Publications by authors named "Sema Buyukkapu Bay"

21 Publications

  • Page 1 of 1

Childhood Rhabdomyosarcoma of the Female Genital Tract: Association with Pathogenic DICER1 Variation, Clinicopathological Features, and Outcomes.

J Pediatr Adolesc Gynecol 2021 Jan 20. Epub 2021 Jan 20.

ResourcePath, Sterling, Virginia; Department of Pathology, Children's National Health System, Washington, DC; George Washington University School of Medicine and Health Sciences, Washington, DC.

Study Objective: Rhabdomyosarcomas (RMSs) of the female genital tract (FGT) have been recently shown to be associated with germline pathogenic variation in DICER1, which can underlie a tumor predisposition disorder. We sought to determine the incidence of a pathogenic variation in DICER1 in a cohort of RMSs of the FGT, as well as to evaluate the clinicopathological features and outcomes of the patients. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: We retrospectively reviewed medical records of the patients diagnosed with RMS of the FGT between 1990 and 2019. Molecular genetic sequencing of the tumor to detect an RNase IIIb domain hot spot mutation in DICER1 samples was performed in 7 patients. Individuals with a missense mutation in the tumor were also screened for a loss of function germline mutation in DICER1.

Results: Of 210 cases of pediatric RMS, 11 arose from the FGT. Molecular genetic sequencing of the tumor samples revealed a somatic missense mutation in the RNase IIIb domain of DICER1 in a total of 3 patients, 2 patients with embryonal RMS of the cervix/uterus, and 1 patient with ovarian embryonal RMS. As a result of genetic testing for the loss of function germline mutation in DICER1, a heterozygous pathogenic variant was also found in 2 of these patients.

Conclusion: Despite the limited number of patients, our findings suggest that it is important to be aware of the possible association between RMS of FGT and pathogenic germline DICER1 variants because the detection of this mutation in a patient or relatives can provide the opportunity for surveillance of related conditions that might improve long-term outcomes and survival.
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http://dx.doi.org/10.1016/j.jpag.2021.01.011DOI Listing
January 2021

c.1162G > A (p.Gly388Arg) Polymorphism Analysis in Turkish Patients with Retinoblastoma.

J Oncol 2020 30;2020:9401038. Epub 2020 Dec 30.

Istanbul University, Oncology Institute, Department of Basic Oncology, Division of Cancer Genetics, Istanbul, Turkey.

Purpose: Various molecular variations are known to result in different gene variants in the gene, known for its oncogenic transformation activity. The goal of this study was to investigate the p.Gly388Arg variant that plays role in the progression of cancer and retinal growth and may be an effective candidate variant in the Turkish population in retinoblastoma patients with no gene mutation.

Methods: Using the Sanger sequencing methods, the p.Gly388Arg variant was bidirectionally sequenced in 49 patients with non- gene mutation in retinoblastoma patients and 13 healthy first-degree relatives and 146 individuals matched by sex and age in the control group.

Results: In Turkish population-specific study, the p.Gly388Arg variant was found in 27 (55.1 percent) of 49 patients; mutation was found in 7 (53.8 percent) of these patients' 13 healthy relatives screened. When p.Gly388Arg mutation status is evaluated in terms of 146 healthy controls, in 70 (47.9 percent) individuals, mutation was observed. Our analysis showed that the p.Gly388Arg allele frequency, which according to different databases is seen as 30 percent in the general population, is 50 percent common in the Turkish population.

Conclusions: In patients with advanced retinoblastoma who were diagnosed with retinoblastoma prior to 24 months, the p.Gly388Arg allele was found to be significantly higher. As a result, these results indicate that the polymorphism of p.Gly388Arg may play a role in both the development of tumors and the progression of aggressive tumors.
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http://dx.doi.org/10.1155/2020/9401038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7787726PMC
December 2020

Respiratory viral panel testing in children with cancer and respiratory tract infections.

Pediatr Blood Cancer 2021 Mar 15;68(3):e28773. Epub 2020 Oct 15.

