Children hepatic tumor

We describe a 2-month-old female infant with macroglossia, macrosomia, omphalocele, neonatal hypoglycemia, earlobe creases, low nasal bridge, midface retrusion, syndromic facies and multiple cutaneous and hepatic hemangiomas (HH). Genetic evaluation confirmed the diagnosis of Beckwith-Wiedemann Syndrome (BWS) with mosaic uniparental disomy 11 as the underlying genetic mechanism suggested by partial hypermethylation of H19/IGF2:IG-DMR and partial hypomethylation of KCNQ1OT1:TSS-DMR on chromosome 11p15.5. Pediatric endocrinology and cardiology assessments were normal...

BACKGROUND: Hepatocellular adenomas (HCAs) are rare benign tumors of the liver that occur predominantly in women taking oral contraceptives. In children, HCAs comprise < 5% of hepatic tumors. We report a case of HCAs in a 7-year-old girl with estrogen and glucose imbalance. CASE PRESENTATION: A 7-year-old girl was presented to our hospital with bilateral breast enlargement for 2 months, polydipsia, polyuria, polyphagia, hyperglycemia, and significant weight gain...

Mesenchymal hamartoma (MH) is a benign liver tumor accounting for 3% to 8% of all liver tumors in children, commonly manifesting before 3 years of life. Distinguishing MH from hepatoblastoma and other liver tumors relies on imaging and alpha-fetoprotein (which is usually within normal range in MH), before histologic examination. We report a case of a hepatic MH associated with elevated alpha-fetoprotein, leading to a misdiagnosis of hepatoblastoma and the administration of chemotherapy. We draw the attention to the diagnostic difficulty and pitfalls related to alpha-fetoprotein elevation in the setting of a liver tumor, and we highlight the importance of imaging and histology in establishing the diagnosis...

Infantile hemangiomas (IHs) are benign vascular tumors commonly observed in children. It is important to familiar with the characteristic features of hemangioma before diagnosis. Lesions located special position including periorbital and beard region, segmental hemangioma related PHACE syndrome and LUMBAR syndrome, hepatic hemangioma and related possible risks should be recognized. Early evaluation and assessment of risk grades should be done as early as possible before proliferation phase, so as to choosing the optimal treatment opportunity and scheme...

Birth weight is an established risk factor for some pediatric cancers but is dependent on gestational age and sex. Furthermore, it is unclear how associations may differ by infant sex, age at diagnosis, maternal race/ethnicity and maternal nativity status. We examined the association between size for gestation and a spectrum of pediatric cancers registered in the Texas Cancer Registry from 1995 to 2011. We analyzed up to 7547 cases and 37 735 controls. Analyses were conducted using logistic regression...

An abdominal inflammatory myofibroblastic tumor (AIMT), is a rare benign tumor composed of inflammatory and other mesenchymal cells. It can affect the entire body, predominantly in children and young adults. The diagnosis is challenging considering the wide clinical presentation and can often be mistaken for malignant tumors. We report a rare case of a 46-year-old female patient, who presented with intermittent abdominal pain weight loss, and an abdominal palpable mass. Abdominal ultrasound found a well-defined 18 cm, rounded mass, with solid and cystic components...

BACKGROUND: Primary hepatic Burkitt lymphoma (PHBL) in children is an extremely rare hepatic malignancy with a dismal prognosis, unless it is detected and treated promptly. An 11-year-old child with abdominal pain was admitted to our hospital. No notable abnormalities were found during his physical examination or laboratory workup, but the abdominal computed tomography and magnetic resonance imaging both indicated a malignant hepatic mass measuring 9.2 × 7.1 × 7...

PURPOSE: A pediatric normal tissue effects in the clinic (PENTEC) comprehensive review of patients with childhood cancer who received radiation therapy (RT) to the liver was performed to develop models that may inform RT dose constraints for the liver and improve risk forecasting of toxicities. METHODS AND MATERIALS: A systematic literature search was performed to identify published data on hepatic toxicities in children. Treatment and outcome data were extracted and used to generate normal tissue complication probability (NTCP) models...

INTRODUCTION: Infantile hepatic hemangioma (IHH) is the most common benign liver tumor in children, and multifocal and diffuse tumors often become life-threatening, necessitating therapy. Propranolol is now considered the first choice of therapy with ample data in Caucasian children. We present a series of nine Indian children with multifocal (n = 5) and diffuse (n = 4) IHH treated with propranolol monotherapy. METHODS: This was a retrospective clinical data-based single-center study...

BACKGROUND: Cancer is a leading cause of death by disease among children and adolescents in the United States. This study updates cancer incidence rates and trends using the most recent and comprehensive US cancer registry data available. METHODS: We used data from US Cancer Statistics to evaluate counts, age-adjusted incidence rates, and trends among children and adolescents aged <20 years diagnosed with malignant tumors during 2003-2019. We calculated average annual percent change and annual percent change (APC) using joinpoint regression...

