The impact of margins and re-resection in pediatric synovial sarcoma.

Introduction: Synovial sarcoma is one of the most common soft tissue sarcomas in children. Guidelines regarding the adequate extent of resection margins and the role of re-resection are lacking. We sought to evaluate the adequate resection margin and the role of re-resection in predicting outcomes in children with synovial sarcomas.

A subset of image-defined risk factors predict completeness of resection in children with high-risk neuroblastoma: An international multicenter study.

Background: Image-defined risk factors (IDRFs) were promulgated for predicting the feasibility and safety of complete primary tumor resection in children with neuroblastoma (NB). There is limited understanding of the impact of individual IDRFs on resectability of the primary tumor or patient outcomes. A multicenter database of patients with high-risk NB was interrogated to answer this question.

An indocyanine green-based liquid biopsy test for circulating tumor cells for pediatric liver cancer.

Background: Hepatoblastoma and HCC are the most common malignant hepatocellular tumors seen in children. The aim of this study was to develop a liquid biopsy test for circulating tumor cells (CTCs) for these tumors that would be less invasive and provide real-time information about tumor response to therapy.

Methods: For this test, we utilized indocyanine green (ICG), a far-red fluorescent dye used clinically to identify malignant liver cells during surgery.

Histopathologic and immunophenotypic characterization of patient-derived pediatric malignant hepatocellular tumor xenografts (PDXs).

Advances in targeted therapies for pediatric hepatocellular tumors have been limited due to a paucity of clinically relevant models. Establishment and validation of intrahepatic patient-derived xenograft (PDX) models would help bridging this gap. The aim of this study is to compare the histomorphologic and immunophenotypic fidelity of patient tumors and their corresponding intrahepatic PDX models.

A novel treatment strategy utilizing panobinostat for high-risk and treatment-refractory hepatoblastoma.

Background & Aims: Patients with metastatic, treatment-refractory, and relapsed hepatoblastoma (HB) have survival rates of less than 50% due to limited treatment options. To develop new therapeutic strategies for these patients, our laboratory has developed a preclinical testing pipeline. Given that histone deacetylase (HDAC) inhibition has been proposed for HB, we hypothesized that we could find an effective combination treatment strategy utilizing HDAC inhibition.

Quantitative ctDNA Detection in Hepatoblastoma: Implications for Precision Medicine.

Hepatoblastoma is characterized by driver mutations in , making it an attractive biomarker for a liquid biopsy approach utilizing circulating tumor DNA (ctDNA). This prospective observational study sought to ascertain the feasibility of ctDNA detection in patients with hepatoblastoma and explore its associations with established clinical indicators and biomarkers, including serum Alpha-fetoprotein (AFP). We obtained 38 plasma samples and 17 tumor samples from 20 patients with hepatoblastoma.

Navigating relapsed hepatoblastoma: Predictive factors and surgical treatment strategy.

Objective: Hepatoblastoma (HB) is the most common primary hepatic malignancy in childhood. Relapse occurs in more than 50% of high-risk patients with a high mortality due to ineffective salvage therapies. The purpose of this study is to identify risk factors for relapsed HB and predictors of survival in a single tertiary referral center.

Development of Iodinated Indocyanine Green Analogs as a Strategy for Targeted Therapy of Liver Cancer.

Liver cancer is one of the leading causes of cancer-related deaths, with a significant increase in incidence worldwide. Novel therapies are needed to address this unmet clinical need. Indocyanine green (ICG) is a broadly used fluorescence-guided surgery (FGS) agent for liver tumor resection and has significant potential for conversion to a targeted therapy.

An indocyanine green-based liquid biopsy test for circulating tumor cells for pediatric liver cancer.

Background And Aims: Hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the most common malignant hepatocellular tumors seen in children. The aim of this work was to develop a liquid biopsy test for circulating tumor cells (CTCs) for these tumors that would be less invasive and provide information about the real-time state of tumors in response to therapies.

Methods: For this test, we utilized indocyanine green (ICG), a far-red fluorescent dye that is used clinically to identify malignant liver cells in the body during surgery.

A population-based assessment of metastatic hepatoblastoma in Texas reveals ethnic disparities.

Background: Hepatoblastoma (HB) is the most common primary liver cancer in children with emerging evidence that incidence is increasing globally. While overall survival for low risk hepatoblastoma is >90%, children with metastatic disease have worse survival. As identifying factors associated with high-risk disease is critical for improving outcomes for these children, a need for a further understanding of the epidemiology of hepatoblastoma is warranted.

HepT1-derived murine models of high-risk hepatoblastoma display vascular invasion, metastasis, and circulating tumor cells.

Hepatoblastoma (HB) is the most common pediatric primary liver malignancy, and survival for high-risk disease approaches 50%. Mouse models of HB fail to recapitulate hallmarks of high-risk disease. The aim of this work was to generate murine models that show high-risk features including multifocal tumors, vascular invasion, metastasis, and circulating tumor cells (CTCs).

Variations in Nuss Procedure Operative Techniques and Complications: A Retrospective Review.

Introduction:  The Nuss procedure is the most common and preferred operative correction of pectus excavatum. Surgeon preference and patient factors can result in variations in Nuss procedure technique. We hypothesize that certain techniques are associated with increased risk of complications.

Intertwin differences in umbilical artery pulsatility index are associated with infant survival in twin-to-twin transfusion syndrome.

Objectives: To evaluate the association of intertwin differences in umbilical artery pulsatility index (DUAPI) and infant survival in twin-to-twin transfusion syndrome (TTTS).

Methods: Absolute DUAPI was calculated prior to laser surgery. Receiver-operating characteristics (ROC) curve analysis provided an intertwin DUAPI cutoff of 0.

Fetal endoscopic tracheal occlusion and pulmonary hypertension in moderate congenital diaphragmatic hernia.

Objective: To study the role of fetal endoscopic tracheal occlusion (FETO) on resolution of pulmonary hypertension (PH) in fetuses with isolated moderate left-sided diaphragmatic hernia (CDH).

Methods: This retrospective study included fetuses with CDH evaluated between February 2004 and July 2017. Using the tracheal occlusion to accelerate lung growth (TOTAL) trial definition, we classified fetuses into moderate left CDH if O/E-LHR (observed/expected-lung head ratio) was 25-34.

Intercostal cryoablation during Nuss procedure: A large volume single surgeon's experience and outcomes.

Background: Recent studies have shown intercostal cryoablation(IC) during the Nuss procedure decreases hospital length of stay(LOS) and opioid administration. However, few studies have also evaluated the risk of postoperative complications related to IC.

Methods: We performed a single center retrospective analysis of all patients who underwent Nuss procedure by one surgeon from 2/2016 to 2/2020, comparing intraoperative IC to other pain management modalities(non-IC).