Department of Pediatric Hematology-Oncology, Oncology Institute, Istanbul University, Istanbul, Turkey.

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http://dx.doi.org/10.1002/pbc.28773DOI Listing
March 2021

Lymphoma Predisposing Gene in an Extended Family: CD70 Signaling Defect.

J Clin Immunol 2020 08 3;40(6):883-892. Epub 2020 Jul 3.

Aziz Sancar Institute of Experimental Medicine, Genetics Department, Istanbul University, Istanbul, Turkey.

Genome-wide sequencing studies in pediatric cancer cohorts indicate that about 10% of patients have germline mutations within cancer predisposition genes. Within this group, primary immune deficiencies take the priority regarding the vulnerability of the patients to infectious agents and the difficulties of cancer management. On the other hand, early recognition of these diseases may offer specific targeted therapies and hematopoietic stem cell transplantation as an option. Besides therapeutic benefits, early diagnosis will provide genetic counseling for the family members. Within this context, an extended family with multiple consanguineous marriages and affected individuals, who presented with combined immune deficiency (CID) and/or Hodgkin lymphoma phenotype, were examined by exome sequencing. A pathogenic homozygous missense CD70 variation was detected (NM_001252.5:c332C>T) in concordance with CD70 phenotype and familial segregation was confirmed. CD70 variations in patients with CID and malignancy have very rarely been reported. This paper reports extended family with multiple affected members with CID and malignancy carrying a missense CD70 variation, and reviews the rare cases reported in the literature. Primary immune deficiencies appear to be a potential cause for pediatric cancers. Better focusing on these inborn disorders to prevent or make an early diagnosis of malignant transformation and reduce mortalities is important.
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http://dx.doi.org/10.1007/s10875-020-00816-4DOI Listing
August 2020

Children with malignant melanoma: a single center experience from Turkey.

Turk Pediatri Ars 2020 9;55(1):39-45. Epub 2020 Mar 9.

Department of Pediatric Hematology-Oncology, İstanbul University, Oncology Institute, İstanbul, Turkey.

Aim: Malignant melanoma is the most frequent skin cancer in children and adolescents. It comprises 1-3% of all malignancies. In this study, we aimed to evaluate the clinical aspects, histopathologic features, and treatment outcomes of our patients with malignant melanoma.

Material And Methods: Patients aged <15 years who were treated between 2003 and 2018 for malignant melanoma were retrospectively analyzed.

Results: Seventeen patients (10 females, 7 males), with a median age of 7 years (range, 7 months-13 years) were evaluated. Five patients had congenital melanocytic nevi. All had cutaneous melanoma except one with mucosal (conjunctival) melanoma. The most frequent primary tumor site was the lower extremities (35%). Sentinel lymphoscintigraphy, sentinel node biopsy, and PET/CT were performed as the staging procedures at initial diagnosis. Localized disease was present in eight patients; nine had regional lymph node metastasis. The only treatment was surgery in localized disease; surgery and adjuvant interferon treatment was given in patients with regional lymph node metastasis. Three developed distant metastasis (bone, lung, brain) at a median of 9 months. A three-year-old patient received a BRAF inhibitor (vemurafenib), and a 13-year-old patient received a check point inhibitor (ipilimumab); both died of progressive disease. The median follow-up for all patients was 25 months. The 5-year overall survival was 76.6%.

Conclusion: Although malignant melanoma is rare in children, prognosis is good if diagnosed early. Physicians should be aware of skin lesions and full-layer biopsy should be obtained in suspicious skin lesions. Patients with congenital melanocytic nevi should also be followed up cautiously.
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http://dx.doi.org/10.14744/TurkPediatriArs.2019.90022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7096563PMC
March 2020

DICER1-Related Embryonal Rhabdomyosarcoma of the Uterine Corpus in a Prepubertal Girl.

J Pediatr Adolesc Gynecol 2020 Apr 12;33(2):173-176. Epub 2019 Dec 12.

Department of Pathology, Children's National Health System, Washington, DC; George Washington University School of Medicine and Health Sciences, Washington, DC; ResourcePath LLC, Sterling, Virginia.

