion versus mass effect.
clinical2:28 ↗
POSTTEXT staging refers to extent of disease after neoadjuvant chemotherapy has been given.
guideline2:51 ↗
Key staging annotations include involvement of the retrohepatic cava or hepatic veins, and the portal vein bifurcation.
guideline4:10 ↗
COG recommendations state that PRETEXT I-II tumors with clear 1 cm margins and no vascular involvement can be resected locally without referral to specialized centers.
guideline7:41 ↗
Most tumor volume shrinkage from chemotherapy occurs within the first two cycles.
clinical10:14 ↗
After 2 cycles of chemotherapy, if the tumor has not shrunk to a resectable size, the patient should be evaluated for transplant.
guideline10:33 ↗
As hepatoblastoma tumors shrink with chemotherapy, they do not shrink away from vascular supply significantly; size decreases but vascular margins do not substantially improve.
clinical11:06 ↗
Survival after extensive or 'heroic' resections (tumor liver explants with back table resections and reimplants, portal vein reconstructions, hepatic vein reconstructions) is not as good as transplant survival.
clinical12:10 ↗
There has been a move away from heroic resections in hepatoblastoma due to inferior survival compared to transplantation.
opinion12:40 ↗
Patients who undergo rescue transplant after failed resection do far worse than patients who have a planned transplant upfront.
clinical14:09 ↗
Current thinking is to refer patients to a transplant center early, even if two cycles of chemotherapy will be given first, to have pre-transplant evaluation completed and the patient integrated into the system.
guideline14:36 ↗
Primary transplant patients do surprisingly well despite immunosuppression for cancer, particularly in liver tumors.
clinical15:11 ↗
In the UK, all biliary atresia patients are referred upfront to a transplant center.
clinical16:02 ↗
For non-transplant center surgeons performing hepatoblastoma resection, there must be 95% confidence of successful resection with adequate margins before proceeding; otherwise referral to a transplant center is indicated.
opinion17:36 ↗
Anatomic resection with a good margin is appropriate for local management; disease near hepatic veins, crossing the liver, or involving the portal vein should be referred.
opinion18:11 ↗
Liver transplant surgeons have extensive experience operating on the liver, which may benefit pediatric surgeons managing borderline-resectable hepatoblastoma cases.
opinion18:55 ↗
Despite advanced imaging, the truth about resectability is determined at the time of operation.
clinical19:43 ↗
In Europe, all liver tumors receive chemotherapy upfront before surgery to make the tumor smaller and potentially avoid transplantation.
clinical19:58 ↗
Trisegmentectomy for cure is a reasonable approach to avoid lifelong immunosuppression from transplantation.
opinion20:19 ↗
Central hepatectomies have been performed successfully for hepatoblastoma cure without requiring transplant, though it is a difficult operation.
clinical20:34 ↗
For core needle biopsy of hepatoblastoma, approximately 10 passes are recommended, going through an area that includes normal parenchyma and tumor.
guideline21:25 ↗
There are two camps regarding pulmonary metastases in hepatoblastoma: one advocates resecting metastases upfront before hepatectomy; the other suggests waiting until after hepatectomy because liver regeneration growth factors may stimulate previously unrecognized lung sites.
clinical22:11 ↗
There is no good data on either side of the pulmonary metastases timing debate; all evidence is limited to a handful of patients.
clinical22:45 ↗
Papers examining stage 4 hepatoblastoma with pulmonary metastases are limited to cohorts of less than 20 patients, making it difficult to draw good conclusions.
epidemiological23:22 ↗
There may be reporting bias in hepatoblastoma transplant literature: successful cases (good chemo response, transplant, long-term survival) are reported, but failures may not be.
opinion23:38 ↗
PLUTO (Pediatric Liver Unresectable Tumor Observatory) is an international group seeking to answer questions about unresectable hepatoblastoma that cannot be answered at individual or multi-center level within North America or Europe.
clinical24:02 ↗
It will likely take several years before PLUTO can answer key questions about unresectable hepatoblastoma management.
opinion24:30 ↗
Clinical & Research Update: Pediatric Liver Tumors - A Case-Based Discussion with Drs. Katherine Somers & Alex Bondoc
Rib fractures or vertebral fractures are fairly common as a presentation factor with hepatic tumors in young children, particularly hepatoblastoma.
clinicalKatherine Somers14:51 ↗
Fractures in hepatoblastoma patients will heal as they go through their therapy, and kids tend to be rather unbothered by their fractures once cancer therapy is underway.
clinicalKatherine Somers15:29 ↗