Epithelioid hemangioendothelioma in children: The European Pediatric Soft Tissue Sarcoma Study Group experience.

The European pediatric Soft tissue sarcoma Study Group analyzed all children with epithelioid hemangioendothelioma prospectively registered in the NRSTS-05 (EUDRACT 2005-001139-31) and in MTS-2008 (NCT00379457) studies: 10 patients with localized and one with metastatic disease. Median age was 14.3 years (range, 9.

Epigenetic footprint enables molecular risk stratification of hepatoblastoma with clinical implications.

Background & Aims: Hepatoblastoma (HB) is a rare disease. Nevertheless, it is the predominant pediatric liver cancer, with limited therapeutic options for patients with aggressive tumors. Herein, we aimed to uncover the mechanisms of HB pathobiology and to identify new biomarkers and therapeutic targets in a move towards precision medicine for patients with advanced HB.

CD44-high neural crest stem-like cells are associated with tumour aggressiveness and poor survival in neuroblastoma tumours.

Background: Neuroblastoma is a paediatric tumour originated from sympathoadrenal precursors and characterized by its heterogeneity and poor outcome in advanced stages. Intra-tumoral cellular heterogeneity has emerged as an important feature in neuroblastoma, with a potential major impact on tumour aggressiveness and response to therapy. CD44 is an adhesion protein involved in tumour progression, metastasis and stemness in different cancers; however, there has been controversies about the significance of CD44 expression in neuroblastoma and its relationship with tumour progression.

Neural crest derived progenitor cells contribute to tumor stroma and aggressiveness in stage 4/M neuroblastoma.

Pediatric tumors arise upon oncogenic transformation of stem/progenitor cells during embryonic development. Given this scenario, the existence of non-tumorigenic stem cells included within the aberrant tumoral niche, with a potential role in tumor biology, is an intriguing and unstudied possibility. Here, we describe the presence and function of non-tumorigenic neural crest-derived progenitor cells in aggressive neuroblastoma (NB) tumors.

[Early clinical trials in paediatric oncology in Spain: a nationwide perspective].

Introduction: Cancer is the leading cause of death between the first year of life and adolescence, and some types of diseases are still a major challenge in terms of cure. There is, therefore, a major need for new drugs. Recent findings in cancer biology open the door to the development of targeted therapies against individual molecular changes, as well as immunotherapy.

Surgical management of Wilms tumor with intravascular extension: a single-institution experience.

The purpose of this study was to retrospectively analyze the clinical presentation, treatment, and outcomes of children with Wilms tumor (WT) and intravascular extension who were treated at a single institution. A retrospective review was conducted of medical records of all children with Wilms tumor and intravascular extension treated at Virgen del Rocio Children's Hospital between 1992 and 2010. Seven patients (median age 3.

Myeloablative therapy against high risk Ewing's sarcoma: A single institution experience and literature review.

Background: Attempts to improve survival outcomes of patients with high risk Ewing's sarcoma (ES) have focused on chemotherapy dose intensification strategies.

Aim: The objective of this study is to retrospectively evaluate clinical characteristics and outcome of pediatric patients with high risk ES treated at a single institution.

Materials And Methods: From 1995 to 2008, seventeen patients (male:female, 14:3) were treated with dose-intensive therapy in our institution.

Role of radiation therapy in the multidisciplinary management of Ewing's Sarcoma of bone in pediatric patients: An effective treatment for local control.

Background: Radiotherapy (RT) plays an important role in the multidisciplinary management of Ewing's Sarcoma (ES), especially in unresectable cases.

Aim: Assessment of efficacy of RT in terms of local control in pediatric patients with primary ES of bone.

Materials And Methods: Thirty-six patients younger than 17 years old with ES treated with combined RT and chemotherapy with (N = 14) or without (N = 22) prior surgery from 1981 to 2008 were retrospectively reviewed.

Surgical treatment of ocular toxocariasis: anatomic and functional results in 45 patients.

Purpose: To evaluate the anatomic and functional outcome after surgery in patients with complicated ocular toxocariasis.

Methods: A retrospective review of the medical records of pediatric patients who underwent a surgical procedure for ocular toxocariasis from July 1990 to January 2005. Patients with postoperative follow-up shorter than 6 months were excluded from the study.

Vincristine-ticlopidine-aspirin: an effective therapy in children with Kasabach-Merritt phenomenon associated with vascular tumors.

Kasabach-Merritt phenomenon (KMP) is a serious coagulopathy with severe thrombocytopenia (<10,000/mm³) that occurs in the presence of an enlarging vascular tumor such as kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). The natural history and treatment of these lesions remain controversial. The authors report a KHE case and a TA case that presented with KMP, describing their successful pharmacological management with vincristine, ticlopidine, and aspirin.

Ocular parasitic diseases: a review on toxocariasis and diffuse unilateral subacute neuroretinitis.

Parasitic infections may damage various ocular tissues, thereby causing visual dysfunction. In 1950, Wilder described the first case in which larval forms of nematodal intestinal roundworms (Ascaridoidea: Ascaris, Toxocara, Ancylostoma, Necator, and Strongyloides) were implicated as a cause of intraocular disease. This review focuses on two disorders associated with parasitic infections: ocular toxocariasis and diffuse unilateral subacute neuroretinitis.

Intravitreous bevacizumab injection: an experimental study in New Zealand white rabbits.

Objectives: To determine the effects of intraocular pressure (IOP) and needle diameter on the amount of reflux after intravitreous bevacizumab injection.

Methods: Prospective randomized interventional study. Twelve New Zealand white rabbits weighing approximately 2.

Initial experience involving treatment and retreatment with carmustine wafers in combination with oral temozolomide: long-term survival in a child with relapsed glioblastoma multiforme.

Background: Glioblastomas occur infrequently in children, and the prognosis is better than for glioblastomas seen in adults. Aggressive treatment is justified in pediatric patients.

Observations: We present the case of a 6-year-old child with malignant posterior temporal glioma treated with surgery, radiotherapy, local chemotherapy with carmustine wafers, and oral therapy with temozolomide, both at initial diagnosis and at relapse 18 months later.

Diffuse unilateral subacute neuroretinitis in Venezuela.

Purpose: To describe the clinical features and management of the largest reported series to date of patients with diffuse unilateral subacute neuroretinitis (DUSN).

Design: Observational case series.

Methods: The charts of all patients coded as having DUSN in a vitreoretinal clinic in Caracas, Venezuela between July 1979 and August 2000 were retrospectively reviewed.

Prevalence and risk factors for proliferative vitreoretinopathy in eyes with rhegmatogenous retinal detachment but no previous vitreoretinal surgery.

Purpose: To determine the prevalence of and risk factors for proliferatative vitreoretinopathy (PVR) in eyes with rhegmatogenous retinal detachment but no previous vitreoretinal surgery.

Design: Observational case series.

Methods: Prospective study.

Retinal detachment in myopic eyes after laser in situ keratomileusis.

Purpose: To report the characteristics and surgical outcomes of rhegmatogenous retinal detachments in myopic eyes after laser in situ keratomileusis (LASIK).

Methods: Clinical charts of patients that developed rhegmatogenous retinal detachment after LASIK were reviewed. Surgery to repair rhegmatogenous retinal detachment was performed in 31 eyes (mean follow-up of 14 months after vitreoretinal surgery).