Buket Kara

Background. Gonadotropin-releasing hormone-independent precocious puberty (GnRH-independent PP) due to a human chorionic gonadotropin (hCG)-secreting mediastinal germ cell tumor is an exceptionally rare presentation, particularly in the context of Klinefelter syndrome. This case highlights the diagnostic challenges posed by GnRH-independent PP and underscores the importance of considering extragonadal germ cell tumors in the differential diagnosis of virilization with suppressed gonadotropins. Case Presentation. A 7-year-9-month-old boy presented with rapidly progressive virilization, including pubic hair development, penile enlargement, and voice deepening. Laboratory evaluation revealed markedly elevated testosterone, estradiol, β-hCG, and alpha-fetoprotein (AFP) levels, with suppressed gonadotropins and a prepubertal GnRH stimulation response. Imaging studies demonstrated a large anterior mediastinal mass containing fat and calcifications, while scrotal ultrasonography showed no testicular lesion. The mediastinal mass was completely resected and histopathology confirmed a mixed germ cell tumor composed of seminoma, yolk sac tumor, and Grade II immature teratoma with β-hCG–positive trophoblastic foci. Karyotype analysis revealed 47,XXY, consistent with Klinefelter syndrome. Following surgery and four cycles of bleomycin, etoposide, and cisplatin (BEP) chemotherapy, tumor markers normalized rapidly, and no recurrence was detected during long-term follow-up. Conclusions. This case illustrates a rare presentation of GnRH-independent PP due to a β-hCG-secreting mediastinal mixed germ cell tumor in a child with previously undiagnosed Klinefelter syndrome. Routine karyotype analysis should be considered in male patients with mediastinal germ cell tumors. In children presenting with unexplained virilization and suppressed gonadotropins, extragonadal germ cell tumors and their biochemical markers (β-hCG, AFP) must be carefully evaluated. Early recognition, timely surgical intervention, metabolic monitoring, and multidisciplinary care are critical for optimizing outcomes.

Pseudothrombocytopenia due to EDTA; It is a condition in which platelets are observed at lower than normal levels as a result of aggregation in the peripheral smear made from blood anticoagulated with EDTA. This condition can be confused with thrombocytopenia and causes incorrect diagnosis and treatment. We wrote this case to draw attention to EDTA-induced pseudothrombocytopenia, which is one of the most commonly observed causes of thrombocytopenia.

Sakrokoksigeal teratomlar yenidoğan döneminde en sık karşılaşılan teratomlardır. Sakrokoksigeal teratomlar sıklıkla doğumda tanı almakla beraber özellikle tip 1 ve 2 olanlar antenatal ultrasonografi takiplerinde fark edilebilmektedirler. Tedavi uygun cerrahi rezeksiyon ile sağlanmaktadır. Operasyon sonrası takip çocuk onkoloji birimi ile birlikte yapılmalıdır. Bu yazıda 22. Gebelik haftasında rutin ultrasonografi muayenesinde fark edilen sakrokoksigeal teratomu olan ve 34 hafta 1 günlük iken fetal distres sebebiyle acil sezaryen ile doğumu gerçekleştirilen bir yenidoğan vakası sunulmuştur.

Objective: The aim of this study is to analyze the clinical utility and cost of the respiratory virus panel test in the febrile neutropenia (FN) episode in children undergoing chemotherapy. Material and Method: From 2014 to 2018, 180 episodes of FN in 93 children with cancer were retrospectively analyzed. The patients were divided into those with (Group A) and without respiratory virus panel (Group B). The demographic and clinical features and cost analysis of the groups A and B were noted. Results: Of these FN episodes, 46 were in Group A (25.5%) and 134 were in Group B (74.5%). We found positivity in 45 (97.8%) of 46 episodes in Group A. While modification was required in 14 FN episodes (30.4%) in Group A, modification was required in 35 FN episodes (26.1%) in group B. The difference was not statistically significant (p=0.570). In Group A, only 5 (10.8%) were modified according to the respiratory virus panel. The respiratory virus panel prices were $72.43 (interquartile range, $38.8). The ratio of respiratory virus panel cost to the total cost was 9.67% (interquartile range 11.6). The median total cost of group A was $663.18 (interquartile range, 850.1), while that of group B was $596.24 (interquartile range, 723.81). The difference was not statistically significant (p=0.141). Conclusion: The respiratory virus panel may contribute to the preference of antibiotics by giving rapid results in FN attacks. However, no effect on modification rates was observed, and only a small percentage of patients underwent antibiotic modification according to respiratory virus panel.

Aim: To evaluate the demographic and clinical characteristics, treatment approaches and outcomes of our pediatric patients with non-Hodgkin lymphoma diagnosed and treated in our center. Material and Methods: Between 2006 and 2002, the oncologic charts of the patients diagnosed and followed up as non-Hodgkin lymphoma were reviewed retrospectively. Results: Eighty children with non-Hodgkin lymphoma were included in this study. There were 55 boys (68.8%) and 25 girls (31.2%). The patients’ ages ranged from 2 to 18 years (median: 11.1 years). Nine patients (11.3%) had primary immunodeficiency. Sixty-three of the patients were stage III (78.7%). The majority pathologic subtype was Burkitt lymphoma (n: 31, 38.8%). The overall survival and event-free survival rates were 71.7% and 71.5%, respectively. The patients’ overall survival rates without and with primary immunodeficiency was 81.1% and 11.1%, respectively. There was a significant difference between these two groups. Cox regression analysis showed that advanced stage and concomitant primary immunodeficiency have been risk factors for prognosis. Conclusion: Intensive treatment approaches have increased overall survival rates in children with non-Hodgkin lymphoma. However, this success rate cannot be achieved in non-Hodgkin lymphoma children with primary immunodeficiency.

