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INTRODUCTION: The ETS-associated gene (ERG) is an oncogene that encodes one of the erythroblast transformation-specific (ETS) transcription factors. Although there are many studies on the effects of different malignancies, especially prostate cancer, Ewing sarcoma and acute myeloid leukemia, its association with Wilms tumor has never been investigated. The aim of this study was to evaluate ERG tissue expression in Wilms tumors. METHODS: ERG expression and its association with clinicopathological features were evaluated in tissue sections obtained from 53 children with Wilms tumors. RESULTS: The mean age of the patients was 3.21 +- 2 years. This series consisted of 25 boys (47.2%) and 28 girls (52.8%). The mean tumor size was 9.1 +- 2.9 cm. The mean kidney weight was 474.5 +- 310.7 g. Thirteen (24.5%) cases were stage I, 20 (37.7%) were stage II, 7 (14%) were stage III and 6 (11.3%) were stage IV. Forty-two cases were alive (79.2%) and were in follow-up. The mean survival time was 65.3 +- 40.2 (2-148) months. Nuclear ERG expression was detected in 21 (39.6%) cases. In 8 (15.1%) cases, ERG expression was limited to pure blastemal component. Eleven cases (20.8%) had ERG expression in blastemal and mesenchymal components. Two patients (3.8%), one of which with bilateral tumors, had diffuse nuclear ERG expression. There was no correlation between ERG expression and prognostic factors except tumor bilaterality. DISCUSSION AND CONCLUSION: This study demonstrates that nuclear ERG expression is not related to the pathogenesis of Wilms tumor. Although there was no statistically significant p value; since ERG expression is more common in bilateral tumors, it is thought that ERG may have a supporting role in the development of WT.

INTRODUCTION: The hypoxia- inducible factor (HIF) is an alpha (α)/ beta (β) heterodimeric DNA binding complex and directs an extensive transcriptional response involving the induction of genes relevant to tumor progression, such as angiogenesis, glucose/ energy metabolism, cellular growth, metastasis, and apoptosis. HIF-1A has also emerged as an attractive target for cancer therapy. The aim of this study is to investigate the association between tissue HIF-1A expression, prognostic significance and the clinicopathologic features of Wilms tumors. METHODS: Nuclear HIF-1A expression in 53 tissue samples harvested from children with Wilms tumor and its relationship with prognostic parameters were evaluated. RESULTS: Tissue samples of 53 cases (male, n=25: 47.2 %, and female, n=28: 52.8 %) with Wilms tumor with a mean age of 3.21+-2 years were analyzed. Mean tumor size, and weight of kidneys were 9.1 +- 2.9 cm in diameter and 474.5+-310.7 gr, respectively. Thirteen (24.5%) cases were in stage I, 20 (37.7%) in stage II, 7 (14%) in stage III, and 6 (11.3%) stage IV. Forty-two cases were alive (79.2%), while 11 cases (20.8%) were deceased. Mean overall survival time was 65.3+-40.2 (2- 148) months. Nuclear HIF-1A expression was positive in only 3 viably tumors (5.7%), while it was negative or cytoplasmic artificially positive in other tumors. Statistically, there was no any correlation between HIF-1A expression and other prognostic factors. DISCUSSION AND CONCLUSION: In contrast to other reports, this study indicated that HIF-1A expression is not associated with development and pathogenesis of Wilms Tumor.

Objective: This study aimed to define the status of juvenile myelomonocytic leukemia (JMML) patients in Turkey in terms of time of diagnosis, clinical characteristics, mutational studies, clinical course, and treatment strategies. Materials and Methods: Data including clinical and laboratory characteristics and treatment strategies of JMML patients were collected retrospectively from pediatric hematology-oncology centers in Turkey. Results: Sixty-five children with JMML diagnosed between 2002 and 2016 in 18 institutions throughout Turkey were enrolled in the study. The median age at diagnosis was 17 months (min-max: 2-117 months). Splenomegaly was present in 92% of patients at the time of diagnosis. The median white blood cell, monocyte, and platelet counts were 32.9x109/L, 5.4x109/L, and 58.3x109/L, respectively. Monosomy 7 was present in 18% of patients. JMML mutational analysis was performed in 32 of 65 patients (49%) and PTPN11 was the most common mutation. Hematopoietic stem cell transplantation (HSCT) could only be performed in 28 patients (44%), the majority being after the year 2012. The most frequent reason for not performing HSCT was the inability to find a suitable donor. The median time from diagnosis to HSCT was 9 months (min-max: 2-63 months). The 5-year cumulative survival rate was 33% and median estimated survival time was 30+-17.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p=0.019). Older age at diagnosis (>2 years), platelet count of less than 40x109/L, and PTPN11 mutation were the factors significantly associated with shorter survival time. Conclusion: Although there has recently been improvement in terms of definitive diagnosis and HSCT in JMML patients, the overall results are not satisfactory and it is necessary to put more effort into this issue in Turkey.

