Comments on and illustrations of the WFUMB CEUS liver guidelines: Rare malignant neuroendocrine and predominant epithelioid liver lesions.

The diagnosis or rare, non-hematologic malignant lesions of the liver may be a challenge owing to the rarity of the disease, and is usually made by histological confirmation. Ultrasound with color Doppler and contrast-enhanced, if required, taking into account the clinical background of the patient, may help to focus the differential diagnosis. In this review, we describe the pathological and ultrasound features of rare malignant neuroendocrine and predominantly epithelioid liver lesions including primary neuroendocrine tumor of the liver, Invasive mucinous cystic neoplasm of the liver, and also hepatoblastoma.

Review on Pediatric Malignant Focal Liver Lesions with Imaging Evaluation: Part I.

Malignant focal liver lesions (FLLs) are commonly reported in adults but rarely seen in the pediatric population. Due to the rarity, the understanding of these diseases is still very limited. In children, most malignant FLLs are congenital. It is very important to choose appropriate imaging examination concerning various factors. This paper will outline common pediatric malignant FLLs, including hepatoblastoma, hepatocellular carcinoma, and cholangiocarcinoma and discuss them against the background of the latest knowledge on comparable/similar tumors in adults. Medical imaging features are of vital importance for the non-invasive diagnosis and follow-up of treatment of FLLs in pediatric patients. The use of CEUS in pediatric patients for characterizing those FLLs that remain indeterminate on conventional B mode ultrasounds may be an effective option in the future and has great potential to be integrated into imaging algorithms without the risk of exposure to ionizing radiation.

Review on Pediatric Malignant Focal Liver Lesions with Imaging Evaluation: Part II.

Malignant focal liver lesions (FLLs) represent various kinds of epithelial and mesenchymal tumors. In pediatric patients, the understanding of pediatric liver diseases and associated imaging manifestations is essential for making accurate diagnosis and differential diagnosis. This paper will discuss the latest knowledge of the common pediatric malignant FLLs, including undifferentiated embryonal sarcoma, rhabdomyosarcoma, epithelioid hemangioendothelioma, angiosarcoma, and malignant rhabdoid tumor. Medical imaging features are not only helpful for clinical diagnosis, but can also be useful in the evaluation and follow-up of pre- and post-treatment. The future perspectives of contrast-enhanced ultrasound (CEUS) enhancement patterns of FLLs in pediatric patients are also mentioned.

Incidental Findings in Pediatric Patients: How to Manage Liver Incidentaloma in Pediatric Patients.

The World Federation for Ultrasound in Medicine and Biology (WFUMB) is addressing the issue of incidental findings (IFs) with a series of publications entitled "Incidental imaging findings-the role of ultrasound". IFs in the liver of newborns and children are rare and much less commonly encountered than in adults; as a result, they are relatively much more frequently malignant and life-threatening, even when they are of benign histology. Conventional B-mode ultrasound is the well-established first line imaging modality for the assessment of liver pathology in pediatric patients. US technological advances, resulting in image quality improvement, contrast-enhanced ultrasound (CEUS), liver elastography and quantification tools for steatosis have expanded the use of ultrasound technology in daily practice. The following overview is intended to illustrate incidentally detected liver pathology covering all pediatric ages. It aims to aid the examiner in establishing the final diagnosis. Management of incidentally detected focal liver lesions (FLL) needs to take into account the diagnostic accuracy of each imaging modality, the patient's safety issues (including ionizing radiation and nephrotoxic contrast agents), the delay in diagnosis, the psychological burden on the patient and the cost for the healthcare system. Moreover, this paper should help the pediatric clinician and ultrasound practitioner to decide which pathologies need no further investigation, which ones require interval imaging and which cases require further and immediate diagnostic procedures.

European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB): An Update on the Pediatric CEUS Registry on Behalf of the "EFSUMB Pediatric CEUS Registry Working Group".

The European Federation of Ultrasound in Medicine and Biology (EFSUMB) created the "EFSUMB Pediatric Registry" (EFSUMB EPR) with the purpose of collecting data regarding the intravenous application of pediatric contrast-enhanced ultrasound (CEUS). The primary aim was to document the current clinical practice and usefulness of the technique and secondarily to assess CEUS safety in children. We issue the preliminary results of this database and examine the overall practice of CEUS in children in Europe.

Orbital inflammation and colitis in pediatric IgG4-related disease: A case report and review of the literature.

IgG4-related disease (IgG4-RD) is an inflammatory disorder characterized by tumor-like swelling in one or more organs, elevated serum IgG4 levels, and histological alterations with infiltration of IgG4-positive plasma cells. IgG4-RD is rare and likely underdiagnosed in children. We report a case of a 16-year-old girl with IgG4-positive colitis that developed weeks after IgG4-related ophthalmic disease and discuss diagnosis and treatment in the context of the literature available. Since the pathophysiology of IgG4-RD is unknown, treatment options are empiric and, for the most part, untargeted. Systemic corticosteroid treatment is the basis of anti-inflammatory treatment in IgG4-RD and induced early remission in our patient. During corticosteroid taper, the patient developed weight loss and intestinal inflammation. Histopathological assessment of the intestinal walls confirmed IgG4-positive colitis. Immune-modulating treatment with non-biologic (e.g., methotrexate (MTX) and mycophenolate mofetil) or biologic (rituximab) disease-modifying antirheumatic drugs has been reported in treatment refractory or corticosteroid-dependent patients. The patient responded to treatment with anti-inflammatory therapy with food rich in TGF-ß2 (modulen) and MTX. This is one of the first pediatric patients reported with IgG4-related colitis extending the phenotype of pediatric IgG4-RD. International collaboration to prospectively document clinical presentation and treatment responses may help to further establish the phenotype and treatment options and to raise awareness for IgG4-RD.

General advice in ultrasound based elastography of pediatric patients.

Ultrasound elastography including transient elastography (TE), point shear wave elastography, (pSWE) and two (three)- dimensional shear wave elastography (2D-SWE) have been introduced mainly for the evaluation of the liver. All the techniques are also feasible for the examination of spleen, whereas pSWE and 2D-SWE can be used for the assessment of the pancreas, kidney, gastrointestinal tract and other organs. Strain elastography also plays a role for non-liver applications. The aim of the current report is to highlight unique features and techniques for the elastographic examinations in children and to report initial results in non-liver applications.