Max Langham

261 statements · 3 topics

Liver Tumors (Hepatoblastoma/HCC) · guest expert Neuroblastoma · guest expert

Featured statements

If you have an upfront resection and the histology shows that it is a well differentiated pure fetal histology, that baby's done. There is no need for any chemotherapy at all, and survival in that instance is 100%.
quote · Hepatoblastoma
The disasters that have happened and that shouldn't really happen during liver resection are because of big time bleeding or because of air embolism.
quote · Hepatoblastoma
If it's a very low risk hepatoblastoma, probably it doesn't need a biopsy. It needs an upfront resection.
quote · Liver Tumors (Hepatoblastoma/HCC)
I would urge all multifocal tumors to be referred to transplantation.
quote · Liver Tumors (Hepatoblastoma/HCC)
In AHEP 0731 and the FIT trial, upfront resection is acceptable if it is a conventional hemihepatectomy or less, with clearly 1 centimeter of normal liver between the middle hepatic vein and tumor, and no annotation factors.
guideline · Neuroblastoma
For the biology arm of the Children's Oncology Group, they want 10 core biopsies.
guideline · Neuroblastoma

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Max's statements about Hepatoblastoma 87 statements

Open the Hepatoblastoma collection →

Hepatoblastoma

Statement Liver tumors are rare in children, and sorting out the diagnosis is crucial for good outcomes. Open
Statement A one-year-old with a liver mass is likely to be hepatoblastoma, although rhabdoid tumor can occur at that age and is a bad actor. Open
Statement If alpha-fetoprotein is quite high and very elevated, the presumptive diagnosis in a 1-year-old would be hepatoblastoma. Open
Statement Hepatoblastoma presents from the first year of life through perhaps 10 years of age, although there are a few that have been diagnosed older than that. Open
Statement If this is a rhabdoid tumor, chemotherapy is not important and the lesion needs to be resected. Open
Quotation If it's a very low risk hepatoblastoma, probably it doesn't need a biopsy. It needs an upfront resection. Open
Statement If it's a very low risk hepatoblastoma, it probably doesn't need a biopsy and needs an upfront resection. Open
Statement Most people favor needle biopsy of the liver rather than an open biopsy or a laparoscopic biopsy. Open
Statement The biopsy should go through the normal part of the liver that will be resected with the specimen, approaching from the side of the tumor to avoid seeding the contralateral lobe. Open
Statement Hepatoblastoma will seed and can create big issues for future resection and cure if the biopsy crosses the uninvolved lobe. Open
Quotation Hepatoblastoma will seed and it can create big issues for future resection and cure. Open
Statement There have been prenatal diagnoses of hepatoblastoma, not super rare but not very common. Open
Statement Rhabdoid tumor is more common in very young children compared to hepatoblastoma. Open
Statement Hepatocellular carcinoma can occur in young children, usually in association with an inborn error of metabolism, with tyrosinemia as the prototype. Open
Statement A baby with tyrosinemia will develop hepatocellular carcinoma if they are not transplanted, usually in the first couple of years of life. Open
Statement There is a mixed tumor with features of hepatoblastoma and hepatocellular carcinoma that tends to occur in children over 6 years of age. Open
Statement There is a predisposition of premature infants to getting hepatoblastomas, usually not in the nursery but in the first year or two of life. Open
Statement For the biology arm of the Children's Oncology Group, they want 10 core biopsies. Open
Statement The complication rate and some mortality seems higher with open biopsies than with the needle technique. Open
Statement There is no role for frozen section in hepatoblastoma, either for initial biopsy or for later margin assessment. Open
Statement The overarching goal of treatment for hepatoblastoma is to get the kids to a safe resection. Open
Quotation The overarching goal of treatment for hepatoblastoma is to get the kids to a safe resection. That's the most important thing. Open
Statement The PRETEXT system is a pre-treatment extent of disease radiographic imaging-based system developed in Europe during SIOPEL 1. Open
Statement Couinaud based the numerology of liver segments on the street map of Paris, with the caudate lobe (segment 1) representing the Île de Paris where Notre Dame is located. Open
Statement A PRETEXT 1 lesion has three contiguous sectors of the liver that are free of tumor. Open
Statement A PRETEXT 2 lesion has two adjoining sectors free of tumor. Open
Statement With PRETEXT 3, there is only one sector free of disease. Open
Statement In PRETEXT 4, all of the sectors are involved with no sectors of the liver free of disease. Open
Statement It is more common for people to over-read PRETEXT than under-read it. Open
