Search Our Scientific Publications & Authors

Am J Med Genet A 2019 Mar 13;179(3):455-466. Epub 2019 Jan 13.

Division of Medical Genetics, Department of Pediatrics, Saint Louis University School of Medicine and SSM Cardinal Glennon Children's Hospital, St. Louis, Missouri.

The purpose of this study was to determine whether trisomy 18 patients are at an increased risk of tumor development and require formal tumor screening recommendations. A literature search of trisomy 18 patients with reports of tumors or malignancies, and compilation of all previously reported as well as new unreported cases was performed. 67 patients with trisomy 18 were found to have documented malignancies. Read More

Eur J Cancer 2019 Jan 5;106:126-132. Epub 2018 Dec 5.

Department of Children and Adolescents Oncology, Gustave Roussy, Villejuif, Paris, France.

Background: To evaluate the impact of a microscopically positive resection margin (microPRM) on the outcome of hepatoblastoma patients pretreated with chemotherapy.

Methods: Local recurrence and survival rates of 431 children treated in the SIOPEL 2 and 3 trials were analysed comparing 58 patients with microPRM with 371 who had a complete resection (CR) and who were then stratified by risk category. The tumour was standard-risk in 312 patients and high-risk (PRETEXT IV and/or extrahepatic disease and/or α-fetoprotein [AFP]<100 ng/ml) in 117 patients. Read More

J Pathol Transl Med 2018 11 26. Epub 2018 Nov 26.

Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.

Patients with Beckwith-Wiedemann syndrome (BWS) are predisposed to developing embryonal tumors, with hepatoblastoma being the most common type. Our patient showed hemihypertrophy, macroglossia, and paternal uniparental disomy in chromosome 11 and was diagnosed with BWS. When the patient was nine months old, a 2. Read More

Pediatr Emerg Care 2018 Oct;34(10):749-750

From the Department of Emergency and Critical Care Medicine, Kobe Children's Hospital, Kobe City, Hyogo, Japan.

Ultrasonography can be useful in the evaluation of children with abdominal complaints and may be the initial imaging modality in patients presenting with a newly recognized abdominal mass. Hepatoblastoma is an uncommon cause of acute abdominal pain in children and can rarely metastasize to the heart through vascular invasion. We report a case in which a 4-year-old girl who presented to the emergency department with abdominal pain promptly underwent point-of-care ultrasound and was subsequently diagnosed with intracardiac invasion of an abdominal tumor. Read More

World J Gastroenterol 2018 Sep;24(35):3980-3999

Department of Pediatrics, King George Medical University, Uttar Pradesh 226003, India.

Pediatric hepatocellular carcinoma (HCC) is the second common malignant liver tumor in children after hepatoblastoma. It differs from the adult HCC in the etiological predisposition, biological behavior and lower frequency of cirrhosis. Perinatally acquired hepatitis-B virus, hepatorenal tyrosinemia, progressive familial intrahepatic cholestasis, glycogen storage disease, Alagille's syndrome and congenital portosystemic shunts are important predisposing factors. Read More

Clin Imaging 2018 Nov - Dec;52:273-279. Epub 2018 Aug 18.

Department of Radiology, Seattle Children's Hospital, Seattle, WA 98105, United States.

Hepatoblastomas are complex pediatric tumors with several pathological subtypes, some of which demonstrate differing imaging features and portend varying prognosis. The radiologist plays a major role not only in the pre-surgical evaluation and baseline staging of the tumor, but also in guiding management and evaluating prognosis based on the PRETEXT (pretreatment extent of tumor) classification. We discuss the pathology, imaging features, and baseline evaluation, with a focus on the role of the radiologist in the management of these tumors. Read More

Lancet Child Adolesc Health 2018 03 1;2(3):173-179. Epub 2018 Feb 1.

Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, MD, USA.

Background: Cancer stage at diagnosis is crucial for assessing global efforts to increase awareness of childhood cancer and improve outcomes. However, consistent information on childhood cancer stage is absent from population cancer registries worldwide. The Toronto Childhood Cancer Stage Guidelines, compiled through an international consensus process, were designed to provide a standard framework for collection of information on stage at diagnosis of childhood cancers. Read More

Oncol Lett 2018 Sep 4;16(3):3784-3790. Epub 2018 Jul 4.

