Children hepatic tumor

BACKGROUND: Clearing all pulmonary metastases is essential for curing pediatric solid tumors. However, intraoperative localization of such pulmonary nodules can be challenging. Therefore, an intraoperative tool that localizes pulmonary metastases is needed to improve diagnostic and therapeutic resections. Indocyanine green (ICG) real-time fluorescence imaging is used for this purpose in adult solid tumors, but its utility in pediatric solid tumors has not been determined. METHODS: A single-center, open-label, nonrandomized, prospective clinical trial (NCT04084067) was conducted to assess the ability of ICG to localize pulmonary metastases of pediatric solid tumors...

Congenital portosystemic shunts (CPSS) are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation. These shunts allow intestinal blood to reach the systemic circulation directly, and if persistent or large, may lead to long-term complications. CPSS can have a variety of clinical presentations that depend on the substrate that is bypassing hepatic metabolism or the degree of hypoperfusion of the liver. Many intrahepatic shunts spontaneously close by 1 year of age, but extrahepatic and persistent intrahepatic shunts require intervention by a single session or staged closure with a multidisciplinary approach...

Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is rare in children, and localizing the source of EAS is often challenging. Here, we report EAS in an adolescent boy who presented with Cushingoid features and had endogenous ACTH-dependent hypercortisolism on hormonal evaluation. Abdominal ultrasound and CT revealed a hepatic lesion with characteristics suggestive of hemangioma, whereas the lesion was tracer non-avid on 68 Ga-DOTANOC positron emission tomography/CT. A regional sampling of ACTH was done to confirm the hepatic lesion as the source of EAS, and a definitive ACTH gradient was observed between the hepatic vein and the right internal jugular vein...

BACKGROUND: The pathophysiology of pediatric hepatic encephalopathy (HE) is not well understood. Various serum biomarkers associated with HE may provide insight into its pathology, but their use and interpretation in clinical practice for diagnosis and prognostication remain undetermined. We sought to investigate reported correlations of serum biomarkers with presence and degree of HE in children. METHODS: We conducted a systematic review of studies examining novel serum biomarkers and cytokines in association with HE that included children on PubMed, Embase, Lilacs, and Scopus...

BACKGROUND: Leiomyosarcoma is classified as a soft tissue sarcoma. In adults, leiomyosarcoma is the most common malignancy affecting the vascular system; however, vascular leiomyosarcoma in children is extremely rare as most pediatric soft tissue tumors are rhabdomyosarcomas. The survival rate is very low, and incomplete resection is a poor prognostic factor. There is also a high rate of distant recurrence, with the lungs and liver being the most common sites of metastasis. There is no established effective chemotherapy, and complete surgical resection is the only potentially curative treatment for leiomyosarcoma...

BACKGROUND: Hepatoblastoma is the most common primary malignancy of hepatic origin in children, with an estimated incidence of 0.5-1.5 per million children. Hepatoblastoma classically has an intraparenchymal location, and pedunculated hepatoblastoma is a relatively rare entity. Accurate diagnosis can be challenging due to its extrahepatic location and possibly its thin peduncle, which is not easily identified in imaging. CASE PRESENTATION: Here, we report a case of asymptomatic giant palpable hepatoblastoma in the LUQ of a four-month-old male infant, initially suspected of neuroblastoma based on abdominal ultrasound findings...

Adult survivors of childhood cancer have an increased incidence of hepatitis due to immunosuppression and frequent blood transfusions. The immunization of children with cancer is critical for hepatitis prevention, however, access to vaccination may be restricted during times of war, such as the Syrian armed conflict. The goal of this study was to assess the pretreatment serological status of hepatitis A, B, and C in 48 Syrian refugee children with cancer diagnosed in our center between 2014 and 2021. The control group consisted of 48 Turkish children with cancer who were age, sex, and disease-matched...

The incidence of pediatric liver tumors in general has been rising over the last years and so is the number of children undergoing liver transplantation for this indication. To contribute to the ongoing improvement of pre- and post-transplant care, we aim to describe outcome and risk factors in our patient cohort. We have compared characteristics and outcome for patients transplanted for hepatoblastoma to other liver malignancies in our center between 1983 and 2022 and analysed influential factors on tumor recurrence and mortality using nominal logistic regression analysis...

AIMS: Inflammatory myofibroblastic tumour (IMT) is an intermediate neoplasm and rarely occurs in the liver. The aim of this study was to analyse the clinicopathological and genetic features of the largest primary hepatic IMT. METHODS AND RESULTS: A total of 10 cases were identified (four males and six females aged 1-48 years, median = 35 years) from 2011 to 2021, which accounted for 2.5% of IMTs occurring in all organ systems. Histological findings revealed that myofibroblastic/fibroblastic cells with inflammatory infiltration and focal hypocellularity were observed in three children...

Small cell undifferentiated (SCU) histology and alpha-fetoprotein (AFP) levels below 100 ng/mL have been reported as poor prognostic factors in hepatoblastoma (HB); subsequent studies reported SMARCB1 mutations in some SCU HBs confirming the diagnosis of rhabdoid tumor. The Children's Hepatic tumors International Collaboration (CHIC) database was queried for patients with HB who had AFP levels less than 100 ng/mL at diagnosis or were historically diagnosed as SCU HBs. Seventy-three of 1605 patients in the CHIC database were originally identified as SCU HB, HB with SCU component, or HB with low AFP levels...