Background: Germline DICER1 mutations increase the risk of developing a wide variety of generally uncommon tumors. We describe a case of DICER1-related embryonal rhabdomyosarcoma (ERMS) of the uterine corpus in a prepubertal girl.

Case: A 10-year-old- girl with a history of cystic nephroma presented with a 3-week history of vaginal bleeding. A 3-cm mass filling the uterine cavity was detected, and histopathologic examination of hysteroscopy-guided biopsy samples revealed ERMS. Molecular genetic sequencing of the tumor sample revealed a DICER1 mutation.

Summary And Conclusion: This report highlights the importance of screening for DICER1 mutations in the presence of the early-onset features of this syndrome, and extends the spectrum of DICER1-related tumors by showing the mutation in a case of ERMS of the uterine corpus.
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http://dx.doi.org/10.1016/j.jpag.2019.12.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7459144PMC
April 2020

Investigation of new candidate genes in retinoblastoma using the TruSight One "clinical exome" gene panel.

Mol Genet Genomic Med 2019 08 17;7(8):e785. Epub 2019 Jun 17.

Division of Cancer Genetics, Department of Basic Oncology, Oncology Institute, Istanbul University, Istanbul, Turkey.

Background: Retinoblastoma (Rb) is the most prevalent intraocular pediatric malignancy of the retina. Significant genetic factors are known to have a role in the development of Rb.

Methods: Here, we report the mutation status of 4813 clinically significant genes in six patients with noncarrier of RB1 gene mutation and having normal RB1 promoter methylation from three families having higher risk for developing Rb in the study.

Results: A total of 27 variants were detected in the study. Heterozygous missense variants c.1162G > A (p.Gly388Arg) in the FGFR4 gene; c.559C > T (p.Pro187Ser) in the NQO1 gene were identified. The family based evaluation of the variants showed that the variant, c.714T > G (p.Tyr238Ter), in the CLEC7A gene in first family; the variant, c.55C > T (p.Arg19Ter), in the APOC3 gene and the variant, c.1171C > T (p.Gln391Ter), in the MUTYH gene in second family; and the variant, c.211G > A (p.Gly71Arg), in the UGT1A1 gene in the third family, were found statistically significant (p < 0.05).

Conclusion: This study might be an important report on emphazing the mutational status of other genes in patients without RB1 gene mutations and having high risk for developing Rb. The study also indicates the interaction between the retinoic acid pathway and Rb oncogenesis for the first time.
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http://dx.doi.org/10.1002/mgg3.785DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6687622PMC
August 2019

RE: Osteosarcoma of the rib: a rare presentation.

Turk Pediatri Ars 2018 Dec 1;53(4):270. Epub 2018 Dec 1.

Istanbul University, Oncology Institute, Department of Radiation Oncology, Istanbul, Turkey.

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http://dx.doi.org/10.5152/TurkPediatriArs.2016.48048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6408185PMC
December 2018

Nimotuzumab-containing regimen for pediatric diffuse intrinsic pontine gliomas: a retrospective multicenter study and review of the literature.

Childs Nerv Syst 2019 01 11;35(1):83-89. Epub 2018 Nov 11.

Oncology Institute, Istanbul University, Istanbul, Turkey.

Purpose: Nimotuzumab is an IgG1 antibody that targets epidermal growth factor receptor (EGFR). Overexpression of EGFR is detected in some pediatric brain tumors including diffuse intrinsic pontine gliomas (DIPG)s.

Methods: Since May 2010, nimotuzumab, combined with carboplatin or vinorelbine or Temozolomide (TMZ), was administered during progressive disease (PD) after the use of the institutional protocol consisting of radiotherapy (RT) + TMZ and adjuvant TMZ. After May 2012, children with newly diagnosed disease received TMZ during RT, and nimotuzumab and TMZ after RT. Nimotuzumab was given as 150 mg/m/dose once a week for 12 weeks, and then every other week with TMZ until PD. PD patients were switched to nimotuzumab + vinorelbine combination until death.