Statement In AHEP 0731 and the FIT trial, upfront resection is acceptable if it is a conventional hemihepatectomy or less, with clearly 1 centimeter of normal liver between the middle hepatic vein and tumor, and no annotation factors. Open
Quotation If you have an upfront resection and the histology shows that it is a well differentiated pure fetal histology, that baby's done. There is no need for any chemotherapy at all, and survival in that instance is 100%. Open
Statement If a lesion is resected upfront and histology shows well-differentiated pure fetal histology, the baby is done with no need for any chemotherapy at all, and survival is 100%. Open
Statement Along with imaging for suspected hepatoblastoma, a chest CT is needed because pulmonary metastases make up the vast majority of metastases, and even small liver tumors can metastasize. Open
Statement If there is a PRETEXT 1 lesion with metastatic disease in the lungs, that is a high-risk patient. Open
Statement For a PRETEXT 1 lesion with clear lungs, no vena cava, hepatic vein confluence, or portal vein involvement, and judged resectable by the surgeon, it should be resected with an anatomic resection and the child will be in a very low risk group. Open
Statement A PRETEXT 1 lesion with vascular involvement (within 1 cm of cava or hepatic veins) would be considered an intermediate risk patient. Open
Statement If a patient with hepatoblastoma is over 8 years of age, that moves the patient into a higher risk environment. Open
Statement Not all PRETEXT 1 lesions are resectable upfront, and there are some PRETEXT 2 lesions that are resectable upfront. Open
Statement Annotation factors include V for vena cava and hepatic veins; if they are more than 1 centimeter away from the vena cava, there is no annotation factor. Open
Statement V0 means the lesion is within 1 centimeter of the cava or confluence of hepatic veins, making the patient no longer eligible for upfront resection. Open
Statement V1 means the tumor is abutting either the vena cava or the three hepatic veins. Open
Statement V2 means the tumor is large enough that it is distorting or pushing the vessels away. Open
Statement V3 means there is evidence of tumor thrombus extending out of the vein, which is particularly problematic. Open
Statement Hepatoblastoma is like Wilms tumor in that you can get direct extension of large tumor thrombus out of the hepatic veins into the vena cava and even up into the heart. Open
Statement Most hepatoblastomas are a mixture of fetal and embryonal histologies. Open
Statement The most common adverse finding is small cell undifferentiated (SCU) histology, which is a primitive tumor with small blue cell morphology and high nuclear-cytoplasmic ratio, making it a much higher risk lesion. Open
Statement In AHEP 0731, patients with mixed fetal and embryonal histology after upfront resection with negative margins were treated with two courses of cisplatin-based chemotherapy with excellent results. Open
Statement If the diagnosis is hepatoblastoma, the child needs long-term central access and will have two rounds of cisplatin-based chemotherapy in the United States, then be reimaged. Open
Statement Most of the tumor shrinkage that will occur with chemotherapy happens in the first two rounds. Open
Statement After two rounds of chemotherapy and reimaging, the surgeon should make a commitment about resectability; if resectable, do 2 more rounds of chemotherapy, resect after 4, then give 2 consolidation rounds for a total of 6. Open
Quotation I would urge all multifocal tumors to be referred to transplantation. Open
Statement Multifocal tumors should be referred to transplantation because they have high recurrence risk after resection. Open
Statement Most surgeons will be able to make the call about resectability at the end of two rounds of chemotherapy. Open
Quotation It's not a defeat. It's just like you just need to know what your system is capable of and again, safe resection. Got to emphasize patient safety. Open
Statement A child that is 6 years of age or older with hepatoblastoma is going to be a significantly higher risk patient. Open
Statement Children who are 8 years of age or older with hepatoblastoma are going to be high risk. Open
Statement Children with hepatoblastoma who have normal or near-normal alpha-fetoproteins (less than 100) are going to be high-risk patients. Open
Statement Pulmonary metastases in hepatoblastoma can have superb results, and current AHEP results look similar to Wilms tumor outcomes with pulmonary mets. Open
Statement Rapid responders whose pulmonary mets disappear with chemotherapy probably do not need any specific pulmonary therapy. Open
Statement A child where the pulmonary mets get smaller but persist will need a pulmonary metastectomy, which can be done thoracoscopically or open. Open
