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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. The PRETEXT group describes the extent of tumor within the liver while the annotation factors help to describe associated features such as vascular involvement (either portal vein or hepatic vein/inferior vena cava), extrahepatic disease, multifocality, tumor rupture and metastatic disease (to both the lungs and lymph nodes). This manuscript is written by members of the Children's Oncology Group (COG) in North America, the International Childhood Liver Tumors Strategy Group (SIOPEL) in Europe, and the Japanese Study Group for Pediatric Liver Tumor (JPLT; now part of the Japan Children's Cancer Group) and represents an international consensus update to the 2005 PRETEXT definitions. These definitions will be used in the forthcoming Trial to Pediatric Hepatic International Tumor Trial (PHITT). [ABSTRACT FROM AUTHOR]

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. However, there remains a subset of children with hepatoblastoma in whom resection or transplantation is not possible. The 5-year survival for children diagnosed with HCC is less than 30% and remains a significant therapeutic challenge. The poor outcomes for children with primary liver tumors motivate investigation of new therapeutic alternatives. Interventional oncology offers a broad scope of percutaneous and transcatheter endovascular cancer therapies that might provide clinical benefits. Minimally invasive approaches are distinct from medical, surgical and radiation oncologic treatments, and in adults these approaches have been established as the fourth pillar of cancer care. Transarterial chemoembolization is a minimally invasive locoregional treatment option performed by interventional radiologists with level-I evidence as standard of care in adults with advanced liver malignancy; transarterial chemoembolization in adults has served to prolong disease-free progression, downstage and bridge patients for surgical and transplant interventions, and improve overall survival. However, while several groups have reported that transarterial chemoembolization is feasible in children, the published experience is limited primarily to small retrospective case series. The lack of prospective trial evidence has in part limited the utilization of transarterial chemoembolization in the pediatric patient population. The purpose of this article is to provide an overview of the role of interventional radiology in the diagnosis and endovascular management of hepatic malignancies in children. [ABSTRACT FROM AUTHOR]

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%. The Paediatric Hepatic International Tumour Trial (PHITT) is an international trial aimed at evaluating both existing and emerging oncologic therapies for primary pediatric liver tumors. Interventional radiology offers a number of minimally invasive procedures that aid in diagnosis and therapy of pediatric liver tumors. For diagnosis, the PHITT biopsy guidelines emphasize and recommend percutaneous image-guided tumor biopsy. Additionally, both percutaneous and endovascular procedures provide therapeutic alternatives that have been, to this point, only minimally utilized in the pediatric population. Specifically, percutaneous ablation offers a number of cytotoxic technologies that can potentially eradicate disease or downstage children with unresectable disease. Percutaneous portal vein embolization is an additional minimally invasive procedure that might be useful to induce remnant liver hypertrophy prior to extended liver resection in the setting of a primary liver tumor. PHITT offers an opportunity to collect data from children treated with these emerging therapeutic options across the world. The purpose of this manuscript is to describe the potential role of minimally invasive percutaneous transhepatic procedures, as well as review the existing data largely stemming from the adult HCC experience. [ABSTRACT FROM AUTHOR]

Objectives: Renovascular disease (RVD) is found in about 10 % of secondary childhood hypertension. Digital subtraction angiography (DSA) is the gold standard to diagnose RVD. Non-invasive imaging methods like Doppler ultrasound (US), magnetic resonance angiography (MRA), and computed tomography angiography (CTA) are increasingly used. Our aim was to evaluate the role and accuracy of US, MRA, and CTA compared to DSA in diagnosing RVD in children. Patients and methods: A retrospective review of 127 children with suspected renovascular hypertension was performed in children referred to Great Ormond Street Hospital between 2006 and 2014 due to clinical suspicion of renovascular hypertension and/or findings on US and/or MRA or CTA. Results: Ninety-nine of 127 children (78 %) were diagnosed with renovascular disease and 80 were treated with percutaneous transluminal angioplasty during the same procedure. The median age at presentation was 5.6 (range, 2.5-10.6) years. Thirty-six children had unilateral renal artery stenosis in major extraparenchymal vessels, 47 bilateral stenosis and 16 intrarenal small vessel disease. US had a sensitivity of 63 % and specificity of 95 %. MRA and CTA were performed in 39 and 34 children, respectively. CTA sensitivity was slightly higher than that of MRA, 88 vs. 80 %, and specificity 81 vs. 63 %. Conclusions: The sensitivity of MRA and CTA is still too low to reliably rule out renovascular disease in children with a strong suspicion of this diagnosis. DSA remains the gold standard to diagnose renovascular hypertension and is recommended when clinical and laboratory criteria are highly suggestive of renovascular disease even with normal radiological investigations from non-invasive methods. [ABSTRACT FROM AUTHOR]