Results: Nimotuzumab was used in 24 children with DIPG (seven in the PD group, 17 in the newly diagnosed patient group). In the PD group, median survival time was 12 months (7-42 months); 1-year and 2-year overall survival (OS) rates were 42.9 ± 18% and 14.3 ± 13%, respectively. The median survival in this group, after the initiation of nimotuzumab was 6 months (3-8 months). In the newly diagnosed patient group, median survival time was 11 months (3-35 months) and median progression free survival was 4 months (1-21 months). The 1-year OS in this group was 35.3 ± 11% and 2 year OS was 11.8 ± 7%. Nimotuzumab ± chemotherapy was well tolerated with no major adverse effect.

Conclusion: Nimotuzumab-containing regimens are feasible and tolerable; it might be that some patients either with newly diagnosed DIPG or with progressive disease may benefit modestly from nimotuzumab-containing combinations.
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http://dx.doi.org/10.1007/s00381-018-4001-9DOI Listing
January 2019

Cutaneous adverse event associated with vemurafenib in a 3-year-old pediatric patient with BRAF mutation-positive metastatic melanoma and factor X deficiency.

Melanoma Res 2019 02;29(1):99-101

Division of Pediatric Hematology-Oncology, Oncology Institute, Istanbul University.

Malignant melanoma is very rare in childhood. The approach to diagnosis and treatment in children has been adopted from adult guidelines. Vemurafenib is indicated in adults with BRAF V600 mutation-positive stage IIIc/IV melanoma and causes cutaneous adverse events. We report on a 3-year-old child with recurrent, metastatic (bone) BRAF mutation-positive melanoma. He also had severe factor X deficiency. Four days after vemurafenib treatment, bilateral palpebral edema and violet-colored hyperpigmentation were observed. There was no objective response to vemurafenib; however, bone pain regressed slightly. Our patient is the youngest patient who received vemurafenib for BRAF V600 mutation-positive metastatic melanoma in the literature.
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http://dx.doi.org/10.1097/CMR.0000000000000537DOI Listing
February 2019

Primary Rhabdomyosarcoma of the Breast: Imaging Findings and Literature Review.

Breast Care (Basel) 2018 Aug 22;13(4):293-297. Epub 2018 May 22.

Pediatric Radiology Division, Radiology Department, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.

Background: Primary breast rhabdomyosarcoma (RMS) can occur in children. There is a lack of knowledge regarding radiologic findings and added diffusion-weighted magnetic resonance imaging (MRI) features of RMS in the literature.

Case Report: A 12-year-old girl was diagnosed with primary alveolar RMS of the breast. Gray scale ultrasound revealed posterior acoustic enhancement behind a well-circumscribed, multilobulated hypoechoic mass. Doppler ultrasound revealed increased peripheral and central vascularity. Hypointense septations on T2-weighted image exhibiting more enhancement than the stroma on late gadolinium-enhanced images were striking within a hyperintense mass. A hyperintense hemorrhagic focus on T1-weighted image was present in the absence of any necrosis. Avid enhancement on early postcontrast images proceeding from the periphery to the center was depicted.

Conclusion: A rapidly enlarging mass with an echogenic peripheral rim together with posterior acoustic enhancement on gray scale ultrasound, intense vascularity on Doppler ultrasound, axillary lymphadenopathy, and satellite nodules on MRI should raise suspicion. Enhancing central and peripheral septations are suggestive of RMS. Dynamic contrast-enhanced MRI in suspected cases can provide valuable data in the differential diagnosis.
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http://dx.doi.org/10.1159/000487750DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6167649PMC
August 2018

Osteosarcoma of the rib: A rare presentation.

Turk Pediatri Ars 2018 Mar 1;53(1):57-60. Epub 2018 Mar 1.

Istanbul University, Oncology Institute, Department of Radiation Oncology, Istanbul, Turkey.