Department of Ultrasonography, Shandong Provincial Qianfoshan Hospital, Shandong University, Jinan, Shandong 250014, P.R. China.

Angiogenesis is required for the growth of hepatoblastoma (HB). In the present study, an ultrasonic contrast agent, microbubbles (MB), was combined with an endoglin antibody, and then injected into nude mice with HB. This was conducted to detect specific binding to microvessels via non-linear harmonic imaging for tumor angiogenesis assessment. Read More

Pediatr Radiol 2018 08 4;48(9):1234-1244. Epub 2018 Aug 4.

Cardiovascular Imaging, Department of Pediatric Radiology, Texas Children's Hospital, 6701 Fannin St., Houston, TX, 77030, USA.

Magnetic resonance imaging (MRI) is commonly used to characterize focal liver masses in the pediatric population. MRI is the preferred modality because of its superior contrast resolution and utility for obtaining functional sequences such as diffusion-weighted imaging (DWI). MR exams performed with a hepatocyte-specific gadolinium-based contrast agent can characterize focal liver lesions, which helps in differentiating a common benign entity such as focal nodular hyperplasia from other liver pathology when the background liver is normal. Read More

Pediatr Radiol 2018 08 4;48(9):1324-1336. Epub 2018 Aug 4.

Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Ave., Boston, MA, 02115, USA.

Most pediatric malignancies require some form of cross-sectional imaging, either for staging or response assessment. The majority of these are solid tumors and this review addresses the role of MRI, as well as other cross-sectional and functional imaging techniques, for evaluating the most common pediatric solid tumors. The primary emphasis is on neuroblastoma, hepatoblastoma and Wilms tumor, three of the most common non-central-nervous-system (CNS) pediatric solid tumors encountered in young children. Read More

Radiol Case Rep 2018 Jun 10;13(3):724-727. Epub 2018 May 10.

Division of Pediatric Radiology, Department of Radiology, University of Virginia Health System, University Hospital, First Floor,1215 Lee St., Charlottesville, VA, 22908.

Pediatric hepatic rhabdoid tumors are rare tumors of the liver, with few cases reported in the literature. These aggressive tumors can be difficult to differentiate from hepatoblastomas on imaging alone, and surgical biopsy combined with special immunohistochemical stains can assist in differentiating these 2 tumor types. We present a case of hepatic rhabdoid tumor in a 7-month-old female infant, which was originally thought to be a hepatoblastoma; however, using BAF47 staining for INI-1 we were able to diagnose a rhabdoid tumor and affect the patient's medical oncologic therapy. Read More

J Laparoendosc Adv Surg Tech A 2018 Sep 15;28(9):1152-1155. Epub 2018 Jun 15.

1 Department of Pediatric Surgery, Keio University School of Medicine , Tokyo, Japan .

Background: The curability of hepatoblastoma (HB) largely depends on the achievement of radical surgical resection, even for metastatic tumors. However, the extension of the metastatic tumor when viewed through an endoscope with the conventional white-light mode is often unclear. Advancements in imaging technology utilizing indocyanine green (ICG) have facilitated precise resection of metastatic HBs, owing to the longer retention of ICG in such lesions than in other normal tissues. Read More

Chin Med J (Engl) 2018 Jun;131(12):1450-1456

Department of Pediatric Cardiology, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.

Background: Cardiotoxicity is one of the most serious chronic complications of anthracyclines therapy. Assessment of the left ventricular ejection fraction (LVEF) fails to detect subtle cardiac dysfunction of left ventricular (LV). This study aimed to detect and evaluate new parameters of subclinical anthracyclines' cardiotoxicity in children with solid tumor. Read More

Radiologe 2018 Jun;58(6):595-608

Sektion Pädiatrische Radiologie, Institut für Diagnostische und Interventionelle Radiologie, Universitätsklinikum Jena, Am Klinikum 1, 07747, Jena, Deutschland.

In comparison to adult patients, other specific entities of abdominal tumors occur in childhood and adolescence. These include malignant tumors originating from embryonic tissue, such as hepatoblastoma. Some of the abdominal tumors are already diagnosed prenatally or in neonates. Read More

Int J Clin Oncol 2018 May 9. Epub 2018 May 9.

Department of Oncology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN, 38105, USA.