Statement The Japanese have published using indocyanine green as a marker for thoracoscopic metastectomy because it is concentrated in the liver and lights up. Open
Statement If pulmonary mets don't disappear, they should be biopsied to confirm metastatic disease and rule out other lesions like histoplasmosis in the Mississippi Valley. Open
Statement Before doing anything of consequence with the liver, ensure you can vascularly isolate it by dissecting the suprahepatic and infrahepatic vena cava and placing umbilical tapes for potential clamping. Open
Statement Disasters during liver resection happen because of major bleeding or air embolism from holes in the low-pressure vena cava system. Open
Quotation The disasters that have happened and that shouldn't really happen during liver resection are because of big time bleeding or because of air embolism. Open
Statement Use hypovolemic anesthesia with low CVP during liver resection, preserving transfusions until later in the case, because high CVP causes the liver to swell, become turgid, bleed more, and makes veins harder to dissect. Open
Statement Take inflow before outflow during liver resection to prevent the liver from swelling. Open
Statement Anatomic resections are preferred; non-anatomic resections are generally not favored except for lesions hanging off the edge of segments 3 or 5. Open
Statement The handheld harmonic device used for thyroidectomies is fast with excellent hemostasis for parenchymal transection. Open
Statement The CUSA is good for dissecting around veins and clearing anatomy but provides no hemostasis. Open
Statement Staplers work well in older kids and adults but in babies the stapler width can be wider than the margin, potentially causing false positive margins or getting into vessels. Open
Statement Liver transplant has great 3- and 5-year disease-free survival but requires lifelong immunosuppression with risks of rejection, secondary malignancies, and post-transplant lymphoproliferative disorders. Open
Statement Extreme resections with vena cava reconstruction or hepatic vein reimplantation can provide equivalent oncologic results to transplant without immunosuppression. Open
Statement Several patients have developed portal hypertension or other problems late after extreme resections that required transplant or other therapy. Open
Statement The complication rate of extreme resections is higher than transplantation. Open
Statement Having a grossly positive margin or leaving gross residual disease behind is not acceptable and should not happen if surgical guidelines are followed. Open
Statement Gross residual disease most often happens in older kids with trauma who rupture a liver tumor, such as during peewee football. Open
Statement Kids relapse either locally in the liver or in the lungs. Open
Statement If a patient has a negative margin resection and relapses locally in the liver, refer them for transplantation; salvage transplantation is not optimal but probably better than re-resection. Open
Statement For pulmonary recurrence, treating with chemotherapy is important but you will probably need to re-excise the pulmonary mets. Open
Statement Hepatoblastoma is not thought to be radiation sensitive, and there is almost no anecdotal data of radiation being used in the lungs. Open
Statement After resection following 4 rounds of chemotherapy with clear margins and no residual tumor, patients usually receive 2 courses of consolidation chemotherapy. Open
Statement Most recurrences will happen within the first 3 years of therapy. Open
Statement After a child gets two cycles of chemotherapy and is restaged, whether it is PRETEXT 2, 3, or has annotation factors, the surgeon's job is to resect that patient after 4 courses of chemotherapy. Open
Statement In a recent SIOPEL study, nearly half of PRETEXT 4 lesions were resected with conventional resections and did not need liver transplants. Open
Statement In the US for PRETEXT 4 in AHEP 0731, about 90% of those patients were transplanted. Open
Statement A patient being PRETEXT 3 or 4 does not mean they cannot be resected ultimately; it just means they need chemotherapy first. Open
Max's statements about Liver Tumors (Hepatoblastoma/HCC) 87 statements

Open the Liver Tumors (Hepatoblastoma/HCC) collection →

Hepatoblastoma

Statement Liver tumors are rare in children, and sorting out the diagnosis is crucial for good outcomes. Open
Statement A one-year-old with a liver mass is likely to be hepatoblastoma, although rhabdoid tumor can occur at that age and is a bad actor. Open
Statement If alpha-fetoprotein is quite high and very elevated, the presumptive diagnosis in a 1-year-old would be hepatoblastoma. Open
Statement Hepatoblastoma presents from the first year of life through perhaps 10 years of age, although there are a few that have been diagnosed older than that. Open
Statement If this is a rhabdoid tumor, chemotherapy is not important and the lesion needs to be resected. Open
