Person: EKER, NURŞAH
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EKER
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NURŞAH
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Publication Metadata only Primer immun yetmezlik tanılı hastalarda malignite çeşitliliği(2018-04-11) TRUE, ÖMER; EKER, NURŞAH; KIYKIM A., SÜREKLİ Ö., NAİN E., KASAP N., AKTÜRK H., DOĞRU Ö., EKER N., CANBOLAT A., SOMER A., KOÇ A., et al.İRİŞ: Primer immün yetmezliği (PIY) olan hastalarda malignite gelişimi normal popülasyona göre daha yüksek oranda görülmektedir. Kliniğimizde takip ettiğimiz primer immün yetmezliği olan hastalarda gelişen maligniteleri, risk faktörlerini ve tedavileri sunmayı amaçladık. Yöntem: Primer immün yetmezliğe sahip olan ve malignite geliştiren 16 hastanın verileri değerlendirildi. Demografik özellikler, geçirilen enfeksiyonlar, otoimmünite, gelişen malignite tipleri, tedavi ve prognozları değerlendirildi. Bulgular: Toplam 17 hastanın yaş: 16.2±8.8 yıl, malignite gelişme yaşı 11.7±6.8 yıl idi. Hastaların tanıları sırasıyla: Kombine immün yetmezlik (n:13), antikor eksikliği ile giden immün yetmezlik (n: 4) idi. Kombine IY grubunda Bloom Sendromu (n:2), Ataksi-Telenjektazi (n:1), DOCK 8 eksikliği (n:2), Pürin Nükleozid Fosforilaz eksikliği (n:1), Epstein-Barr Virus ilişkili immün yetmezlik (n: 2), Nijmegen breakage sendromu (n:1), 4 hastada ise kombine immün yetmezliğin moleküler nedeni belirlenemedi. Antikor eksikliği grubunda, yaygın değişken immün yetmezlik (n:2), fosfoinositol 3 kinaz reseptör-1 eksikliği (n:2) bulunmaktaydı. En sık görülen malignite lenfoma (n:8) iken, kolanjiokarsinom, Wilms tümörü, gastrik ve kolon adenokarsinom, vulvar skuamöz karsinom, akut myeloid lösemi, nazal skuamöz karsinom diğer görülen malignitelerdi. Üç olguda ciddi non-neoplastik lenfoproliferasyon saptandı. Lenfoma geliştiren olguların 5'inde EBV pozitifliği saptandı. Toplam 6 olgu malignite tedavisi sırasında kaybedildi. Genel sağkalım oranı %64.7 olarak belirlendi (Şekil 1). Sonuç: PIY'lerde malignite gelişimi bir çok faktörle ilişkili olabilmektedir. Altta yatan genetik nedenler kronik yangı, DNA tamir kusurları, myeloid ve lenfoid hücrelerde gelişim bozukluklarına neden olarak maligniteye yatkınlık yaratabilmektedir. Bunun yanında sık geçirilen enfeksiyonlar ve çevresel etkenler de PIY hastalarında malignite oluşumunu kolaylaştırmaktadır.Publication Metadata only Outcome of ewing sarcoma in children, twenty years experience from a single center in Turkey(2017-09-01) EKER, NURŞAH; TOKUÇ, AYŞE GÜLNUR; TRUE, ÖMER; EKER N., YILMAZ B., TOKUÇ A. G., ŞENAY R. E., BERK B., DOĞRU Ö.Background/Objectives: Ewing sarcoma (ES) is the second common primary bone malignancy in pediatric patients. Usually, these tumors occur in bone but sometimes they can olsa orginate in soft tissue. These tumors are agressive and treatment involves multidurgs chemotherapy, radiotherapy and surgery. The aim of this study was to determine outcomes of Ewing sarcoma in pediatric patients who was treated in our instution. Design/Methods: This is a retrospective study of 75 pediatric patients with Ewing Sarcoma treated in between 1996 to 2016. Results: During a 20-year period, 75 patients were identified with Ewing Sarcoma in hospital database and their records were analyzed retrospectively. Of the 75 patients, 45 (60%) were males, 30 (40%) were females. The mean age was 10 years (ranging from 1year to 17 years). All of the patients had received the same chemotherapy protocol at presentation. This protocol involved ifosfamide, etoposide, vincristine, doxorubicine, cyclophosphamide and actinomycin. After 3 cycles of chemotherapy, surgery had been performed for most of the patients. Radiotherapy had been performed for the patients who had more than 10% viable cells after pathological examinations. For these patients, chemotherapy had been changed and continued during and after radiotherapy. The second chemotherapy protocol invoved vincristine, cyclophosphamide and topotecan. At the presentation, 22 (29 %) patients had metastatic disease. During the follow up 16 (23 %) patients had relapsed. The 5-year event free survival and overall survival were 46 % and 58,5 %. Metastatic disease at presentation was the significant factor on overall survival. Conclusions: The management of a child or adolescent with Ewing sarcoma is best carried out in a specialized center under the care of a multidisciplinary team, in order to obtain the best outcome for the patient. Early diagnosis is very important because metastatic disease at presentation reduces the overall survival.Publication Metadata only Central nervous system tumors(2017-09-01) EKER, NURŞAH; TOKUÇ, AYŞE GÜLNUR; TRUE, ÖMER; YILMAZ B., EKER N., TOKUÇ A. G., DOĞRU Ö., ŞENAY R. E., BERK B.Background/Objectives: Central nervous system (CNS) tumors are the most common solid tumors in childhood. Our instution is the one of the major referral center for pediatric brain tumors in Turkey. We aimed to analyzed children with brain tumors who were diagnosed and treated at our center in this study. Design/Methods: This is a retrospective study