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Androgens have represented the historical therapeutic backbone of bone marrow failure (BMF) syndromes. However, their role has been rarely analyzed in prospective setting and systematic and long-term data are currently unavailable regarding their usage, effectiveness and toxicity in both acquired and inherited BMF. Here, taking advantage of a unique disease-specific international dataset, we retrospectively analyzed the so far largest cohort of BMF patients who received androgens before or in absence of an allogeneic hematopoietic cell transplantation (HCT), reappraising their current use in these disorders. We identified 274 patients across 82 EBMT affiliated centers, 193 with acquired (median age of 32) and 81 with inherited BMF (median age of 8 years). With a median duration of androgen treatment of 5.6 and 20 months respectively, complete/partial remission rates at 3 months were of 6%/29% in acquired and 8%/29% in inherited disorders. Five-year overall survival and failure free survival (FFS) were respectively 63% and 23% in acquired and 78% and 14% in inherited contexts. Androgen initiation after second line treatments for acquired, and after > 12 months post-diagnosis for inherited group were identified as factors associated with improved FFS in multivariable analysis. Androgen use was associated with a manageable incidence of organ-specific toxicity and low rates of solid and hematological malignancies. Sub-analysis of transplant-related outcomes after exposure to these compounds showed probabilities of survival and complications similar to other transplanted BMF cohorts. This study delivers a unique opportunity to track androgen use in BMF syndromes and represents the basis for general recommendations on their use on behalf of the SAAWP of the EBMT.

Microencapsulation is a promising method that has considerable effects on protection of probiotic viability. A variety of coating materials have been utilized to enhance the stability of probiotic microorganisms during the transition through gastrointestinal tract. The aim of this research was to determine optimum coating material combinations for probiotic microencapsulation against gastric conditions. Fructooligosaccharides, peptide, sodium alginate, gelatin and gellan gum were used as entrapment substances to microencapsulate Lactobacillus plantarum and Lactobacillus casei Shirota with extrusion technique. The response surface technique was applied to detect the optimum proportion of encapsulation substances against gastric condition. Microencapsulation protected probiotic cultures against stress factors such as simulated gastric juice and bile-salt solution. Optimum rate of encapsulation substances varied according to the type of probiotic bacteria. Test results showed that L. plantarum should be coated with 1.5% alginate, 0.92% gellan gum, 0.18% gelatin, 0.36% peptide and 1.31% fructooligosaccharides for highest protection. L. casei Shirota should also be coated with 2% alginate, 0.98% gellan gum, 0.51% gelatin, 0.86% peptide and 1.98% fructooligosaccharides for highest protection. This research concluded that microencapsulation with encapsulation materials at optimum concentration provided improved protection for the probiotics.

Loss-of-function mutations in magnesium transporter 1 (MAGT1) gene cause X-linked magnesium deficiency with Epstein–Barr virus (EBV) infection and neoplasm (X-MEN), a disease with quite diverse clinical and immunological consequences. The phenotypic characteristics of the initially described patients included CD4+ T cell lymphopenia, immune deficiency, EBV viremia, and EBV-related lymphoproliferative disease. To date, a total of 25 patients have been reported. The spectrum of the MAGT1 defect ranges from other viral infections (HSV, VZV, CMV, MCV) and sinopulmonary bacterial infections, autoimmune diseases, non-EBV driven lymphoproliferative disease, Castleman disease, HHV8+ Kaposi's sarcoma, vasculitis (Kawasaki) to glycosylation defects in new patients. Here, we report 2 patients from two different families with novel MAGT1 mutations and different clinical features. The first patient presented with B cell lymphoma and low IgM level without recurrent infections. The second patient presented with recurrent upper respiratory tract infections, Kawasaki-like disease, hypogammaglobulinemia, and T cell lymphopenia. X-MEN disease is the first phenotype identified due to MAGT1 mutation. The identification of new mutations and atypical presentations will clarify whether there is a relationship between the genotype and the phenotype and the characteristics of the disease.