Statement If it's a very low risk hepatoblastoma, it probably doesn't need a biopsy and needs an upfront resection. Open
Quotation If it's a very low risk hepatoblastoma, probably it doesn't need a biopsy. It needs an upfront resection. Open
Statement Most people favor needle biopsy of the liver rather than an open biopsy or a laparoscopic biopsy. Open
Statement The biopsy should go through the normal part of the liver that will be resected with the specimen, approaching from the side of the tumor to avoid seeding the contralateral lobe. Open
Statement Hepatoblastoma will seed and can create big issues for future resection and cure if the biopsy crosses the uninvolved lobe. Open
Quotation Hepatoblastoma will seed and it can create big issues for future resection and cure. Open
Statement There have been prenatal diagnoses of hepatoblastoma, not super rare but not very common. Open
Statement Rhabdoid tumor is more common in very young children compared to hepatoblastoma. Open
Statement Hepatocellular carcinoma can occur in young children, usually in association with an inborn error of metabolism, with tyrosinemia as the prototype. Open
Statement A baby with tyrosinemia will develop hepatocellular carcinoma if they are not transplanted, usually in the first couple of years of life. Open
Statement There is a mixed tumor with features of hepatoblastoma and hepatocellular carcinoma that tends to occur in children over 6 years of age. Open
Statement There is a predisposition of premature infants to getting hepatoblastomas, usually not in the nursery but in the first year or two of life. Open
Statement For the biology arm of the Children's Oncology Group, they want 10 core biopsies. Open
Statement The complication rate and some mortality seems higher with open biopsies than with the needle technique. Open
Statement There is no role for frozen section in hepatoblastoma, either for initial biopsy or for later margin assessment. Open
Quotation The overarching goal of treatment for hepatoblastoma is to get the kids to a safe resection. That's the most important thing. Open
Statement The overarching goal of treatment for hepatoblastoma is to get the kids to a safe resection. Open
Statement The PRETEXT system is a pre-treatment extent of disease radiographic imaging-based system developed in Europe during SIOPEL 1. Open
Statement Couinaud based the numerology of liver segments on the street map of Paris, with the caudate lobe (segment 1) representing the Île de Paris where Notre Dame is located. Open
Statement A PRETEXT 1 lesion has three contiguous sectors of the liver that are free of tumor. Open
Statement A PRETEXT 2 lesion has two adjoining sectors free of tumor. Open
Statement With PRETEXT 3, there is only one sector free of disease. Open
Statement In PRETEXT 4, all of the sectors are involved with no sectors of the liver free of disease. Open
Statement It is more common for people to over-read PRETEXT than under-read it. Open
Statement In AHEP 0731 and the FIT trial, upfront resection is acceptable if it is a conventional hemihepatectomy or less, with clearly 1 centimeter of normal liver between the middle hepatic vein and tumor, and no annotation factors. Open
Quotation If you have an upfront resection and the histology shows that it is a well differentiated pure fetal histology, that baby's done. There is no need for any chemotherapy at all, and survival in that instance is 100%. Open
Statement If a lesion is resected upfront and histology shows well-differentiated pure fetal histology, the baby is done with no need for any chemotherapy at all, and survival is 100%. Open
Statement Along with imaging for suspected hepatoblastoma, a chest CT is needed because pulmonary metastases make up the vast majority of metastases, and even small liver tumors can metastasize. Open
Statement If there is a PRETEXT 1 lesion with metastatic disease in the lungs, that is a high-risk patient. Open
Statement For a PRETEXT 1 lesion with clear lungs, no vena cava, hepatic vein confluence, or portal vein involvement, and judged resectable by the surgeon, it should be resected with an anatomic resection and the child will be in a very low risk group. Open
Statement A PRETEXT 1 lesion with vascular involvement (within 1 cm of cava or hepatic veins) would be considered an intermediate risk patient. Open
Statement If a patient with hepatoblastoma is over 8 years of age, that moves the patient into a higher risk environment. Open
Statement Not all PRETEXT 1 lesions are resectable upfront, and there are some PRETEXT 2 lesions that are resectable upfront. Open
Statement Annotation factors include V for vena cava and hepatic veins; if they are more than 1 centimeter away from the vena cava, there is no annotation factor. Open
Statement V0 means the lesion is within 1 centimeter of the cava or confluence of hepatic veins, making the patient no longer eligible for upfront resection. Open