In children and adolescents with chest pain and dyspnea, pneumonia, pleural effusion, and empyema are the frequent causes in the differential diagnosis. Malignant tumors of the chest wall are rare and most originate from the ribs. In children, the most frequent malignant tumor of the rib is Ewing's sarcoma. Osteosarcomas of the rib are very rare. Osteosarcoma has a predilection for rapidly growing long bones including the femur, tibia and humerus in adolescents. In this paper, we present an adolescent girl who presented with chest pain and dyspnea with osteosarcoma that originated from the rib and extended to the right hemithorax.
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http://dx.doi.org/10.5152/TurkPediatriArs.2018.4689DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6070218PMC
March 2018

Vincristine, irinotecan, and temozolomide treatment for refractory/relapsed pediatric solid tumors: A single center experience.

J Oncol Pharm Pract 2019 Sep 6;25(6):1343-1348. Epub 2018 Aug 6.

4 Division of Pediatric Hematology-Oncology, Department of Pediatrics, Bezmialem Vakif University, Istanbul, Turkey.

Background: Although the survival of pediatric cancer has increased dramatically in the last decades, the survival of refractory, relapsed, and metastatic cases is still dismal. The combination of irinotecan and temozolomide has shown activity against refractory/relapsed pediatric solid tumors.

Method: Thirty-four children with refractory/relapsed solid tumors who had previously been heavily pretreated and who were given vincristine, irinotecan, and temozolomide as third- or further line chemotherapy during 2004-2015 were evaluated.

Results: Patients were diagnosed with Ewing sarcoma (n = 15), rhabdomyosarcoma (n = 8), neuroblastoma (n = 8), osteosarcoma (n = 2), and Wilms' tumor (n = 1). Thirty patients presented with disease progression on therapy and the other four presented with relapsing. A total of 141 cycles were administered. Radiotherapy was used in 17 patients and surgery in 4 as local therapy. Among all patients, 6 had complete response, 3 had partial response, 14 had stable disease, and 11 had progressive disease. The objective response was 26.4% (complete response + partial response) and median survival duration was six months. The first and second year overall survival rates were 22.3% and 16.8%. The objective response in Ewing sarcoma patients was 40%. Diarrhea was the most common toxicity and 14 (10%) courses were associated with grade 3-4 diarrhea.

Conclusions: In heavily pretreated patients with refractory/relapsed solid tumors, the vincristine, irinotecan, and temozolomide regimen seemed promising in Ewing sarcoma patients and was well tolerated.
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http://dx.doi.org/10.1177/1078155218790798DOI Listing
September 2019

Primary Osteosarcoma of the Rib: Distribution of Sex.

J Pediatr Hematol Oncol 2018 08;40(6):490-491

Department of Pediatrics, Division of Pediatric Hematology-Oncology, Bezmialem Vakif University, Istanbul, Turkey.

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http://dx.doi.org/10.1097/MPH.0000000000001126DOI Listing
August 2018

Respiratory viral infection`s frequency and clinical outcome in symptomatic children with cancer: A single center experience from a middle-income country.

Turk J Pediatr 2018 ;60(6):653-659

Department of Microbiology and Clinical Microbiology, Istanbul University Faculty of Medicine, Istanbul, Turkey.