Sarcomatoid carcinoma of the urinary bladder is rare and presented as a high grade and an advanced stage tumor. A 79-year old man presented with macroscopic hematuria was carried out transurethral resection. Pathological examination revealed a tumor composed of epitelial and mesenchimal areas which were showed strong positivity with pancytokeratin and vimentin, respectively. We want to present this rare neoplasm of the bladder with clinic and pathologic findings.

Background The prognosis is still poor for patients with a metastatic bone tumor and new treatment approaches (anti-VEGF and tyrosine kinase inhibitors vs) are therefore needed. Objectives The aim of our study was to evaluate how the primary and metastatic lesions of our patients with a bone tumor were affected by these treatments and to determine the importance of the 18F-FDG PET method. Patients and Methods Twenty metastatic bone tumor cases were included. Sorafenib and anti-VEGF were added to the standard treatment in cases with widespread metastatic disease at diagnosis or after neoadjuvant chemotherapy showing less than 90% tumor necrosis in the surgical sample. Positron emission tomography (PET) imaging was performed at diagnosis, the preoperative period following neoadjuvant chemotherapy, during postoperative follow-up, and when treatment was discontinued. Results The primary treatment region median SUVmax level decreased from 7.35 to 2.5 in the living patients (n = 16) while there was no significant decrease in the patients who succumbed to the disease (P < 0.001). Comparison of the pre- and post-treatment metastasis region median SUVmax levels in patients with metastatic involvement showed a decrease from 2.1 to 0 in the surviving patients but only from 4.8 to 3.2 in the deceased patients (P < 0.01). Survival results indicated that 28.6% of the patients receiving classical treatment only died while all the patients receiving additional sorafenib and anti-VEGF survived. Conclusions 18F-PET may be a useful technique before and during the follow-up of neoadjuvant treatment in pediatric metastatic bone tumor patients. The addition of sorafenib and anti-VEGF to classical treatment has a favorable contribution to the response and therefore the survival duration.

Introduction TPOG-NBL 2003 national protocol was designed to improve treatment results of the high risk patients by adjunction of stem cell rescue with intensive multimodal therapy. Material and Methods High risk stratification was made according to COG criteria. Just before the third cycle of chemotherapy, patients without progression were allocated into two treatment groups non-randomly by physicians’ and/or parent’s choices guided to the center’s facility, toxicity and social-economical facility to attain the megatherapy. After an induction of 6 chemotherapy cycles, the protocol was divided into two arms which were designed to continue the intensive conventional chemotherapy (CCT), or initiate myeloablative therapy with autologous stem-cell rescue (ASCR). All patients were also given 13-cis-retinoic acid as maintenance therapy. Results Fifty-six percent (272 patients) of all neuroblastoma patients was evaluated as high risk. Response rate to induction chemotherapy was 81% (CR/VGPR: 32%, PR: 49%) in patients at the end of induction chemotherapy. Overall EFS and OS at 3-years were 36% and 45%, respectively. Intention-to-treat analysis documented post-induction (after the six cycles of induction chemotherapy) EFS of 46% in CCT arm (137 patients) and 37% in ASCR group (55 patients) (p= 0.037); whereas, OS was 59% and 43%, respectively (p=0.052). Thirty-one patients (11%) died of treatment-related complications. Conclusion Survival rates of high-risk neuroblastoma have improved over the last decade in Turkey. The main problems when managing these patients were an effective local control, early progression and death. Megatherapy has not augmented the therapeutic end point in our country’s circumstances. However; the better the supportive care and the higher the patients’ compliance is attained, the higher the survival rates might be obtained in Turkish neuroblastoma patients. Disclosures: No relevant conflicts of interest to declare.