Statement V1 means the tumor is abutting either the vena cava or the three hepatic veins. Open
Statement V2 means the tumor is large enough that it is distorting or pushing the vessels away. Open
Statement V3 means there is evidence of tumor thrombus extending out of the vein, which is particularly problematic. Open
Statement Hepatoblastoma is like Wilms tumor in that you can get direct extension of large tumor thrombus out of the hepatic veins into the vena cava and even up into the heart. Open
Statement Most hepatoblastomas are a mixture of fetal and embryonal histologies. Open
Statement The most common adverse finding is small cell undifferentiated (SCU) histology, which is a primitive tumor with small blue cell morphology and high nuclear-cytoplasmic ratio, making it a much higher risk lesion. Open
Statement In AHEP 0731, patients with mixed fetal and embryonal histology after upfront resection with negative margins were treated with two courses of cisplatin-based chemotherapy with excellent results. Open
Statement If the diagnosis is hepatoblastoma, the child needs long-term central access and will have two rounds of cisplatin-based chemotherapy in the United States, then be reimaged. Open
Statement Most of the tumor shrinkage that will occur with chemotherapy happens in the first two rounds. Open
Statement After two rounds of chemotherapy and reimaging, the surgeon should make a commitment about resectability; if resectable, do 2 more rounds of chemotherapy, resect after 4, then give 2 consolidation rounds for a total of 6. Open
Quotation I would urge all multifocal tumors to be referred to transplantation. Open
Statement Multifocal tumors should be referred to transplantation because they have high recurrence risk after resection. Open
Statement Most surgeons will be able to make the call about resectability at the end of two rounds of chemotherapy. Open
Quotation It's not a defeat. It's just like you just need to know what your system is capable of and again, safe resection. Got to emphasize patient safety. Open
Statement A child that is 6 years of age or older with hepatoblastoma is going to be a significantly higher risk patient. Open
Statement Children who are 8 years of age or older with hepatoblastoma are going to be high risk. Open
Statement Children with hepatoblastoma who have normal or near-normal alpha-fetoproteins (less than 100) are going to be high-risk patients. Open
Statement Pulmonary metastases in hepatoblastoma can have superb results, and current AHEP results look similar to Wilms tumor outcomes with pulmonary mets. Open
Statement Rapid responders whose pulmonary mets disappear with chemotherapy probably do not need any specific pulmonary therapy. Open
Statement A child where the pulmonary mets get smaller but persist will need a pulmonary metastectomy, which can be done thoracoscopically or open. Open
Statement The Japanese have published using indocyanine green as a marker for thoracoscopic metastectomy because it is concentrated in the liver and lights up. Open
Statement If pulmonary mets don't disappear, they should be biopsied to confirm metastatic disease and rule out other lesions like histoplasmosis in the Mississippi Valley. Open
Statement Before doing anything of consequence with the liver, ensure you can vascularly isolate it by dissecting the suprahepatic and infrahepatic vena cava and placing umbilical tapes for potential clamping. Open
Quotation The disasters that have happened and that shouldn't really happen during liver resection are because of big time bleeding or because of air embolism. Open
Statement Disasters during liver resection happen because of major bleeding or air embolism from holes in the low-pressure vena cava system. Open
Statement Use hypovolemic anesthesia with low CVP during liver resection, preserving transfusions until later in the case, because high CVP causes the liver to swell, become turgid, bleed more, and makes veins harder to dissect. Open
Statement Take inflow before outflow during liver resection to prevent the liver from swelling. Open
Statement Anatomic resections are preferred; non-anatomic resections are generally not favored except for lesions hanging off the edge of segments 3 or 5. Open
Statement The handheld harmonic device used for thyroidectomies is fast with excellent hemostasis for parenchymal transection. Open
Statement The CUSA is good for dissecting around veins and clearing anatomy but provides no hemostasis. Open
Statement Staplers work well in older kids and adults but in babies the stapler width can be wider than the margin, potentially causing false positive margins or getting into vessels. Open
Statement Liver transplant has great 3- and 5-year disease-free survival but requires lifelong immunosuppression with risks of rejection, secondary malignancies, and post-transplant lymphoproliferative disorders. Open