Büyükkapu-Bay S, Kebudi R, Görgün Ö, Meşe S, Zülfikar B, Badur S. Respiratory viral infection`s frequency and clinical outcome in symptomatic children with cancer: A single center experience from a middle-income country. Turk J Pediatr 2018; 60: 653-659. In developing countries, acute respiratory tract infections are a significant cause of morbidity and mortality in children, particularly in pediatric cancer patients. A majority of these illnesses are precipitated by viral infections. In our country, studies were conducted on the single respiratory viral infection in a pediatric hematology-oncology unit; however, the analysis of respiratory viral infections in children with cancer is lacking. The present study aimed to provide analysis of multiple respiratory viral infections and clinical outcome in children with cancer who receive chemotherapy and show signs and symptoms of respiratory tract infections. During January, 2014 and January, 2015 children with cancer under treatment who presented with respiratory tract infections were assessed for viruses by using multiplex real-time reverse transcription polymerase chain reaction (rRT-PCR). Specimens were collected by nasal swabbing at in-patient and out-patient clinics. Overall, 72 samples of respiratory tract infection episodes, collected from children with cancer were evaluated with the simultaneous detection of 20 respiratory viruses. A respiratory viral pathogen was obtained in 56.9% samples. Rhinovirus (24.3%) and co-infection with two viruses (19.5%) were the most frequently isolated pathogens. There were four (9.6%) samples of severe pneumonia. Patients with febrile neutropenic episodes and pneumonia were hospitalized and treated with broad-spectrum antibiotics. Other non-neutropenic and mild respiratory tract infections were treated with supportive care as outpatient procedures. There were no deaths. Because there are no effective antiviral agents for certain respiratory viruses, infection control and early diagnosis are crucial in preventing the spread of infection. Clinical findings and serological results of viral respiratory tract infections help us to accurately determine the treatment approach and avoid the unnecessary use of antibiotics.
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http://dx.doi.org/10.24953/turkjped.2018.06.005DOI Listing
January 2018

Respiratory Viral Infections and Coinfections Caused by Human Metapneumovirus in Children With Cancer.

Pediatr Infect Dis J 2017 09;36(9):917

Department of Pediatric Hematology-Oncology, Istanbul University, Oncology Institute Pediatric Hematology-Oncology, Istanbul, Turkey Department of Pediatric Hematology-Oncology, Istanbul University, Cerrahpasa Medical Faculty and Oncology Institute Pediatric Hematology-Oncology, Istanbul, Turkey Department of Microbiology and Clinical Microbiology, Istanbul University Faculty of Medicine, Istanbul, Turkey.

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http://dx.doi.org/10.1097/INF.0000000000001624DOI Listing
September 2017

Osteosarcoma After Hematopoietic Stem Cell Transplantation in Children and Adolescents: Case Report and Review of the Literature.

Pediatr Blood Cancer 2016 09 17;63(9):1664-6. Epub 2016 May 17.

Department of Pathology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.

Osteosarcoma as a secondary malignancy after hematopoietic stem cell transplantation (HSCT) is very rare. We present a case and review of 18 other cases reported to date. Our patient underwent HSCT for myelodysplastic syndrome at the age of 4 years. She developed osteosarcoma 13 years later. She underwent surgery after three courses of neoadjuvant chemotherapy followed by chemotherapy and mifamurtide. She has no evidence of disease 28 months after termination of chemotherapy. In 18 other cases of secondary osteosarcoma in the literature, 15 had received total body irradiation, eight had received alkylating agents, and six had received etoposide. The median interval from HSCT to the onset of osteosarcoma was 6.5 years (range 2.5-15.3), which confirms that children undergoing HSCT should be followed up for many years. In conclusion, osteosarcoma must be included in the differential diagnosis among solid tumors that may develop following HSCT.
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http://dx.doi.org/10.1002/pbc.26067DOI Listing
September 2016

Prognostic factors and treatment results of pediatric Hodgkin's lymphoma: A single center experience.

Turk J Pediatr 2015 Jul-Aug;57(4):359-66

Division of Pediatric Hematology-Oncology, Oncology Institute, Istanbul University, Istanbul, Turkey.

The aim of this study was to assess the demographic, clinic data, prognostic factors and treatment/follow-up results of children who were diagnosed with Hodgkin lymphoma and followed in our center of Pediatric Oncology, Kocaeli University Medical Faculty, Kocaeli, Turkey, for 10 years. This retrospective study evaluated 41 patients with Hodgkin lymphoma who were younger than 18 years-old. All patients were treated with risked adapted ABVD (Adriamycin, Bleomycin, Vincristine, Dacarbazine) chemotherapy and also received involved field radiotherapy. Thirty-two patients (78%) were males and 9 (22%) were females, with a mean age of 10.7±4.0 years. The histopathological diagnosis was mixed cellular type in 51.2% of the patients. B symptoms (unexplained fever, unexplained weight loss, drenching night sweats) were present in 53.7% of the patients and 36.6% of the patients were at advanced stage at the time of the diagnosis. The 3-year overall and event-free survival rates were 88% and 5-year overall and event-free survival rates were 88%, 78%. Age, stage, treatment risk groups, presence of B symptoms and hematological parameters had no significant effect on overall and event-free survival in univariate analysis while bulky disease was the only significant factor on overall survival. Our treatment policy was succesful regarding the similar survival rates in the treatment risk groups, however novel treatment strategies adopting the early response with the reduction of adverse effects are planned in the near future.
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February 2017