Human immunodeficiency virus (HIV) infection is associated with a myriad of musculoskeletal manifestations. Inflammatory arthritis has been described in association with HIV in both adults and children. Biologic disease-modifying anti-rheumatic drugs, particularly tumor necrosis factor inhibitors (TNFi), have been reported to manage inflammatory arthritis in adults with HIV when conventional therapy fails to control arthritis. In this report, we describe the management of arthritis and enthesitis in a 12-year-old adolescent male with HIV using the TNFi adalimumab...

BACKGROUND: We describe three cases involving three patients with PRETEXT III hepatoblastoma invading the hepatic hilum. After portal vein embolization, the patients underwent uncomplicated trisectionectomy. METHODS: Medical records between March 2016 and March 2021 were reviewed, and three patients were selected. A literature review of techniques for increasing future liver remnant in children diagnosed with hepatoblastoma was also conducted. RESULTS: All tumors involved the right lobe and hepatic hilum (PRETEXT III)...

Fibrolamellar carcinomas (FLCs), lethal tumors occurring in children to young adults, have genetic signatures implicating derivation from biliary tree stem cell (BTSC) subpopulations, co-hepato/pancreatic stem cells, involved in hepatic and pancreatic regeneration. FLCs and BTSCs express pluripotency genes, endodermal transcription factors, and stem cell surface, cytoplasmic and proliferation biomarkers. The FLC-PDX model, FLC-TD-2010, is driven ex vivo to express pancreatic acinar traits, hypothesized responsible for this model's propensity for enzymatic degradation of cultures...

OBJECTIVE: To evaluate the hepatic vasculature/tumor relations in hepatoblastoma patients with three-dimensional (3D) reformatted images after triple-phase multi-detector computed tomography (MDCT) and to compare these with the surgical findings to judge the accuracy of this investigation. MATERIALS AND METHODS: The study was carried out in hepatoblastoma patients after appropriate neo-adjuvant chemotherapy, prior to resection. Images were postprocessed at a dedicated workstation for multi-planar reformations, maximum intensity projection, curved planar reformations, and volume-rendered technique reconstructions...

BACKGROUND: Liver tumors are rare in children with histologic heterogeneity that makes diagnosis challenging. Systematic histopathological review, performed as part of collaborative therapeutic protocols, identified relevant histologic subtypes that are important to distinguish. The Children's Hepatic tumors International Collaboration (CHIC) was established to study pediatric liver tumors on a global scale and led to establishment of a provisional consensus classification for use in international clinical trials...

BACKGROUND: Malignant rhabdoid tumors (MRTs) are rare, aggressive tumors that mainly affect children and currently lack effective chemotherapeutic regimens. Liver MRTs are particularly challenging to manage due to the difficulty of performing one-stage liver resection, and preemptive liver transplantation is associated with high recurrence rates. However, the associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) technique offers a promising surgical approach for advanced-stage liver tumors where conventional liver resection is not feasible...

Tumor-induced osteomalacia (TIO), a rare paraneoplastic syndrome is seen in association with the overproduction of fibroblast growth factor-23 (FGF-23) by certain mesenchymal tumors in adults. In children, these phosphaturic mesenchymal tumors produce features of rickets similar to TIO. This condition is characterized by elevated blood levels of FGF-23, low phosphate, low or normal active vitamin D, and high alkaline phosphatase. Though the removal of the tumor is curative; in cases where surgical resection is not possible, medical treatment is successful with phosphate and active vitamin D in the improvement of symptoms...

A 20-year-old woman was admitted with abdominal pain and a cystic liver tumor. A hemorrhagic cyst was suspected. Contrast-enhanced computed tomography(CT)and magnetic resonance imaging(MRI)revealed a space-occupying solid mass in the right lobule. Positron emission tomography(PET)-CT revealed 18F-fluorodeoxyglucose uptake in the tumor. We performed a right hepatic lobectomy. Histopathological evaluation of the resected tumor revealed an undifferentiated embryonal sarcoma of the liver(UESL). The patient refused adjuvant chemotherapy but showed no recurrence 30 months postoperatively...

Hepatic malignancies account for 1-4% of all childhood solid tumours and Hepatoblastoma is the most common malignant liver tumour in children. Its extrahepatic origin is rare. Here we present the case of a three-year-old boy who came with a large non-tender mass in the right upper abdomen for six months. Ultrasound abdomen revealed a huge heterogenous mass anterior to the right kidney and inferior to the liver with internal vascularity and calcifications, mimicking a neuroblastoma. Tru-cut needle biopsy showed foetal-type hepatoblastoma...

Congenital portosystemic shunts (CPSS) are rare vascular anomalies that cause abnormal communications between the portal and systemic venous systems and may be incidentally detected on imaging or via abnormal laboratory parameters due to the lack of specificity in the condition's clinical presentation. Ultrasound (US) is a common tool for examining abdominal solid organs and vessels and is the initial imaging modality for diagnosing CPSS. Here we report the case of an 8-year-old Chinese boy with CPSS diagnosed using color Doppler US...