Statement Extreme resections with vena cava reconstruction or hepatic vein reimplantation can provide equivalent oncologic results to transplant without immunosuppression. Open
Statement Several patients have developed portal hypertension or other problems late after extreme resections that required transplant or other therapy. Open
Statement The complication rate of extreme resections is higher than transplantation. Open
Statement Having a grossly positive margin or leaving gross residual disease behind is not acceptable and should not happen if surgical guidelines are followed. Open
Statement Gross residual disease most often happens in older kids with trauma who rupture a liver tumor, such as during peewee football. Open
Statement Kids relapse either locally in the liver or in the lungs. Open
Statement If a patient has a negative margin resection and relapses locally in the liver, refer them for transplantation; salvage transplantation is not optimal but probably better than re-resection. Open
Statement For pulmonary recurrence, treating with chemotherapy is important but you will probably need to re-excise the pulmonary mets. Open
Statement Hepatoblastoma is not thought to be radiation sensitive, and there is almost no anecdotal data of radiation being used in the lungs. Open
Statement After resection following 4 rounds of chemotherapy with clear margins and no residual tumor, patients usually receive 2 courses of consolidation chemotherapy. Open
Statement Most recurrences will happen within the first 3 years of therapy. Open
Statement After a child gets two cycles of chemotherapy and is restaged, whether it is PRETEXT 2, 3, or has annotation factors, the surgeon's job is to resect that patient after 4 courses of chemotherapy. Open
Statement In a recent SIOPEL study, nearly half of PRETEXT 4 lesions were resected with conventional resections and did not need liver transplants. Open
Statement In the US for PRETEXT 4 in AHEP 0731, about 90% of those patients were transplanted. Open
Statement A patient being PRETEXT 3 or 4 does not mean they cannot be resected ultimately; it just means they need chemotherapy first. Open
Max's statements about Neuroblastoma 87 statements

Open the Neuroblastoma collection →

Hepatoblastoma

Statement Liver tumors are rare in children, and sorting out the diagnosis is crucial for good outcomes. Open
Statement A one-year-old with a liver mass is likely to be hepatoblastoma, although rhabdoid tumor can occur at that age and is a bad actor. Open
Statement If alpha-fetoprotein is quite high and very elevated, the presumptive diagnosis in a 1-year-old would be hepatoblastoma. Open
Statement Hepatoblastoma presents from the first year of life through perhaps 10 years of age, although there are a few that have been diagnosed older than that. Open
Statement If this is a rhabdoid tumor, chemotherapy is not important and the lesion needs to be resected. Open
Statement If it's a very low risk hepatoblastoma, it probably doesn't need a biopsy and needs an upfront resection. Open
Quotation If it's a very low risk hepatoblastoma, probably it doesn't need a biopsy. It needs an upfront resection. Open
Statement Most people favor needle biopsy of the liver rather than an open biopsy or a laparoscopic biopsy. Open
Statement The biopsy should go through the normal part of the liver that will be resected with the specimen, approaching from the side of the tumor to avoid seeding the contralateral lobe. Open
Quotation Hepatoblastoma will seed and it can create big issues for future resection and cure. Open
Statement Hepatoblastoma will seed and can create big issues for future resection and cure if the biopsy crosses the uninvolved lobe. Open
Statement There have been prenatal diagnoses of hepatoblastoma, not super rare but not very common. Open
Statement Rhabdoid tumor is more common in very young children compared to hepatoblastoma. Open
Statement Hepatocellular carcinoma can occur in young children, usually in association with an inborn error of metabolism, with tyrosinemia as the prototype. Open
Statement A baby with tyrosinemia will develop hepatocellular carcinoma if they are not transplanted, usually in the first couple of years of life. Open
Statement There is a mixed tumor with features of hepatoblastoma and hepatocellular carcinoma that tends to occur in children over 6 years of age. Open
Statement There is a predisposition of premature infants to getting hepatoblastomas, usually not in the nursery but in the first year or two of life. Open
Statement For the biology arm of the Children's Oncology Group, they want 10 core biopsies. Open
Statement The complication rate and some mortality seems higher with open biopsies than with the needle technique. Open
Statement There is no role for frozen section in hepatoblastoma, either for initial biopsy or for later margin assessment. Open
Statement The overarching goal of treatment for hepatoblastoma is to get the kids to a safe resection. Open
