Derya Özyörük

Pulmonary Functions in Children With Thalassemia Major

Background: Thalassemia major (TM) is a chronic disease requiring regular transfusions that may result in generalized iron loading, such as in the heart, the liver, endocrine organs, and the lungs. We aimed to determine pulmonary function abnormalities in children with TM in our center. Patients and Methods: In this study, pulmonary function tests (PFTs) of 49 patients with TM who received regular blood transfusion and had no history of chronic respiratory disease were evaluated. The relationship between PFTs and the age, the body surface area, pretransfusional hemoglobin, and serum ferritin was evaluated. Results: Among the &bgr;-TM patients included in this study, 61% were male and 39% were female, with a mean age of 10.8±3 years (range, 5 to 17 y). The patients’ mean level of ferritin was 3873±2011 ng/dL (range, 676 to 9476 ng/dL). A reduced forced vital capacity (FVC) was found in 33 patients (67%). A reduced forced expiratory volume in 1 second (FEV1) was found in 15 patients (30%). A forced expiratory volume in 1 second to forced vital capacity (FEV1/FVC) ratio of >80% was found in all patients. The peak expiratory flow (PEF) was decreased in 23 patients (46.9%). The forced mid-expiratory flow between 25% and 75% of the exhaled vital capacity (MEF25%-75%) was decreased in 5 patients (10%). FVC and FEV1 values in patients with a high ferritin level (>2500 ng/dL) were decreased compared with patients with a low ferritin level (<2500 ng/dL) (P=0.04, 0.03). FVC, FEV1, and PEF parameters were negatively correlated with the age and the body surface area. Age was a predictor of FVC (&bgr;=−0.450, P<0.001), FEV1 (&bgr;=−0.419, P<0.001), and PEF (&bgr;=−0.505, P<0.001), and hemoglobin was a predictor of FEV1/FVC (&bgr;=0.366, P=0.01) and MEF25%-75% (&bgr;=0.323, P=0.003). Conclusions: Our results concluded that the respiratory system should be evaluated by PFTs even in asymptomatic patients with high serum ferritin levels during the adolescent period annually to prevent the squeal of pulmonary disease in TM. Patients who have abnormal PFTs should be reevaluated for compliance with chelation therapy and the transfusion program.

The management of bilateral Wilms tumor

Wilms tumor (WT) is the most common malignant renal tumor in childhood. Approximately 5-7% of WT patients present with bilateral disease, either synchronously or metachronously. Bilateral WT usually occurs in younger children and more often in girls. Management of a child with bilateral WT is very challenging. In contrast to unilateral WT, there has not been uniform agreement about the therapeutic strategy in the management of bilateral WT. As surgery is a critical component in the treatment of WT, the aim is to achieve a high cure rate while maintaining adequate long-term renal function in patients with bilateral WT. In the past, radical surgical procedures which lead to the patients on dialysis have been traditionally recommended in these patients. After several multicentre trials, bilateral biopsies followed by pre-operative chemotherapy and then renal salvage surgery have been recommended. The management of bilateral WT has evolved from primary surgical extirpation to kidney-preserving resection after preoperative chemotherapy. Preoperative chemotherapy often results in significant reduction in tumor size, thereby facilitating subsequent renal salvage. The analysis of children with bilateral WT shows that preservation of renal parenchyma is possible following initial preoperative chemotherapy. Only centers with experience in bilateral WT should treat the cases with bilateral WT to provide optimal treatment.

Primary Ovarian Malignant Melanoma Arising in Teratomatous Component of Mixed- Germ Cell Tumor in a Child: Case report

Primary ovarian malignant melanoma arising in teratomatous component of germ cell tumors is seen extremely rare with most reports being only of single cases and small series in reproductive aged woman and mostly from cystic teratoma, whereas information on pediatric presentation is sparse. This case is reported for being extremely rare tumor.

Chronic lymphocytic leukemia in a child: a challenging diagnosis in pediatric oncology practice.

Chronic lymphocytic leukemia/lymphoma (CLL) is an extremely rare disease during childhood. We report a 16‐year‐old female who presented with lymphadenopathies and she was diagnosed as T cell lymphoblastic lymphoma. Her chemotherapy response was minimal and clinical findings were unusual. Therefore, her biopsy specimen was re‐examined and diagnosis was changed to CLL. Chemotherapy protocol including fludarabine, cyclophosphamide, rituximab was administrated and good response was observed. In our patient deletion at 1q21.2 region that includes aryl hydrocarbon receptor nuclear translocator (ARNT) gene was detected via comparative genomic hybridization method. ARNT gene deletion may be a new mutation in chronic lymphocytic leukemia development. Pediatr Blood Cancer 2014;61:933–935.