Amaç: Bu çalışmanın amacı hemanjiyom tanısı alan çocuklarda demografik ve klinik özellikleri ile tedavileri ve tedavi sonuçlarının incelenmesidir. Hastalar ve Yöntemler: Ekim 2017- Eylül 2019 tarihleri arasında hemanjiyom tanısı alan 100 çocuk hastanın dosyası geriye dönük olarak incelendi. Hastaların demografik ve klinik özellikleri, tedavi yaklaşımları ve sonuçları not edildi. Bulgular: Çalışmaya dâhil edilen 100 hastanın 66’sı (%66) kız, 34’ü (%34) erkekti. Hastaların yaş gruplarına göre, 0-3 ay, 3-6 ay, 6-12 ay, 12-24 ay ve >24 ay yaş gruplarında sırasıyla 35, 28, 23, 8 ve 6 hasta vardı. Atmış bir hastada (%61), hemanjiyom baş-boyun bölgesinde yerleşimli iken, baş-boyun dışı yerleşim 39 (%39) hastada saptandı. Tedavi yaklaşımları 71 hastada oral propranolol, 21 hastada topikal timolol damla ve 8 hastada oral propranolol + oral metilprednisolon idi. Oral propranolol ile tedavi edilen 37 hastada (%52), topikal timolol damla ile tedavi edilen 7 hastada (%33,3) ve oral propranolol + oral metilprednisolon ile tedavi edilen üç hastada (37,5) yanıt alındı. Stabil hastalık, oral propranolol alan hastaların 13'ünde (%45,1), topikal timolol damla uygulanan hastaların 13'ünde (%61,9) ve oral propranolol + oral kortikosteroid alan hastaların dördünde (%50) saptandı. Sonuç: çocuklarda, hemanjiyom tedavisinde özellikle oral propranolol tedavisinin etkili ve güvenilir bir tedavi yöntemi olduğu gözlemlenmiştir.

Background: Biological inflammatory status in cancer patients is evaluated with various parameters. Limited studies have been conducted on this issue in children. Objectives: This study was done to evaluate the importance of the biological inflammatory status, including systemic inflammatory response index (SIRI), systemic immune-inflammation (SII) index, and hemoglobin-albumin-lymphocytes-platelets (HALP) score in children with cancer. Methods: Demographic characteristics, complete blood count, and biochemical analysis of the patients on admission were recorded retrospectively. Since not meeting the necessary assumptions, the Mann-Whitney U test was used for the comparison of the two groups, and the Kruskal-Wallis test was used for the comparison of more than two groups Results: The SIRI of the patients was statistically higher than the control group (P < 0.0001). Considering lymphoma, central nervous system tumors, and solid tumors, there was a statistical difference between the groups in SII index and SIRI (P values: .0245, and .0060, respectively). Regarding inflammatory biomarkers, according to the extent of cancer (localized or advanced disease), it was found that SIRI was higher in patients with advanced disease (P = 0.0175). The patients who died had a statistically higher HALP score (P = 0.0472). Conclusions: The inflammatory biomarkers can be used in childhood cancers to determine the extent of the disease and predict outcomes. However, in larger patient series, ideal values should be achieved by analyzing the receiver operating characteristic curves and the area under the curve.

Background: Medulloblastoma is the most common central nervous system tumor in childhood. This study aims to evaluate the clinical features, treatments, and outcomes of pediatric patients diagnosed with medulloblastoma Materials and Method:Between 2006 and 2019, the medical records of children with medulloblastoma were reviewed retrospectively. Patients who died after surgery, before chemotherapy or radiotherapy were not included in the survival analysis Results: During the study period, 38 children were diagnosed with medulloblastoma. Twenty-one of the patients were male (55.3%) and 17 were female (44.7%). The ages of the children ranged from 3 months to 17 years (median age 8 years). The ages of five patients were under 3 years (13.1%). The most common complaints were headache (n: 26, 68.4%), imbalance (n: 21, 55.3%), and vomiting (n: 20, 52.6%). The mass sizes ranged between 3 and 6 cm in 32 patients (84.2%). At the time of diagnosis, 5 patients had seeding metastasis (13.1%). The most commonlyused chemotherapy protocol included vincristine, cisplatin, etoposide (60.5%). Five patients died after surgery without any chemotherapy or radiotherapy. Of the 33 patients included in the life analysis, 12 died (36.4%). Follow-up times ranged from 2 months to 14 years (median, 44 months). The overall survival rate was 59.1%. Eight patients had relapsed (24.2%). Late relapse was detected in 3 of the relapsed patients (relapse times were the 91st, 69th, and 72nd months). Conclusions: It is possible to achieve satisfactory treatment results in children with medulloblastoma using international treatment guidelines and recommendations, with an experienced professional team dedicated to pediatric neurooncology.