Quotation The overarching goal of treatment for hepatoblastoma is to get the kids to a safe resection. That's the most important thing. Open
Statement The PRETEXT system is a pre-treatment extent of disease radiographic imaging-based system developed in Europe during SIOPEL 1. Open
Statement Couinaud based the numerology of liver segments on the street map of Paris, with the caudate lobe (segment 1) representing the Île de Paris where Notre Dame is located. Open
Statement A PRETEXT 1 lesion has three contiguous sectors of the liver that are free of tumor. Open
Statement A PRETEXT 2 lesion has two adjoining sectors free of tumor. Open
Statement With PRETEXT 3, there is only one sector free of disease. Open
Statement In PRETEXT 4, all of the sectors are involved with no sectors of the liver free of disease. Open
Statement It is more common for people to over-read PRETEXT than under-read it. Open
Statement In AHEP 0731 and the FIT trial, upfront resection is acceptable if it is a conventional hemihepatectomy or less, with clearly 1 centimeter of normal liver between the middle hepatic vein and tumor, and no annotation factors. Open
Quotation If you have an upfront resection and the histology shows that it is a well differentiated pure fetal histology, that baby's done. There is no need for any chemotherapy at all, and survival in that instance is 100%. Open
Statement If a lesion is resected upfront and histology shows well-differentiated pure fetal histology, the baby is done with no need for any chemotherapy at all, and survival is 100%. Open
Statement Along with imaging for suspected hepatoblastoma, a chest CT is needed because pulmonary metastases make up the vast majority of metastases, and even small liver tumors can metastasize. Open
Statement If there is a PRETEXT 1 lesion with metastatic disease in the lungs, that is a high-risk patient. Open
Statement For a PRETEXT 1 lesion with clear lungs, no vena cava, hepatic vein confluence, or portal vein involvement, and judged resectable by the surgeon, it should be resected with an anatomic resection and the child will be in a very low risk group. Open
Statement A PRETEXT 1 lesion with vascular involvement (within 1 cm of cava or hepatic veins) would be considered an intermediate risk patient. Open
Statement If a patient with hepatoblastoma is over 8 years of age, that moves the patient into a higher risk environment. Open
Statement Not all PRETEXT 1 lesions are resectable upfront, and there are some PRETEXT 2 lesions that are resectable upfront. Open
Statement Annotation factors include V for vena cava and hepatic veins; if they are more than 1 centimeter away from the vena cava, there is no annotation factor. Open
Statement V0 means the lesion is within 1 centimeter of the cava or confluence of hepatic veins, making the patient no longer eligible for upfront resection. Open
Statement V1 means the tumor is abutting either the vena cava or the three hepatic veins. Open
Statement V2 means the tumor is large enough that it is distorting or pushing the vessels away. Open
Statement V3 means there is evidence of tumor thrombus extending out of the vein, which is particularly problematic. Open
Statement Hepatoblastoma is like Wilms tumor in that you can get direct extension of large tumor thrombus out of the hepatic veins into the vena cava and even up into the heart. Open
Statement Most hepatoblastomas are a mixture of fetal and embryonal histologies. Open
Statement The most common adverse finding is small cell undifferentiated (SCU) histology, which is a primitive tumor with small blue cell morphology and high nuclear-cytoplasmic ratio, making it a much higher risk lesion. Open
Statement In AHEP 0731, patients with mixed fetal and embryonal histology after upfront resection with negative margins were treated with two courses of cisplatin-based chemotherapy with excellent results. Open
Statement If the diagnosis is hepatoblastoma, the child needs long-term central access and will have two rounds of cisplatin-based chemotherapy in the United States, then be reimaged. Open
Statement Most of the tumor shrinkage that will occur with chemotherapy happens in the first two rounds. Open
Statement After two rounds of chemotherapy and reimaging, the surgeon should make a commitment about resectability; if resectable, do 2 more rounds of chemotherapy, resect after 4, then give 2 consolidation rounds for a total of 6. Open
Statement Multifocal tumors should be referred to transplantation because they have high recurrence risk after resection. Open
Quotation I would urge all multifocal tumors to be referred to transplantation. Open
Statement Most surgeons will be able to make the call about resectability at the end of two rounds of chemotherapy. Open
Quotation It's not a defeat. It's just like you just need to know what your system is capable of and again, safe resection. Got to emphasize patient safety. Open
