Unresectable hepatoblastoma: current perspectives

Trobaugh‐Lotrario, Angela D.; Meyers, Rebecka L.; O’Neill, Allison F.; Feusner, James

doi:10.2147/hmer.s89997

Cited by 39 publications

(40 citation statements)

References 47 publications

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“…This, in turn, emphasizes the importance of surgical expertise, need for balance between the benefits and risks of more aggressive surgical resection, and the necessity of earlier consideration of LT in those predicted to have very close surgical margin. Indeed, the proportion of patients who were able to undergo definitive surgery upfront and throughout the course of treatment in our cohort compared less favorably with that of published experiences of major collaborative groups . Patients with posttreatment extent of disease (POST‐TEXT) III or IV should be considered candidates for extended hepatectomy .…”

Section: Discussionmentioning

confidence: 79%

“…LT in HB is indicated in patients where all sections of the liver are involved, especially by multifocal tumors, as well as in patients with major vascular involvement . Outcome of upfront LT has been shown to be far superior to LT at recurrence, and consultation with the transplant team early would allow timely planning in patients presenting with extensive disease who remains unresectable after neoadjuvant therapy .…”

Section: Discussionmentioning

confidence: 99%

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Treatment outcome and pattern of failure in hepatoblastoma treated with a consensus protocol in Hong Kong

Liu

Leung

et al. 2018

Pediatric Blood & Cancer

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Background and aim:We reviewed the results and pattern of failure of the consensus HB/HCC 1996 treatment protocol for pediatric hepatoblastoma (HB) in Hong Kong. The role of SIOPEL and Children's Hepatic tumors International Collaboration (CHIC) risk stratification was evaluated. Methods:Patients enrolled on the protocol from 1996 to 2014 were included. PRETEXT staging, SIOPEL, and CHIC risk groups were retrospectively assigned.Results: Sixty patients were enrolled with median age at diagnosis of 1.1 years and median followup time of 6.8 years. Alpha-fetoprotein (AFP) was raised (>100 ng/mL) in 58 (97%) patients.Five (8%) had metastases at presentation and 7 (12%) experienced tumor rupture prior to or during treatment. Twenty-nine patients (48%) received a first-line cisplatin, 5-fluorouracil, and vincristine regimen only while 23 (38%) also had alternative chemotherapeutic agents. Hepatic resection could be performed in 48 (80%) patients. Three (5%) patients underwent upfront liver transplantation. Five-year event-free survival and overall survival rates were 69.2% ± 6.1% and 77.6% ± 5.5% respectively. Among the 16 patients with relapse/progression, 9 had intrahepatic failure only, 5 had distant failure only, and 2 had combined local and distant failure. Predictors of inferior outcome included advanced Evans staging, disease involving both lobes, rupture, low AFP, and suboptimal response to first-line chemotherapy. Assigned in 44 patients, PRETEXT staging, SIOPEL, and CHIC risk groups significantly predicted EFS and OS. Conclusions:Although the consensus HB/HCC 1996 protocol led to cure in three-quarters of pediatric HB patients, an upfront risk stratification system is required to identify and improve the outcome of high-risk patients. K E Y W O R D Shepatoblastoma, pediatric, pretreatment extent of disease, relapse, risk stratification

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Section: Discussionmentioning

confidence: 79%

Section: Discussionmentioning

confidence: 99%

Treatment outcome and pattern of failure in hepatoblastoma treated with a consensus protocol in Hong Kong

Liu

Leung

et al. 2018

Pediatric Blood & Cancer

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“…[ 1 , 15 ] However, approximately 60% of the tumors cannot be completely removed because of their large size or extensive invasion. [ 16 , 17 ] In the current case, the patient underwent complete tumor resection. Liver transplantation can be considered for tumors that cannot be removed by conventional surgery.…”

Section: Discussionmentioning

confidence: 93%

Hepatoblastoma with pure fetal epithelial differentiation in a 10-year-old boy

2018

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Rationale:Hepatoblastoma is a rare malignant embryonal tumor that only accounts for approximately 1% of all pediatric cancers and mostly develops in children younger than 5 years old. Moreover, the occurrence of hepatoblastoma in adults is extremely rare.Patient concerns:Herein, we present a rare case of hepatoblastoma with pure epithelial differentiation in a 10-year-old boy.Pathological examination was performed. The tumor was 15 cm × 15 cm in size with clear margins. The cut surface was multiple nodular and grey-yellow. Histologically, the small cuboidal tumor cells were arranged in trabeculae with 2–3 cell layers. The tumor cells had eosinophilic or clear cytoplasm, formed dark and light areas, and were positive for alpha-fetoprotein, CK, CK8/18, CD10, hepatocyte, and GPC3. CD34 staining revealed that the sinusoids were lined by endothelial cells in the tumor tissues. The Ki67 index was approximately 20%.Diagnoses:Based on these findings, the case was diagnosed as hepatoblastoma with pure fetal epithelial differentiation.Interventions:The tumor was completely removed.Outcomes:No recurrence was found 3 months after the operation.Lessons:Hepatoblastoma with pure epithelial differentiation can also occur in older children. Children rarely notice and report any physical abnormality, and this may be among the primary reasons for the late diagnosis of the tumor. Annual heath checks may be beneficial in the detection of these rare tumors and improvement of patient outcomes.

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“…11 Although these results favored liver transplantation over aggressive liver resections, the treatment of unresectable hepatoblastoma remained challenging, due to transplant-related vascular and biliary complications, opportunistic infections exacerbated by the need for immunosuppression, relapse or secondary neoplasms. 12 In the past decades, multiple new studies have arisen in the search for risk factors for hepatoblastoma, like our own review. Some factors present at diagnosis, including PRE-TEXT, metastatic disease, and resectability, have become part of the classification and treatment protocols.…”

Section: Discussionmentioning

confidence: 99%