Pancreatoblastoma, rarely encountered in the literature, is a malignant exocrine tumor seen in the pancreas. A 5-year-old boy suffering from abdominal pain was sent to our institute for further examination and treatment. Clinical examination was normal but for a palpable abdominal tumor mass. Abdominal Doppler ultrasonography showed a mass with well-defined margins within the body of the pancreas. Laboratory tests, including lactic dehydrogenase, alpha-fetoprotein and cancer antigen 125 were abnormal. The tumor invading the splenic vein and transverse colon was removed totally. We observed a hypercellular tumor in histopathological examination. The tumor had epithelial acinar cells and squamoid morules (corpuscles) separated by stromal bands. Adjuvant chemotherapy was used after surgery. However, the patient died 14 months later. All data about pancreatoblastoma have to be collected in order to choose the treatment to elucidate the molecular pathogenesis of the tumor, to diagnose it early and to develop target-specific treatments.

Objective: The prognostic factors and a new childhood prognostic index after autologous hematopoietic stem cell transplantation (AHSCT) in patients with relapsed/refractory Hodgkin's lymphoma (HL) were evaluated. Materials and Methods: The prognostic factors of 61 patients who underwent AHSCT between January 1990 and December 2014 were evaluated. In addition, the Age-Adjusted International Prognostic Index and the Childhood International Prognostic Index (CIPI) were evaluated for their impact on prognosis. Results: The median age of the 61 patients was 14.8 years (minimummaximum: 5-20 years) at the time of AHSCT. There were single relapses in 28 patients, ≥2 relapses in eight patients, and refractory disease in 25 patients. The chemosensitivity/chemorefractory ratio was 36/25. No pretransplant radiotherapy, no remission at the time of transplantation, posttransplant white blood cell count over 10x103/ μL, posttransplant positron emission tomography positivity at day 100, and serum albumin of

Central nervous system (CNS) involvement of Epstein-Barr virus (EBV)-associated lymphoproliferative disease is a rare and serious complication in children with leukemia. Although rituximab therapy seems to be promising in these cases, persistent hypogammaglobulinemia may appear after treatment due to complete depletion of normal B lymphocytes in the peripheral blood. Here we report isolated CNS involvement of EBV-associated lymphoproliferative disorder in a 4-year-old boy with acute leukemia. The patient was treated with rituximab and interferon alpha; however, persistent hypogammaglobulinemia developed as a complication. Given the rarity of the complication in children receiving these agents, our experience with such a case may be helpful to others.

OBJECTIVE: Immune thrombocytopenic purpura (ITP) is the most common cause of acquired thrombocytopenia in children. The objective of this study was to evaluate the presenting features, variation in the clinical courses, initial response rate to therapy, and long-term outcome in patients with ITP. METHODS: Three hundred and fifty out of 491 newly diagnosed patients with ITP between the initial diagnosis ages of 6 months to 16 years were included in this retrospective, descriptive study. Patients with acute vs chronic ITP, acute vs recurrent ITP and chronic vs recurrent ITP were compared in terms of age at diagnosis, gender, initial platelet count, response rate to initial therapy, long-term outcome, and total duration of follow-up. RESULTS: The clinical courses of the patients were determined as acute, chronic and recurrent in 63.8%, 29.1%, and 7.1%, respectively. Platelet count >20x109/L and initial diagnosis age >10 years were found to increase the probability of chronic outcome by at least two-fold. CONCLUSION: It is concluded that ITP in childhood is a common disease with low morbidity and mortality. In addition to the acute and chronic form, a rare recurrent form, which accounts for about 4-7% of all ITP patients, should be considered.