Background: Neoadjuvant chemotherapy is given to children with unresectable hepatoblastoma to increase the rate and safety of curative complete surgical resection. Elevated levels of serum alpha-fetoprotein (sAFP) decline with tumor shrinkage. In this single-institution retrospective study, we determined early dynamic changes of sAFP levels and tumor volume in children during therapy for unresectable hepatoblastoma. Read More

J Pediatr Surg 2018 May 12;53(5):1042-1045. Epub 2018 Feb 12.

Division of Pediatric Surgery, The Michael E. DeBakey Department of Surgery, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, United States. Electronic address:

Purpose: The purpose of this study was to evaluate treatment and surgical outcomes of patients of Beckwith-Wiedemann Syndrome (BWS) treated at a tertiary children's hospital.

Methods: A retrospective review of infants evaluated at Texas Children's Hospital for BWS from August 2000 to December 2016 was performed. Data collected included demographic information, clinical presentation, genetic evaluation, fetal imaging, operative treatment, and outcomes. Read More

Clin Nucl Med 2018 May;43(5):e164-e165

Beckwith-Wiedemann syndrome (BWS) is a rare congenital overgrowth disorder variably characterized by macrosomia, macroglossia, congenital hypoglycemia, and hemihyperplasia. The BWS predisposes affected individuals to embryonal tumors during childhood. The BWS is caused by abnormal gene regulation in a particular region of chromosome 11. Read More

Mymensingh Med J 2018 Jan;27(1):6-12

Dr Mosammat Hoshneara, Junior Consultant, Department of Radiology & Imaging, Mymensingh Medical College Hospital (MMCH), Mymensingh, Bangladesh.

Ultrasonography is a very useful diagnostic tool to evaluate pediatric abdominal mass. This cross sectional study was conducted among clinically suspected patients having malignant abdominal mass attending in the department of Radiology & Imaging, Mymensingh Medical College Hospital, Mymensingh, Bangladesh from January 2008 to December 2009. Total 56 patients were included in this study. Read More

J Int Med Res 2018 Apr 13;46(4):1570-1578. Epub 2018 Feb 13.

Department of Paediatric Surgery, 159390 Guangzhou Women and Children's Medical Centre , Guangzhou Medical University, Guangzhou, Guangdong Province, China.

Objective To investigate the impact of using a three-dimensional (3D) printed liver model for patient education. Methods Children with hepatic tumours who were scheduled for hepatectomy were enrolled, and patient-specific 3D liver models were printed with photosensitive resin, based on computed tomography (CT) images. Before surgery, their parents received information regarding liver anatomy, physiology, tumour characteristics, planned surgery, and surgical risks using these CT images. Read More

Pediatr Radiol 2018 04 9;48(4):536-554. Epub 2018 Feb 9.

Division of General and Thoracic Surgery, Cincinnati Children's Hospital, Cincinnati, OH, USA.

Imaging is crucial in the assessment of children with a primary hepatic malignancy. Since its inception in 1992, the PRETEXT (PRE-Treatment EXTent of tumor) system has become the primary method of risk stratification for hepatoblastoma and pediatric hepatocellular carcinoma in numerous cooperative group trials across the world. The PRETEXT system is made of two components: the PRETEXT group and the annotation factors. Read More

Pediatr Radiol 2018 04 2;48(4):565-580. Epub 2018 Feb 2.

Department of Radiology, Lucille Packard Children's Hospital, Stanford University, Stanford, CA, USA.

Hepatoblastoma and hepatocellular carcinoma (HCC) are the most common pediatric liver malignancies, with hepatoblastoma occurring more commonly in younger children and HCC occurring more commonly in older children and adolescents. Although surgical resection (including transplant when necessary) and systemic chemotherapy have improved overall survival rate for hepatoblastoma to approximately 80% from 30%, a number of children with this tumor type are not eligible for operative treatment. In contradistinction, pediatric HCC continues to carry a dismal prognosis with an overall 5-year survival rate of 30%. Read More

F1000Res 2018 15;7:53. Epub 2018 Jan 15.

Department of Surgery and Urology for Children and Adolescents, Medical University of Gdansk, Ul. Nowe Ogrody 1-6, 80-803 Gdansk, Poland.

