Surgical and anesthetic management for hepatectomy in two pediatric patients with trisomy 18, pulmonary hypertension, and hepatoblastoma

Lucas, Donald J.; Rubinstein, Jeremy D.; Gosain, Ankush; Tiao, Greg; Head, Tim; Pratap, Jayant Nick; Williams, Regan F.; Helmig, Sara; Geller, James I.; Langham, Max R.; Trobaugh‐Lotrario, Angela D.

doi:10.1002/pbc.27678

Cited by 11 publications

(14 citation statements)

References 26 publications

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Section: Genetic Syndromessupporting

confidence: 74%

“…Trisomy 18, regarded as a fatal disease, affects approximately 1 per 6,000 newborns (30). The correlation between trisomy 18 and hepatoblastoma has been reported in several previous studies (21,(31)(32)(33). Tomlinson et al raised an interesting point that the hepatoblastoma cases with trisomy 18 almost were females, and this situation was contrary to hepatoblastoma with higher prevalence in males (34).…”

Section: Genetic Syndromesmentioning

confidence: 93%

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The Genetic Changes of Hepatoblastoma

Chen

Guan

et al. 2021

Front. Oncol.

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Hepatoblastoma is the most common malignant liver cancer in childhood. The etiology of hepatoblastoma remains obscure. Hepatoblastoma is closely related to genetic syndromes, hinting that hepatoblastoma is a genetic predisposition disease. However, no precise exposures or genetic events are reported to hepatoblastoma occurrence. During the past decade, significant advances have been made in the understanding of etiology leading to hepatoblastoma, and several important genetic events that appear to be important for the development and progression of this tumor have been identified. Advances in our understanding of the genetic changes that underlie hepatoblastoma may translate into better patient outcomes. Single nucleotide polymorphisms (SNPs) have been generally applied in the research of etiology’s exploration, disease treatment, and prognosis assessment. Here, we reviewed and discussed the molecular epidemiology, especially SNPs progresses in hepatoblastoma, to provide references for future studies and promote the study of hepatoblastoma’s etiology.

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Section: Genetic Syndromessupporting

confidence: 74%

Section: Genetic Syndromesmentioning

confidence: 93%

The Genetic Changes of Hepatoblastoma

Chen

Guan

et al. 2021

Front. Oncol.

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“…While more and more HBL patients with trisomy 18 have undergone anatomical hepatectomy, it has also been challenging. 7 In the present case, primary right hepatectomy could be a treatment option; however, neoadjuvant chemotherapy would usually be applied because the tumor of segment 5 was questionably expanding into segment 4 and was very near to the left branch of the portal vein. The patient had been suffering from spontaneous apnea and failure-to-thrive, so anatomical hepatectomy was considered somewhat invasive.…”

Section: Discussionmentioning

confidence: 80%

“…As patients with trisomy 18 with HBL come to be frequently reported, the number of successful treatment reports is increasing, partly because of the improvement in patient management. 6,7 In addition, increasing the number of trisomy 18 patients who survived longer has encouraged the physician to approach comorbidity positively. The fundamental treatments for HBL are complete surgical resection employing anatomical hepatectomy and adjuvant/ neoadjuvant chemotherapy based on the pretreatment risk stratification; 10 this is also a prerequisite to patients with trisomy 18.…”

Section: Discussionmentioning

confidence: 99%

“…6 Cardiotoxicity related to a chemotherapeutic agent, fluid imbalance resulting from hydration, and physical burden related to the congenital heart defect and surgery will make the attending physician hesitate to tackle the challenging problem. 6,7 Therefore, a palliative approach is often undertaken. 8 We, herein, report a case of HBL in a patient with trisomy 18 that was successfully treated by combining chemotherapy and surgical resection and discuss a key to successful treatment.…”

mentioning

confidence: 99%

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Successful Treatment for Hepatoblastoma in Trisomy 18: A Case Report

Shirane,

Yoshimi,

Masuko

et al. 2023

Journal of Pediatric Hematology/Oncology

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Children with trisomy 18 tend to develop hepatoblastoma. Since the introduction of appropriate management for organ malfunction, individuals with trisomy 18 have come to have a longer life expectancy. However, the predisposition to hepatoblastoma becomes a significant issue for the quality of a case. Here, we present a rare multifocal hepatoblastoma involving predominantly Couinaud segments 5 and 7 in a 10-month-old boy with trisomy 18. Though the first-line cisplatin monotherapy resulted in unsatisfactory tumor shrinkage, the second-line neoadjuvant chemotherapy administrating irinotecan and vincristine gave rise to significant tumor reduction in volume, leading to the completion of partial resection of the liver without the microscopic residual disease. The patient has been free from recurrence for 44 months. Because anatomical right hepatectomy can cause circulatory instability, including acute onset of pulmonary hypertension in trisomy 18 patients, physicians should balance treatment benefits and potential adverse effects. Our successful experience utilizing a combination of efficacious and less cardiotoxic neoadjuvant chemotherapy followed by the partial hepatectomy encourages physicians to treat a patient with trisomy 18 and tackle hepatoblastoma with a genetic background.

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The impact of congenital heart disease on treatment and survival of patients with hepatoblastoma: A single‐center experience

Espinoza,

Montgomery,

Maamari

et al. 2024

Pediatric Blood & Cancer

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BackgroundPatients with hepatoblastoma (HB) have a higher risk of congenital heart defects (CHD). There is limited literature on the management and outcomes of these patients. The purpose of this study was to identify demographics and outcomes of these patients in a single tertiary referral center.MethodsAn Institutional Review Board (IRB)‐approved retrospective chart review of patients with newly diagnosed HB from October 2004 to January 2021 was performed. CHD was defined as the presence of a septal defect, patent ductus arteriosus, pulmonary atresia, or bicuspid aortic valve. Chi‐square and t‐test were utilized for statistical analyses.ResultsOf the 151 patients diagnosed with HB during the study timeframe, 29 patients were found to have CHD. Five‐year overall survival (OS) for non‐CHD HB patients was 81.9% compared to 68.9% in the CHD cohort (p = .12). The 5‐year OS for patients without surgically intervened CHD was 63.6% compared to 70.5% for those with surgically repaired CHD (p = .88). Pre‐treatment extent of tumor IV was present more often in patients with HB and CHD who passed away (6/9, 66.7%) compared to those who survived (3/16,18.8%, p = .01).ConclusionsPatients with HB and CHD have similar survival compared to those without CHD. Our data support that patients with HB and CHD should be treated with curative intent including cardiac surgical intervention, medical oncology therapy, and oncological surgery for their HB.

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Surgical and anesthetic management for hepatectomy in two pediatric patients with trisomy 18, pulmonary hypertension, and hepatoblastoma

Cited by 11 publications

References 26 publications

The Genetic Changes of Hepatoblastoma

The Genetic Changes of Hepatoblastoma

Successful Treatment for Hepatoblastoma in Trisomy 18: A Case Report

The impact of congenital heart disease on treatment and survival of patients with hepatoblastoma: A single‐center experience

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