Objective Polyarteritis nodosa (PAN) is a rare disease of childhood. The aims of this study were to describe the clinical features, treatment, and outcome of systemic childhood PAN and to identify predictors of relapse. Methods A single-center retrospective medical records review of children with PAN fulfilling the European League Against Rheumatism (EULAR)/Paediatric Rheumatology European Society (PRES)/Paediatric Rheumatology International Trials Organisation (PRINTO) classification criteria who were seen over a 32-year period was performed. Data on demographic and clinical features, treatments, relapses (recurrence of clinical signs/symptoms or occurrence of new symptoms after initial remission requiring escalation or resumption of immunosuppressive therapy), and deaths were recorded. A disease activity score was retrospectively assigned using the Paediatric Vasculitis Activity Score (PVAS) instrument. Cox regression analysis was used to identify significant predictors of relapse. Results Sixty-nine children with PAN were identified; 55% were male, and their median age was 8.5 years (range 0.9-15.8 years). Their clinical features at presentation were fever (87%), myalgia (83%), skin (88%), renal (19%), severe gastrointestinal (GI) (10%), and neurologic (10%) involvement. The PVAS at presentation was 9 of 63 (range 4-24). Histopathologic analysis of the skin showed necrotizing vasculitis in biopsy samples from 40 of 50 children. Results of selective visceral arteriography suggested the presence of PAN in 96% of patients. Treatment included cyclophosphamide and corticosteroids (83%), plasma exchange (9%), and biologic agents (after 2002; 13%). The relapse rate was 35%, and the mortality rate was 4%. Severe GI involvement was associated with increased risk of relapse ( P = 0.031), while longer time to induce remission ( P = 0.022) and increased cumulative dose of cyclophosphamide ( P = 0.005) were associated with lower relapse risk. Conclusion Childhood PAN is a severe inflammatory disease of insidious onset and variable clinical presentation. Relapses occurred more frequently in those with severe GI involvement. A higher cumulative dose of cyclophosphamide was associated with a lower risk of relapse. [ABSTRACT FROM AUTHOR]

Background: Children with renovascular hypertension often present with severe hypertension. Some children have severe obstruction of their renal arteries resulting in <10% relative function on [Tc]dimercaptosuccinic acid (DMSA) scan. Conventional treatment of these children has been nephrectomy of the poorly functioning kidney to normalise their blood pressure (BP). Case-Diagnosis/treatment: We describe three children aged 20 months to 9 years with severe renal artery stenosis and severe hypertension who had radionucleotide uptake of 0% in one kidney. In one case, no renal perfusion was demonstrated by duplex ultrasound scan. Significant recovery of relative renal function of 18 to 52% was achieved after revascularisation by percutaneous angioplasty or open surgery of the obstructed renal artery. Conclusion: These cases illustrate that scintigraphy alone cannot be used to predict salvageable function in children with renovascular disease. [ABSTRACT FROM AUTHOR]

Renovascular disease (RVD) is an important cause of hypertension in children, as it often is amenable to potentially curative treatment. Imaging aimed at finding RVD therefore needs to have high sensitivity so as not to miss important findings. Digital subtraction angiography is the gold standard investigation. Doppler ultrasonography, computed tomography (CT) angiography and magnetic resonance (MR) angiography can all be helpful, but none has, at present, high enough sensitivity to rule out RVD in a child with a suggestion of that diagnosis. [ABSTRACT FROM AUTHOR]

Imaging is a crucial part of the evaluation of children with primary liver malignancies. In addition to staging the intrahepatic extent of the tumour, the 2005 revision of the PRETEXT system specifies certain criteria for assessment of extrahepatic disease. This article reviews the imaging findings of two of these criteria: direct extrahepatic extension of the tumour (E1, E1a, E2, E2a) and tumour rupture at diagnosis (H1). [ABSTRACT FROM AUTHOR]