: Hepatoblastoma (HB) is the most common primary malignant liver neoplasm in children. Its increasing survival rate is related to the progress in modern imaging, surgical techniques, and new chemotherapy regimens. : One of the past achievements was the development of the pretreatment extension of disease (PRETEXT) system. Read More

Pediatr Radiol 2018 04 23;48(4):555-564. Epub 2018 Jan 23.

Department of Radiology and Imaging Sciences, Division of Interventional Radiology and Image-guided Medicine, Emory University School of Medicine, Atlanta, GA, USA.

Primary liver malignancies are rare in children. Hepatoblastoma and hepatocellular carcinoma (HCC) together represent the overwhelming majority of cases. Overall survival of hepatoblastoma approaches 80% with multimodal treatment approaches that include chemotherapy, surgery and transplantation. Read More

Chirurg 2018 Mar;89(3):205-211

Abteilung für Kinderchirurgie und Kinderurologie, Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Tübingen, Hoppe-Seyler-Straße 3, 72076, Tübingen, Deutschland.

The overall survival of children with solid tumors has shown a substantial increase in the past decades due to progress in all of the disciplines involved in the treatment. The poor prognosis for advanced stages of disease and the morbidity related to therapeutic procedures are still a challenge. Innovations in the surgical treatment of solid tumors can contribute to increase the survival rate of affected children and to decrease the treatment-related morbidity. Read More

World J Gastroenterol 2017 Dec;23(46):8248-8255

Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy.

Nested stromal-epithelial tumor (NSET) is a non-hepatocytic and non-biliary tumor of the liver consisting of nests of epithelial and spindled cells with associated myofibroblastic stroma and variable intra-lesional calcification and ossification, which represents a very rare and challenging disease. Most of the reported cases have been treated with surgery, obtaining a long survival outcome. Here, we report the case of a 31-year-old Caucasian man who underwent surgery at our institution for a large, lobulated, multinodular mass of the right hemi-liver. Read More

Khirurgiia (Mosk) 2017(12):70-74

Dmitry Rogachev National Research and Practical Center for Pediatric Hematology, Oncology and Immunology, Moscow, Russia.

Pediatr Radiol 2018 03 19;48(3):325-332. Epub 2017 Dec 19.

Department of Radiology, Division of Thoracoabdominal Imaging, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, 3333 Burnet Ave., Cincinnati, OH, 45229-3039, USA.

Background: Reticuloendothelial system MRI signal hypointensity is common in pediatric oncology patients with solid abdominal tumors.

Objective: To assess changes in liver, spleen and bone marrow T2-weighted MRI signal intensity over time and their relationship to blood transfusion history in children with solid abdominal tumors.

Materials And Methods: In this retrospective study we measured liver, spleen and bone marrow signal intensity on axial T2-weighted MR images obtained December 2009 through February 2016 in children with hepatoblastoma, neuroblastoma, ganglioneuroblastoma and Wilms tumor. Read More

Sci Rep 2017 Dec 19;7(1):17751. Epub 2017 Dec 19.

Divisions of Pediatric Surgery and Surgical Research, Michael E. DeBakey Department of Surgery, Texas Children's Surgical Oncology Program, Texas Children's Liver Tumor Program, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, TX, 77030, USA.

Currently, preclinical testing of therapies for hepatoblastoma (HB) is limited to subcutaneous and intrasplenic xenograft models that do not recapitulate the hepatic tumors seen in patients. We hypothesized that injection of HB cell lines into the livers of mice would result in liver tumors that resemble their clinical counterparts. HepG2 and Huh-6 HB cell lines were injected, and tumor growth was monitored with bioluminescence imaging (BLI) and magnetic resonance imaging (MRI). Read More

Pediatr Blood Cancer 2018 Apr 18;65(4). Epub 2017 Dec 18.

Division of Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.

Background: Conflicting reports on the frequency of germline adenomatous polyposis coli (APC) gene mutations in patients with hepatoblastoma (HB) have called into question the clinical value of APC mutation testing on apparently sporadic HB.

Methods: An Institutional Review Board approved retrospective review of clinical data collected from patients with HB who received APC testing at our institution was conducted. All HB patients seen at Cincinnati Children's Hospital Medical Center were eligible for testing. Read More

Pediatr Radiol 2018 04 8;48(4):581-585. Epub 2017 Dec 8.