Pulmonary arterial hypertension in a child with stage-IV neuroblastoma after autologous hematopoietic stem cell transplantation and review of the literature.

PH is a rare condition with high mortality rate after pediatric HSCT. As clinical presentation is non‐specific and may mimic other conditions, a high degree of suspicion is required for diagnosis. Here, we present a patient with stage‐IV neuroblastoma who developed PAH after autologous HSCT. After exclusion of other causes of PH, we regarded that this condition was secondary to HSCT.

Gastric Schwannoma Without Neurofibromatosis in a 16-Year-Old Adolescent.

BACKGROUND Schwannomas are benign, slowgrowing, encapsulated nerve-sheath tumors arising from Schwann cells. Gastric schwannomas occur more frequently in female patients in the fifth to the sixth decades of life and account for only 0.2% of all gastric tumors and 4% of all benign gastric neoplasms in adulthood. Schwannomas are rare in children without neurofibromatosis (NF).1–3 We describe a 16-year-old female patient diagnosed with gastric schwannoma.

Teratoid Wilms’ Tumor Exhibiting Extensive Squamous Differentiation

Teratoid Wilms’ tumor is a rare renal tumor. Herein, we report an unusual variant of such tumor which simulated renal teratoma because of abundant keratinized squamous epithelium within the tumor.

Anaplastic lymphoma kinase gene expression in small round cell tumors of childhood--a comparative ımmunohistochemical study.

The focus of this study was to investigate anaplastic lymphoma kinase (ALK) expression by immunohistochemistry using a highly specific antibody. Distribution and frequency of ALK expression may provide a clue for ALK inhibitor use in small round cell tumors of childhood. The study group involved 76 small round cell tumors of childhood, which composed of 11 rhabdomyosarcomas, 13 Wilms tumors, 7 Ewing sarcoma/primitive neuroectodermal tumors, 34 peripheral neuroblastic tumors, and 11 acute lymphoblastic lymphoma. Anaplastic lymphoma kinase protein expression in small round cell tumors of childhood is poorly described in the literature. The findings of our study highlight a potential and possible role of targeting ALK in pediatric solid tumors by using ALK immunohistochemistry. Anaplastic lymphoma kinase may also have an oncogenic role in rhabdomyosarcomas and peripheral neuroblastic tumors, and they may possibly be treated with ALK inhibitors. Anaplastic lymphoma kinase expression in Wilms tumors is not reported in the literature, previously. Our study evaluated ALK expression in Wilms tumor samples.

Successful Desensitization to Etoposide in a Preschool Pediatric Patient

Kulhas Celik I1, Guvenir H1, Buyuktiryaki B1, Dibek Misirlioglu E1, Ozyoruk D2, Toyran M1 1University of Health Sciences, Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital, Division of Pediatric Allergy and Immunology, Ankara, Turkey 2University of Health Sciences, Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital, Division of Pediatric Hematology Oncology, Ankara, Turkey

The Retrospective Evaluation of Our Cervical Lymphadenopathy Cases

Objective: Cervical lymphadenopathy is a common finding in childhood and creates anxiety in the families. It usually results from infections but can also signal a more serious underlying disease so its management and clinical follow-up ranges from observation and comforting the family to aggressive medical and surgical procedures. We aimed to investigate the etiology, and the clinical and laboratory features of cervical lymphadenopathy at the children’s oncology outpatient clinic. Material and Methods: Ninety-four patients with cervical lymphadenopathy that were referred to the children’s oncology clinic from general pediatrics in Sanliurfa Childrens Hospital were retrospectively analyzed between May 2011 and June 2012. Results: The mean age was 5.4±3.7 years. 22 (23.4%) patients were female and 72 (76.6%) were male. Seventeen (18.1%) patients had unilateral and the other 77 (81.9%) cases had bilateral cervical lymph nodes. The long axis diameter of the lymph nodes was > 3 cm in 9 (9.6%) patients and 1 to 3 cm in the other 85 (90.4%) patients. A total of 12 (13%) patients had lymph node excisional biopsy performed. We found seven Hodgkin’s lymphoma, 2 reactive hyperplasia, 1 tuberculosis lymphadenitis, 1 nodular fasciitis and 1 necrotizing lymphadenitis. The other 82 (87%) patients had lymphadenopathy related to nonspecific infections that regressed in 4-6 weeks. Conclusion: We wanted to emphasize the childhood cervical lymphadenopathy is often originated from infectious reasons.