[A MOLECULAR APPROACH TO DIAGNOSIS AND TREATMENT OF PEDIATRIC SOFT TISSUE SARCOMA FROM "E PLURIBUS UNUM" TO "EX UNO PLURES" AND BACK].

Soft tissue sarcoma is a heterogeneous group of mesenchymal (fat, muscle, cartilage and blood vessel) tumors. It collectively comprises 1% of adult malignancies, and 7% of pediatric malignancies and according to the last WHO classification, contains over 50 histologic diagnoses. Due to its relatively infrequent occurrence, diversity of sub-types and high rates of inter-observer disagreement regarding the histological sub-type, the approach to both basic and clinical research, and, accordingly, treatment has traditionally been "one size fits all".

Ocular venous occlusion in pediatrics: Should thrombophilia investigation and anticoagulant treatment be initiated?

Retinal vein occlusion (RVO) and superior ophthalmic vein thrombosis (SOVT) are rare diseases in the pediatric population; however, the ophthalmic and neurologic morbidity are significant. As published data are scarce for these conditions, we present our experience with pediatric ocular venous thrombosis in four patients, and discuss recommended management for evaluation and treatment. We suggest performing thrombophilia workup for all pediatric patients with RVO or SOVT.

Genetic testing and surveillance in infantile myofibromatosis: a report from the SIOPE Host Genome Working Group.

Infantile myofibromatosis (IM), which is typically diagnosed in young children, comprises a wide clinical spectrum ranging from inconspicuous solitary soft tissue nodules to multiple disseminated tumors resulting in life-threatening complications. Familial IM follows an autosomal dominant mode of inheritance and is linked to PDGFRB germline variants. Somatic PDGFRB variants were also detected in solitary and multifocal IM lesions.

Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for disseminated intra-abdominal malignancies in children-a single-institution experience.

Purpose: Our purpose was to present our institutional experience with performing complete cytoreduction surgery and heated intraoperative chemotherapy (CRS-HIPEC) for children with disseminated intraabdominal malignancies, guided by a leading international center performing CRS-HIPEC in children.

Methods: Retrospective chart review of all cases of CRS-HIPEC in children in our institution, examining diagnosis, preoperative management, operative management, postoperative treatment, short term outcome including length of stay and complications, and long term outcome including survival and recurrence of disease.

Results: 9 children underwent CRS-HIPEC over 48months.

Recurrent Immune Thrombocytopenia After Influenza Vaccination: A Case Report.

Immune thrombocytopenia (ITP) is an isolated autoimmune condition, often preceded by a viral infection. Vaccines, mainly the measles-mumps-rubella vaccine, have also been associated with an increased risk of developing the disease. Although some case reports of ITP after influenza immunization in adults have been published, epidemiologic studies examining the role of the influenza vaccine as a trigger of ITP have not conclusively proven causality.

The Risk of Serious Bacterial Infection in Neutropenic Immunocompetent Febrile Children.

Only few reports have looked into the risk of invasive bacterial infection in children with neutropenia that is not malignancy related. The objective of the current study was to determine the clinical significance of neutropenia as a predictor of serious bacterial infection (SBI) in immunocompetent children. We conducted a retrospective case-control study including children 3 months to 18 years of age with fever ≥ 38°C hospitalized or presenting to the emergency department.

Characterization of two unique α-globin gene cluster deletions causing α-thalassemia in Israeli Arabs.

The molecular basis of α-thalassemia (α-thal) is complex. The use of multiplex ligation-dependent probe amplification (MLPA) has offered the possibility of identifying more gene deletions causing α-thal. Our objective was to determine the molecular basis of two patients with Hb H (β4) disease.

Delta 9-tetrahydrocannabinol inhibits cell cycle progression by downregulation of E2F1 in human glioblastoma multiforme cells.

Background: The active components of Cannabis sativa L., Cannabinoids, traditionally used in the field of cancer for alleviation of pain, nausea, wasting and improvement of well-being have received renewed interest in recent years due to their diverse pharmacologic activities such as cell growth inhibition, anti-inflammatory activity and induction of tumor regression. Here we used several experimental approaches, which identified delta-9-tetrahydrocannabinol (Delta(9)-THC) as an essential mediator of cannabinoid antitumoral action.

Mechanisms operative in the antitumor activity of temozolomide in glioblastoma multiforme.

Purpose: Glioblastoma multiforme (GBM) is the most frequent and incurable brain tumor in adults. Although temozolomide (TMZ) does not cure GBM, it has demonstrated anti-GBM activity and has improved survival (8-14 months) and quality of life. We studied the mechanisms by which TMZ affects 2 human GBM cell lines; U251-MG and U87-MG, aiming to unravel the drug-activated cascades to enable the development of combination therapies that will improve the efficacy of TMZ.

CD133-positive hematopoietic stem cell "stemness" genes contain many genes mutated or abnormally expressed in leukemia.

Affymetrix human Hu133A oligonucleotide arrays were used to study the expression profile of CD133+ cord blood (CB) and peripheral blood (PB) using CD133 cell-surface marker. An unsupervised hierarchical clustering of 14,025 valid probe sets showed a clear distinction between the CD133+ cells representing the hematopoietic stem cell (HSC) population and CD133-differentiated cells. Two hundred forty-four genes were found to be upregulated by at least twofold in the CD133-positive cells of both CB and PB compared with the CD133-negative cells.