Department of Radiology, Nemours Children's Health System, Nemours Children's Hospital, Orlando, FL, USA.

Background: Hepatoblastoma is the most common primary hepatic malignancy in children. We have recently noticed an increased incidence of unsuspected fractures in children with newly diagnosed hepatoblastoma. This association has been suggested in the past, but the incidence and pathophysiology remain uncertain. Read More

J Med Case Rep 2017 Nov 21;11(1):327. Epub 2017 Nov 21.

Department of General Surgery, Children's Hospital of Nanjing Medical University, Nanjing, 210008, China.

Background: Almost all hepatoblastomas are isolated to the liver. Hepatoblastoma arising from and limited to extrahepatic tissue is an extremely rare clinical entity.

Case Presentation: Here we present a case of a 7-year-old Chinese boy of Han ethnicity with hepatoblastoma originating from the hepatogastric ligament. Read More

Interact Cardiovasc Thorac Surg 2017 12;25(6):1010-1011

Department of Thoracic Surgery, Kyoto University, Kyoto, Japan.

Indocyanine green fluorescent image-guided surgery was developed to identify primary and metastatic nodules of various malignancies. However, currently, surgeons need to identify the fluorescent image on a monitor, which impedes surgical procedures. Herein, we developed a novel projection mapping device that can cast the real-time fluorescent image onto the surface of the targeted organ. Read More

J Clin Oncol 2017 Oct 11;35(30):3465-3473. Epub 2017 Sep 11.

Allison F. O'Neill and Christopher B. Weldon, Dana-Farber Cancer Institute, Boston Children's Hospital, and Harvard Medical School, Boston, MA; Alexander J. Towbin and Greg M. Tiao, Cincinnati Children's Hospital, Cincinnati, OH; Mark D. Krailo, University of Southern California Keck School of Medicine, Los Angeles; Caihong Xia and Yun Gao, Children's Oncology Group, Monrovia; Marcio Malogolowkin, University of California Davis Comprehensive Cancer Center, Sacramento, CA; M. Beth McCarville, Wayne L. Furman, and Carlos Rodriguez-Galindo, St Jude Children's Research Hospital; Max R. Langham Jr, Le Bonheur Children's Hospital and University of Tennessee, Memphis; Howard M. Katzenstein, Vanderbilt University School of Medicine, Monroe Carell Jr Children's Hospital, Nashville, TN; Rebecka L. Meyers, Primary Children's Hospital and University of Utah, Salt Lake City, UT; Eugene D. McGahren, University of Virginia Children's Hospital and University of Virginia, Charlottesville, VA; Stephen P. Dunn, duPont Hospital for Children and Thomas Jefferson University, Philadelphia; Sarangarajan Ranganathan, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, Pittsburgh, PA; and Milton J. Finegold, Baylor College of Medicine, Houston, TX.

Purpose To determine whether the pattern of lung nodules in children with metastatic hepatoblastoma (HB) correlates with outcome. Methods Thirty-two patients with metastatic HB were enrolled on Children's Oncology Group Protocol AHEP0731 and treated with vincristine and irinotecan (VI). Responders to VI received two additional cycles of VI intermixed with six cycles of cisplatin/fluorouracil/vincristine/doxorubicin (C5VD), and nonresponders received six cycles of C5VD alone. Read More

Clin Nucl Med 2017 Oct;42(10):e449-e451

From the Department of Nuclear Medicine, First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.

We report the case of a 4-year-old girl with a biochemical relapse (plasma α-fetoprotein of 57,987.6 μg/L) after hepatoblastoma and extrahepatic metastases removal and adjuvant chemotherapy. Abdominal ultrasound, CT, and MRI failed to determine the site of recurrence. Read More

Adv Exp Med Biol 2017 ;959:101-109

Beatrix Children's Hospital, Division of Metabolic Diseases, University of Groningen, University Medical Center of Groningen, HPC CA33, Antwoordnummer 333, 9700 VB, Groningen, The Netherlands.

Hereditary Tyrosinemia type I (HT1) is clinically mainly characterised by severe liver disease. Most patients present in their first months of life with liver failure, but others can present later with issues of compensated cirrhosis, renal tubulopathy or acute intermittent porphyria. If patients survive the acute phase with liver failure or if they present later with compensated cirrhosis, they often develop hepatocellular carcinoma early but also later in life. Read More

Transplant Direct 2017 Jun 25;3(6):e165. Epub 2017 May 25.