This paper reviews current concepts and results in the management of congenital tracheal stenosis (CTS). Diagnostic options are considered and the requirements for successful management defined. Chief amongst these is a multidisciplinary approach with individualised patient management.Severe long-segment CTS represents the biggest challenge to clinicians and the worst problems for affected families. Near-death episodes are frequent in affected infants and some cannot be ventilated and require Extra-Corporeal Membrane Oxygenation (ECMO). Associated cardiovascular anomalies are frequent. Patients require immediate resuscitation and transfer to a specialist unit.After careful assessment, accurate diagnosis, and discussion, primary resection and end-to-end repair with a slide technique should always be the first option, with concomitant repair of associated cardiac anomalies. If this is impossible because of the severity of the lesion, some form of patch tracheoplasty will be indicated. Cardiopulmonary bypass is often required. Patches include pericardium, autograft trachea, carotid artery, cartilage, and allograft trachea.Mortality ranges from 0% to 30% in the literature, which largely comprises single-centre long-term experience. Recurrence is common and can be managed by stenting and tracheal homograft implantation. Long-term quality of life of survivors is little reported but seems good. Physiological data are lacking.To improve results, we suggest a treatment algorithm to rationalise care. [Copyright &y& Elsevier]

Alveolar soft-part sarcoma (ASPS) is a rare tumour. Certain distinctive clinical and radiological features suggest the correct diagnosis. There is moderate predilection for young women. ASPS almost always arises in skeletal muscle and occurs most frequently in the lower limbs. There is often a long clinical history and a large mass at presentation. Two young females with ASPS presented with very vascular tumours in the thigh, with prominent intra- and extra-tumoural blood vessels. The imaging findings and the existing literature are reviewed. [ABSTRACT FROM AUTHOR]

Background: The findings at chest radiography in HIV negative patients with cryptococcosis are said to differ from those in HIV positive patients. No large series of HIV negative patients has been reported.Methods: A review of the medical charts and radiographs of 44 consecutive HIV negative patients with proven cryptococcosis was undertaken.Results: Air space consolidation involving one or more lobes was found in 15 patients (34%), one or more pulmonary masses in 13 patients (30%), and interstitial opacities in three patients (7%). No other radiographic finding was detected in more than one patient. Twelve of 23 patients (52%) with cryptococcal meningitis had abnormal chest radiographs, and 10 of 16 patients (62%) who presented with respiratory symptoms had air space consolidation.Conclusions: HIV negative patients tend to have different radiographic findings from those reported in patients with AIDS. Cryptococcal meningitis in this group is usually associated with pulmonary mass(es) or a normal chest radiograph, whereas a respiratory presentation is usually associated with air space consolidation. [ABSTRACT FROM AUTHOR]

Medulloepithelioma is a rare tumour with a very poor prognosis. The most frequent site in the central nervous system is the cerebral hemispheres. Appearances on MRI are very variable, possibly reflecting the divergent forms of differentiation seen at histological examination. We report a rare case of sellar and suprasellar medulloepithelioma in a 2-year-old boy and discuss the imaging, differential diagnosis and pathologic findings. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Hyponatremia in a Child With Takayasu Arteritis: Questions and Answers" by S. Masavkar et al.

The author argues on the diagnosis and treatment of rapidly involuting congenital hemangioma (RICH). He says that Franchi-Abella and colleagues showed that antenatally diagnosed liver haemangiomas behave like RICH, but he and his colleagues found that the hepatic infantile haemangioma were certainly actually RICH. He added that reports recognized RICH as an entity because they are disconnected from experts including dermatologists, radiologists, and other specialists.

The article discusses renovascular disease as a cause of hypertension in children. The pathogenesis, diagnostic methods, and various medical treatments associated with the condition are explained. The various syndromes that have been linked with renovascular hypertension, including medial fibroplasia and neurofibromatosis, are examined. The role of vasculitides such as Takayasu's disease in studies of children with renovascular hypertension in India and South Africa are analyzed. The genetic component of the disease and the difficulties of diagnosing the condition in children are discussed.