of 96 pediatric patients with brain tumors treated in between 2009 to 2017. Sixteen patients were lost to follow-up and 80 patients were included in the analysis. Demographic informations, histologic subtypes, stage at diagnosis, treatment modalities and outcomes were evaluated, retrospectively. Results: Of the 80 patients, 42 (52.5%) were males, 38.5 (47.5%) were females. The mean age was 6.8 ± 4.6 years (ranging from 0.17 years to 15.5 years). The mean duration of follow up was 30 months. Mean survival time was 71.4 months (95% CI: 61.8 - 80.9). The most common localization was the infratentorial area (38.8%). Among all of the patients, gliomas are the most common histologic form (54 %) and 51,3% had grade IV stage for WHO. Five-year overall survival (OS) is 68 %. The most important factor for OS was tumor total resectabity. Total resectable patients OS was 81% vs gross total resection (GTR) was 49.5% vs partial resection was only 28% within 5 years. The children with diffuse infiltrative pontine glioma (DIPG) and atypical teratoid/rhabdoid tumor (AT/RT) had the worst prognosis and these patients died in the first year of their treatment. Conclusions: In developing countries, as the use of molecular studies could not be routinely performed in clinical practice. Brain tumors are relatively common cancers among children.usually the first and the most important step in therapy. Patients with the most complete resection have significantly longer survival despite all of the technological advances.Publication Metadata only Malignancy and lymphoid proliferation in primary immune deficiencies; hard to define, hard to treat(WILEY, 2020) KOÇ, AHMET; Kiykim, Ayca; Eker, Nursah; Surekli, Ozlem; Nain, Ercan; Kasap, Nurhan; Akturk, Hacer; Dogru, Omer; Canbolat, Aylin; Somer, Ayper; Koc, Ahmet; Tokuc, Gulnur; Bozkurt, Suheyla; Turkoz, Kemal; Karakoc-Aydiner, Elif; Ozen, Ahmet; Baris, SafaBackground Regarding the difficulties in recognition and management of the malignancies in primary immune deficiencies (PIDs), we aimed to present the types, risk factors, treatment options, and prognosis of the cancers in this specific group. Methods Seventeen patients with PID who developed malignancies or malignant-like diseases were evaluated for demographics, clinical features, treatment, toxicity, and prognosis. Results The median age of malignancy was 12.2 years (range, 2.2-26). Lymphoma was the most frequent malignancy (n = 7), followed by adenocarcinoma (n = 3), squamous cell carcinoma (n = 2), cholangiocarcinoma (n = 1), Wilms tumor (n = 1), and acute myeloid leukemia (n = 1). Nonneoplastic lymphoproliferation mimicking lymphoma was observed in five patients. The total overall survival (OS) was 62.5% +/- 12.1%. The OS for lymphoma was 62.2% +/- 17.1% and found to be inferior to non-PID patients with lymphoma (P = 0.001). Conclusion In patients with PIDs, malignancy may occur and negatively affect the OS. The diagnosis can be challenging in the presence of nonneoplastic lymphoproliferative disease or bone marrow abnormalities. Awareness of susceptibility to malignant transformation and early diagnosis with multidisciplinary approach can save the patients' lives.Publication Metadata only Twenty years’ experience in the management of childhood germ cell tumors(2017-09-01) TRUE, ÖMER; TOKUÇ, AYŞE GÜLNUR; EKER, NURŞAH; ŞENAY R. E., DOĞRU Ö., TOKUÇ A. G., EKER N., YILMAZ B., BERK B.Background/Objectives: Germ cell tumors (GCT) account for 2-3% of childhood tumors. It is most commonly seen under 3 years and over 12 years old. GCTs are most commonly localized in the gonads. We have examined our patients who diagnosed and treated with GCT at our center retrospectively. Design/Methods: Sixty two children and adolescence who were diagnosed and treated with GCT at our center between 1996 and 2016 analyzed retrospectively. Results: The patients age ranged between 5 days to 17 years. Thirty-five of patients were girls (57%) and 27 were boys (43%). The most common tumor localization with 29(47%) patients was gonads. Sixteen patients (25,8%) had metastatic disease at presentation. The most common histologic subtype was mature teratoma with 14 (22%) patients and 14 patients (22%) with yolk sac tumor. Alpha-fetoprotein (AFP) was elevated in 29 (46%) of cases whereas beta-human chorionic gonadotropin (𝛽-hCG) was elevated in only 4 (4%) of cases. Twenty-two cases (35%) were treated with total surgical resection and they didn't receive chemotherapy. Six patients (10%) received radiotherapy in addition to surgery and chemotherapy. Thirty-three patients (53,3 %) received BEP (bleomycin, etoposide, cisplatin) as a first line chemotherapy. During the follow up 6 (10 %) patients had relapsed. Five years overall survival rate of our patients was 95%, retrospectively. Conclusions: Germ cell tumors have favorable outcome in children. These tumors can be cured with surgical resection followed by cisplatin-based chemotherapy and radiotherapy for selected patients. As a result of our own data analysis, it has been found that our survival rates are similar to current literature data.