Mezenterik lipomalar matür yağ hücrelerinin iyi huylu tümörleridir. Genellikle asemptomatik olmalarına rağmen, lokalizasyonlarına ve büyüklüğüne bağlı olarak klinik tablo oluştururlar. Çok nadir görülmelerine rağmen, çok büyümüş dev mezenterik lipomalar kısmi veya tam barsak tıkanıklığına neden olabilirler. Kısmı barsak tıkanıklığı oluşturan lipomalar aralıklı karın ağrısı ve karın şişliği gibi şikayetlere neden olurken, tam barsak tıkanıklığı gelişen olgular akut batın tab- losuyla karşımıza çıkarlar. Tedavileri etkilenmiş barsak bölümüyle bir- likte kitlenin eksize edilmesidir. Bu çalışmada dev mezenterik lipom nedeniyle barsak tıkanıklığı gelişmiş 2 yaşında bir kız olgu ve şu ana kadar ingilizce literatürde yayınlanmış 16 olgunun klinik özellikleri sunulmuştur., Mesenteric lipomas are benign tumors of mature fat cells. They are usually asymptomatic and create a clinical picture that depends on the localization and size of the lipoma. Although rare, unusually large mesenteric giant lipomas can cause partial or complete bowel obstruction. Lipomas resulting in partial bowel obstruction can present with symptoms such as intermittent abdominal pain and abdominal distention. With complete obstruction, a child can present with an acute abdomen. Treatment is the excision of the mass along with the affected portion of bowel. in this case study, a 2-year-old female presented with a bowel obstruction due to the presence of a giant mesenteric lipoma. Clinical features of 16 cases published in the English literature to date are presented.

The current report describes a 23-month-old boy with paratesticular rhabdomyosarcoma who was incidentally diagnosed as also having an isolated mediastinal cystic lymphangioma. The association of childhood rhabdomyosarcoma with various congenital anomalies and genetic alterations such as p53 mutations have been well known. However, mediastinal cystic lymphangioma has not been reported among the congenital anomalies diagnosed in rhabdomyosarcoma. Both rhabdomyosarcoma and lymphangioma originate from mesenchymal cells. This association may be coincidental or may point to a common genetic and/or developmental disorder of the mesencymal tissue.

Angioleiomyoma is an uncommon type of leiomyoma of the uterus that originates from smooth muscle cells and contains thick-walled vessels. A 45-year-old woman with the complaint of lower abdominal pain was admitted to the hospital. In the operation a ruptured, bleeding uterine tumor was seen. She underwent total hysterectomy and bilateral salpingo-oophorectomy. On pathologic examination of the specimen, the tumor was diagnosed as angioleiomyoma. Here, an unusual clinical presentation of uterine angioleiomyoma was reported.

WOS: 000375613200005, Eight samples of mature sourdough were collected from five provinces of Turkey. Lactic acid bacteria and yeasts were isolated and identified and used in different combinations to produce liquid sourdoughs. Microbiological and physicochemical characteristics of the experimental sourdoughs made with different flour types and dough yields were studied. The main lactic acid bacteria species identified were Lactobacillus (L.) sanfranciscensis, Pediococcus pentosaceus, L. plantarum, L. namurencis, L. rossiae, Leuconostoc mesenteroides and L. zymae. L. spicheri, L. paralimentarius, L. mindensis, L. farciminis, L. acetotolerans, L. casei, Enterococcus faecium and Enterococcus durans were also found in sourdoughs at subdominant levels. Among yeasts, mainly Saccharomyces cerevisiae, but also Pichia guiliermondii and Torulaspora delbrueckii were the predominant species of yeasts identified in sourdoughs. Lactic acid bacteria and yeasts of liquid sourdoughs after fermentation were in the range of 9.61-9.89 log cfu/g and 6.55-7.36 log cfu/g, respectively. Various chemical parameters such as pH, total titratable acidity, lactic and acetic acids, ethanol and sugars were determined for liquid sourdoughs. Acidification and metabolite contents of these products were different, depending on the starter culture, flour type and dough yield. Total titratable acidity was more pronounced in the sourdoughs produced with whole wheat flour (14.08 mL NaOH) and rye flour (13.56 mL NaOH), dough yield 250 (13.93 mL NaOH) and control sample (13.12 mL NaOH) which were produced without inoculum., Cukurova University Academic Research Projects Unit [ZF2009D20], This work has been funded by a grant from Cukurova University Academic Research Projects Unit (ZF2009D20). The authors also thank Prof. F. Ozcelik (Ankara University, Turkey), Assoc. Prof. M.S. Ozer (Cukurova University, Turkey) and Prof. H. Ozkan (Cukurova University, Turkey) for their kind collaboration.