Centre for Research on Advanced Surgery and Transplants (CICAT), Universidad Icesi, Cali, Colombia.

Background: Hepatoblastoma is the most common primary malignant liver tumor in children and is usually diagnosed during the first 3 years of life. Overall survival has increased 50% due to chemotherapeutic schemes, expertise surgery centers, and liver transplantation.

Methods: A retrospective collection of data was performed from pediatric patients with diagnosis of hepatoblastoma. Read More

J Clin Gastroenterol 2018 Mar;52(3):262-267

Department of Radiology and Biomedical Imaging, Yale School of Medicine, Division of Interventional Radiology.

Background: Primary liver cancer, including Hepatoblastoma (HB) and hepatocellular carcinoma (HCC), in pediatric populations is often fatal. The outcomes are poor despite universal health care access in pediatric patients.

Aim: We investigated the sociodemographic factors affecting outcomes in pediatric patients with primary liver cancer. Read More

Sci Rep 2017 05 11;7(1):1721. Epub 2017 May 11.

Key Laboratory for the Physics & Chemistry of Nanodevices, and Department of Electronics, Peking University, Beijing, 100871, P. R. China.

To monitor the temperature distribution of a cell and its changes under varied conditions is currently a technical challenge. A variety of non-contact methods used for measuring cellular temperature have been developed, where changes of local temperature at cell-level and sub-cell-level are indirectly calculated through the changes in intensity, band-shape, bandwidth, lifetime or polarization anisotropy of the fluorescence spectra recorded from the nano-sized fluorescent materials pre-injected into the target cell. Unfortunately, the optical properties of the fluorescent nano-materials may be affected by complicated intracellular environment, leading to unexpected measurement errors and controversial arguments. Read More

Zhonghua Er Ke Za Zhi 2017 May;55(5):364-368

Department of Hematology/Oncology, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China.

To investigate the efficacy and the prognostic factors in pediatric hepatoblastoma according to the standard diagnostic and therapeutic regimen. Eighty-four consecutive patients were enrolled in this study between June 2000 and June 2015. Diagnosis and staging was decided by the multi-disciplinary team including oncologists, surgeons, pathologists and sub-specialized radiologists refering to protocol of Children's Oncology Group(COG) and International Society of Pediatric Oncology Liver Tumor Study Group (SIOPEL) in a case observational study. Read More

Asian Cardiovasc Thorac Ann 2017 May 29;25(4):300-303. Epub 2017 Apr 29.

1 Queensland Paediatric Cardiac Services, Lady Cilento Children's Hospital, Brisbane, Australia.

Hepatoblastoma is very uncommon in children, and intracardiac extension is rare. The SIOPEL-4 regime for metastatic hepatoblastoma has extended the surgical options with good results. We successfully treated a boy aged 2 years and 9 months with hepatoblastoma, using a multidisciplinary surgical strategy involving an extended left hepatectomy, left adrenalectomy, biopsy of the paraaortic and coeliac lymph nodes, and resection of the inferior vena caval-right atrial extension of the hepatoblastoma, under cardiopulmonary bypass and deep hypothermia. Read More

Clin Imaging 2017 Jul - Aug;44:33-37. Epub 2017 Apr 6.

Department of Radiology, Seattle Children's Hospital, University of Washington, Seattle, WA, United States. Electronic address:

Background: Hepatoblastoma is the most common hepatic malignancy occurring in the pediatric population. Intratumoral cellular behavior varies, and the small-cell undifferentiated histopathology carries a poorer prognosis than other tissue subtypes. Neoadjuvant chemotherapy is recommended for this tumor subtype prior to surgical resection in most cases. Read More

Pediatr Dev Pathol 2018 Jan-Feb;21(1):84-90. Epub 2017 Apr 6.

1 Department of Pathology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Fanconi-Bickel syndrome is a rare autosomal recessive disorder due to mutations in the facilitative glucose transporter 2 ( GLUT2 or SLC2A2) gene resulting in excessive glycogen storage predominantly in the liver and kidney. Previous case reports of this condition have described liver biopsies with glycogen storage and variable steatosis and/or fibrosis. Unlike in other types of glycogen storage disease, hepatocellular adenomas and carcinomas have not been described to date in this syndrome. Read More

Indian J Pediatr 2017 Jun 29;84(6):456-464. Epub 2017 Mar 29.