BACKGROUND: Undifferentiated embryonic sarcoma of the liver (UESL) is a rare primary liver sarcoma that mainly affects children. Since it was first reported in 1978, less than 100 cases of UESL in adults have been reported. Due to the lack of specificity of clinical symptoms and imaging findings, the misdiagnosis rate of UESL is very high, and the definite diagnosis is usually in the late stage. Paraneoplastic syndrome (PNS) is a clinical symptom caused by the production of hormones or hormone-like substances in some tumors...

The widespread use of acetaminophen (APAP) in children as an over-the-counter treatment can cause acute liver failure through accidental overdose or ingestion. Therefore, the current research sought to investigate the function of hemin in mitigating the acute hepatotoxic effect of APAP in rat offspring. Thirty-two rats were assigned into four groups: control, hemin, APAP, and hemin/APAP groups. Liver enzymes were measured in serum along with oxidative stress indicators, tumor necrosis factor-α (TNF-α), interleukin-1beta (IL-1β), total nitrites (NOx), and caspase 3 in liver...

Hepatoblastoma is the most common type of hepatic tumors occurring in children between 0 and 5 years. And the exact pathophysiology of the disease is still mysterious. Accumulating studies on LncRNA have shown its pivotal role in the development and progression of distinct human cancers. However, the role of LINC01023 in hepatoblastoma is unknown. The relative expression of LINC01023, miR-378a-5p, and Wnt3 on hepatoblastoma tissue and cell lines was determined by quantitative polymerase chain reaction (qRT-PCR)...

BACKGROUND: To identify prognostic factors for overall survival through the analysis of 132 patients with Wilms tumor followed at a single center, with emphasis on the inferior vena cava/right atrium extension. METHODS: Retrospective analysis of overall survival using logistic regression models and including age, sex, clinical features, associated syndromes, comorbidities, tumor size before chemotherapy, stage, presence of metastatic disease and its site, invasion of adjacent structures, inferior vena cava/right atrium extension, laterality, tumor histology, chemotherapy protocol, and radiotherapy as potential risk factors...

BACKGROUND: Surgical management of tumor thrombus extending to the major vascular system for children with hepatoblastoma is challenging and insufficiently discussed. METHODS: We conducted a retrospective review of hepatoblastoma with tumor thrombus extending to the major vascular system (inferior vena cava, 3 hepatic veins, and portal vein trunk) treated at our center between May 2010 and June 2021. We describe our preoperative assessment, surgical strategies, and outcomes...

BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) of the liver are rare neoplasms. These tumors are difficult to distinguish from other neoplasms by radiological examination, have uncertain evolution, and there is no consensus on the treatment of these lesions. Hilar tumors can involve the portal vein, hepatic artery, bile duct, and spread to the branches of the portal triad, causing obstructive symptoms, occlusive phlebitis, and portal hypertension. Thus, liver transplantation (LT) is an excellent alternative for locally advanced hilar tumors, since it ensures complete tumor resection with free margins...

OBJECTIVE: To analyze the incidence trend and establish a model to predict the prognosis of hepatic malignant tumors in children (CHMTs). METHODS: We analyzed the incidence data of CHMTs from 1975 to 2018 from the Surveillance, Epidemiology, and End Results (SEER) database, and evaluated the incidence trends based on different demographic and pathological features. We also analyzed clinicopathologic data from 2000 to 2018 from the SEER database. Univariate and multivariate Cox regression analyses were performed to explore prognostic factors related to overall survival (OS)...

Acquired diaphragmatic hernia (ADH) is a rare complication after liver surgery in adult and pediatric patients. In the literature, a few low case series have been reported. Its incidence is estimated to be between 0.74% and 2.9%. Main descriptions of ADH concern liver resection for tumors, living donor hepatic donation in adult patients, and partial liver graft transplant in children [1,2]. We encountered a rare case of ADH in the postoperative time of a liver transplant with thrombosis of hepatic artery due to median arcuate ligament syndrome (MALS)...

Mesenchymal hamartoma of liver (MHL) is a benign liver tumour that occurs mainly in children, especially those under 2 years old. The pathogenesis of this tumor is still unknown. It is believed that MHL is derived from the Ito cells and either a developmental malformation of primitive hepatic mesenchyme; result of toxic or ischemic insult; or a true neoplasm. It is amenable to treatment and has a good prognosis but presents with varied clinical, imaging and histological findings. However, it can be confused with other hepatic tumors and can mimic malignancy...

BACKGROUND: No previous research papers have reported a comparative survey of local radiologic diagnoses and central review in children with hepatoblastoma. OBJECTIVE: To evaluate the utility of central review of children with hepatoblastoma enrolled in a clinical trial. MATERIALS AND METHODS: The study included 91 children enrolled in a clinical trial conducted by the Japanese Study Group for Pediatric Liver Tumor. We compared the results of the initial pre-treatment extent of tumor (PRETEXT) disease staging performed at local sites with the results obtained on central review to determine the concurrence rates for tumor staging and additional criteria...