Statement A child that is 6 years of age or older with hepatoblastoma is going to be a significantly higher risk patient. Open
Statement Children who are 8 years of age or older with hepatoblastoma are going to be high risk. Open
Statement Children with hepatoblastoma who have normal or near-normal alpha-fetoproteins (less than 100) are going to be high-risk patients. Open
Statement Pulmonary metastases in hepatoblastoma can have superb results, and current AHEP results look similar to Wilms tumor outcomes with pulmonary mets. Open
Statement Rapid responders whose pulmonary mets disappear with chemotherapy probably do not need any specific pulmonary therapy. Open
Statement A child where the pulmonary mets get smaller but persist will need a pulmonary metastectomy, which can be done thoracoscopically or open. Open
Statement The Japanese have published using indocyanine green as a marker for thoracoscopic metastectomy because it is concentrated in the liver and lights up. Open
Statement If pulmonary mets don't disappear, they should be biopsied to confirm metastatic disease and rule out other lesions like histoplasmosis in the Mississippi Valley. Open
Statement Before doing anything of consequence with the liver, ensure you can vascularly isolate it by dissecting the suprahepatic and infrahepatic vena cava and placing umbilical tapes for potential clamping. Open
Statement Disasters during liver resection happen because of major bleeding or air embolism from holes in the low-pressure vena cava system. Open
Quotation The disasters that have happened and that shouldn't really happen during liver resection are because of big time bleeding or because of air embolism. Open
Statement Use hypovolemic anesthesia with low CVP during liver resection, preserving transfusions until later in the case, because high CVP causes the liver to swell, become turgid, bleed more, and makes veins harder to dissect. Open
Statement Take inflow before outflow during liver resection to prevent the liver from swelling. Open
Statement Anatomic resections are preferred; non-anatomic resections are generally not favored except for lesions hanging off the edge of segments 3 or 5. Open
Statement The handheld harmonic device used for thyroidectomies is fast with excellent hemostasis for parenchymal transection. Open
Statement The CUSA is good for dissecting around veins and clearing anatomy but provides no hemostasis. Open
Statement Staplers work well in older kids and adults but in babies the stapler width can be wider than the margin, potentially causing false positive margins or getting into vessels. Open
Statement Liver transplant has great 3- and 5-year disease-free survival but requires lifelong immunosuppression with risks of rejection, secondary malignancies, and post-transplant lymphoproliferative disorders. Open
Statement Extreme resections with vena cava reconstruction or hepatic vein reimplantation can provide equivalent oncologic results to transplant without immunosuppression. Open
Statement Several patients have developed portal hypertension or other problems late after extreme resections that required transplant or other therapy. Open
Statement The complication rate of extreme resections is higher than transplantation. Open
Statement Having a grossly positive margin or leaving gross residual disease behind is not acceptable and should not happen if surgical guidelines are followed. Open
Statement Gross residual disease most often happens in older kids with trauma who rupture a liver tumor, such as during peewee football. Open
Statement Kids relapse either locally in the liver or in the lungs. Open
Statement If a patient has a negative margin resection and relapses locally in the liver, refer them for transplantation; salvage transplantation is not optimal but probably better than re-resection. Open
Statement For pulmonary recurrence, treating with chemotherapy is important but you will probably need to re-excise the pulmonary mets. Open
Statement Hepatoblastoma is not thought to be radiation sensitive, and there is almost no anecdotal data of radiation being used in the lungs. Open
Statement After resection following 4 rounds of chemotherapy with clear margins and no residual tumor, patients usually receive 2 courses of consolidation chemotherapy. Open
Statement Most recurrences will happen within the first 3 years of therapy. Open
Statement After a child gets two cycles of chemotherapy and is restaged, whether it is PRETEXT 2, 3, or has annotation factors, the surgeon's job is to resect that patient after 4 courses of chemotherapy. Open
Statement In a recent SIOPEL study, nearly half of PRETEXT 4 lesions were resected with conventional resections and did not need liver transplants. Open
Statement In the US for PRETEXT 4 in AHEP 0731, about 90% of those patients were transplanted. Open
Statement A patient being PRETEXT 3 or 4 does not mean they cannot be resected ultimately; it just means they need chemotherapy first. Open