Department of Medical Oncology, Dr. B.R.A Institute-Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.

Dramatic advancement has been made in the management of children with hepatoblastoma (HB) over the past 3 decades owing to the improvement in diagnostic imaging, new chemotherapeutic agents, better surgical care and availability of liver transplantation. These advances are the end results of contributions from 4 major study groups across the globe including International Society of Pediatric Oncology - Liver Tumor Strategy Group (SIOPEL), Children's Oncology Group (COG), German Pediatric Hematology Oncology Group (GPOH) and Japanese Pediatric Liver Tumor Study Group (JPLT). The current manuscript is written with the objective of developing a consensus guideline for practitioners at a National level. Read More

J Obstet Gynaecol 2017 Jul 17;37(5):670-672. Epub 2017 Mar 17.

d Santa Maria del Carmine Hospital , Provincial Health Care Services, Institute of Pathology , Rovereto , Italy.

Foetal magnetic resonance imaging (MRI) plays an increasingly important role in the diagnosis of foetal abnormalities. Over the years, we have successfully applied bi-parametric MRI (bp-MRI) to the following obstetric conditions: (1) neurologic vascular diseases; (2) assessment of lung parenchyma maturation; (3) renal pathologies, such as polycystic kidney, suspected renal infarction, unilateral or bilateral renal agenesis; (4) placental pathologies, as twin-twin transfusion syndrome or placenta accreta; (5) benignant and malignant congenital tumours or cysts of the liver, such as haemangioendothelioma, hepatoblastoma or metastatic neuroblastoma, of the kidney (e.g. Read More

Radiol Bras 2017 Jan-Feb;50(1):68

Hospital de Clínicas da Universidade Federal do Paraná (UFPR), Curitiba, PR, Brazil.

Radiol Case Rep 2017 Mar 7;12(1):191-195. Epub 2016 Dec 7.

Department of Diagnostic Radiology, Beaumont Hospital, Royal Oak, MI.

Postmortem magnetic resonance imaging (MRI) is emerging as a valuable tool to accompany traditional autopsy and has potential for use in cases when traditional autopsy is not possible. This case report will review the use of postmortem MRI with limited tissue sampling to differentiate between metastatic neuroblastoma and hepatoblastoma which could not be clearly differentiated with prenatal ultrasound, prenatal MRI, or emergent postnatal ultrasound. The mother presented to our institution at 27 weeks gestation after an obstetric ultrasound at her obstetrician's office identified a large abdominal mass. Read More

Cancer 2017 Jun 17;123(12):2360-2367. Epub 2017 Feb 17.

Department of Pediatric Surgery, Primary Children's Medical Center, Salt Lake City, Utah.

Background: The identification of new therapies for high-risk (HR) hepatoblastoma is challenging. Children's Oncology Group study AHEP0731 included a HR stratum to explore the efficacy of novel agents. Herein, the authors report the response rate to the combination of vincristine (V) and irinotecan (I) and the outcome of patients with high-risk hepatoblastoma. Read More

J Korean Med Sci 2017 Feb;32(2):315-320

Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.

The diagnosis of hepatocellular carcinoma (HCC) is based on imaging studies particularly in high-risk patients without histologic confirmation. This study evaluated the prevalence and characteristics of false-positively diagnosed HCC in a liver resection cohort for HCC. A retrospective review was performed of 837 liver resection cases for clinically diagnosed HCC between 2005 and 2010 at our institute. Read More

Semin Pediatr Surg 2016 Oct 3;25(5):311-317. Epub 2016 Sep 3.

Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN.

Most children who succumb to solid malignancies do so because of the burden of metastatic disease or due to complications associated with the therapy administered to treat metastatic disease. Approximately one-quarter of children with solid tumors will present with metastatic disease, and an additional 20% ultimately develop metastatic disease, most commonly in the lung. The role of surgery in the treatment of metastatic solid tumors, given its disseminated nature, is not intuitive, yet there are circumstances in which surgical resection of metastatic disease can potentially be curative. Read More