Concomitant meningioma and glioma within the same optic nerve in neurofibromatosis type 1.

J Child Neurol 2014 Mar 17;29(3):385-8. Epub 2013 Feb 17.

1Department of Pediatrics, Kocaeli University School of Medicine, Kocaeli, Turkey.

A patient with neurofibromatosis type 1 and the rare finding of concomitant meningioma and optic pathway glioma within the same optic nerve is presented. A 4-year-old boy was admitted to our hospital with right-sided proptosis. He also had numerous café-au-lait macules and axillary freckling on physical exam. According to National Institutes of Health (NIH) criteria, he met the diagnostic criteria for neurofibromatosis type 1. On magnetic resonance imaging (MRI), a mass originating from the right optic nerve sheath with normal appearance of the optic nerve was observed, which was consistent with optic nerve sheath meningioma. Another mass lesion was observed in the prechiasmatic region of the same optic nerve, which was consistent with optic nerve glioma. Two different types of optic pathway tumors in the same optic nerve is an extraordinary case. It is important to recognize imaging findings of these tumors and make correct diagnosis.
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http://dx.doi.org/10.1177/0883073812475157DOI Listing
March 2014

Mediastinal Hodgkin lymphoma arising from cystic lymphangioma: case report in a child.

Turk J Pediatr 2012 May-Jun;54(3):298-300

Division of Pediatric Oncology, Department of Pediatrics, Kocaeli University Faculty of Medicine, Kocaeli, Turkey.

Mediastinal disease is a frequent clinical presentation in children with Hodgkin lymphoma. It is usually due to mediastinal lymphadenopathy or involvement of the thymus gland. Cystic lymphangiomas are benign tumors of the lymphatic system, and less than 1% present as a solitary mediastinal mass. To our knowledge, there has been no report in the literature describing Hodgkin lymphoma arising from cystic lymphangioma. In this report, we describe a patient with mediastinal cystic lymphangioma, from which Hodgkin lymphoma was determined to have arisen.
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December 2012

Preliminary results of propranolol treatment for patients with infantile hemangioma.

Turk J Pediatr 2011 Mar-Apr;53(2):137-41

Department of Pediatrics, Kocaeli University Faculty of Medicine, Kocaeli, Turkey.

Propranolol, a non-selective beta-blocker, has recently been introduced as a treatment for infantile hemangiomas. In this study, we evaluated the effect of propranolol in 12 infants with hemangioma. Twelve infants (9 girls) with a median age of 4.5 months were included in the study. All of the patients in the study group received short-term (1-9 weeks, median: 4 weeks) systemic corticosteroids as a first-line therapy. All patients received propranolol 2 mg/kg/day, divided into three doses. They were treated in an inpatient setting for the first 72 hours of the treatment. Vital signs, blood pressure and blood glucose were monitored. Propranolol treatment was given for 4-9 months (median: 5 months). In the study group, regression rate of the mean dimension of the lesion was 38% +/- 15 (range 15%-50, median 45%) at the 2nd month of therapy. Over 9 months, which was the maximum follow-up period, the regression rate of the mean dimension of the lesion was 55% +/- 31 (range 20%-80, median 50%). One patient had transient bradycardia, which improved spontaneously. No other side effect was observed in the study population. Propranolol appears to be an effective drug for infantile hemangiomas with good clinical tolerance. We suggest that propranolol is the preferable drug as the first-line therapy for infantile hemangiomas.
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November 2011