Publications by authors named "Canan Akyüz"

143 Publications

Approach to pheochromocytoma and paraganglioma in children and adolescents: A retrospective clinical study from a tertiary care center.

J Pediatr Urol 2021 Feb 4. Epub 2021 Feb 4.

Hacettepe University, Institute of Health Sciences, Department of Medical and Surgical Research, Ankara, Turkey; Hacettepe University, Faculty of Medicine, Department of Pediatric Surgery, Ankara, Turkey.

Aim: Pheochromocytoma (PCC) and paraganglioma (PGL) are rare tumors in childhood. They are catecholamine secreting tumors and present with signs or symptoms related to their excess. Most common signs and symptoms are hypertension, headache and diaphoresis. The management of children usually depend on experience of adulthood. This study is conducted to present the clinical characteristics, surgical management and outcome of childhood PCC and PGL in a tertiary care center.

Material And Methods: We reviewed clinical records of all patients operated for PCC and PGL between 2000 and 2020 retrospectively.

Results: There were 18 children operated for PCC and PGL in the study period. The female to male ratio was 1:1. The median age at diagnosis was 13 (IQR, 9-15) years. The most common presenting symptoms were headache and diaphoresis. Hypertension was the most common sign. Three patients had von Hippel-Lindau (VHL). Tumors of two patients with VHL were detected during routine follow-up. Three patients had multifocal disease. Medical preparation for surgery was carried out in all patients. Antihypertensive treatments were administered preoperatively. Since the patients are at risk for postoperative hypotension due to chronic vasoconstriction and blood volume contraction, high salt diet was recommended. Intravenous normal saline at a rate of 3000 ml/m body surface area per day was started for intravascular volume expansion preoperatively. The mean duration for preoperative medication to achieve normal blood pressure was 22 days (range, 16-30). Twenty-five tumors were excised in eighteen patients. One patient who had bone metastases on diagnosis and is on IMIBG therapy. The median follow-up time was 5.6 years (range, 1 months - 21 years). Five patients reached adulthood during the study period. Four of these had recurrent metastases (n = 2) and new tumors (pancreatic neuroendocrine tumor, n = 1 and pancreatic neuroendocrine tumor and renal cell carcinoma, n = 1) after the age of 18.

Conclusion: Multidisciplinary approach is necessary to achieve safe surgical treatment and surveillance of PCC and PGL. Detection of associated familial cancer susceptibility syndromes and long-term follow-up is essential to detect late recurrences and new tumors.
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http://dx.doi.org/10.1016/j.jpurol.2021.01.043DOI Listing
February 2021

Adrenocortical tumours in children: a review of surgical management at a tertiary care centre.

ANZ J Surg 2021 Jan 12. Epub 2021 Jan 12.

Department of Medical and Surgical Research, Hacettepe University, Institute of Health Sciences, Ankara, Turkey.

Background: Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management.

Methods: Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively.

Results: The median age of 24 children was 78 months. Fourteen patients had adrenocortical carcinoma, nine had adrenocortical adenoma and one had neuroendocrine differentiation of ACT. Endocrine dysfunction was noted in 79% of the patients. Five patients had preoperative chemotherapy but none had a decrease in tumour size. Transabdominal approach was used in all but two patients who had thoracoabdominal incision for excision of giant tumours and ipsilateral lung metastases. Two patients had visceral excision to achieve R0 resection. Five patients, four of whom had spillage and one with partial resection died of widespread disease. Two patients with stage 4 adrenocortical carcinoma are still on chemotherapy. All patients with stage I-III disease who had total excision without spillage (n = 17) are disease-free for 2-170 months.

Conclusions: Our results show the importance of excision in ACT without spillage for survival. However, multicentre prospective studies should enhance the knowledge of children about ACT and develop alternative therapies for stage III and IV cases.
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http://dx.doi.org/10.1111/ans.16542DOI Listing
January 2021

Serum levels of VEGF and bFGF in infantile hemangiomas treated with propranolol.

Turk J Pediatr 2020 ;62(6):979-985

Department of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Background: Infantile hemangiomas (IH) represent the most common type of benign tumors of infancy. Vascular endothelial growth factor (VEGF) and basic fibroblastic growth factor (bFGF) have a central role in the pathogenesis of infantile hemangiomas.

Methods: In this prospective study, we aimed to investigate the relationship between serum VEGF and bFGF levels and clinical characteristics and the serological changes in VEGF and bFGF levels associated with propranolol treatment in infants diagnosed with IH. Blood samples were taken from 34 patients with IH and 10 controls. Serum VEGF and bFGF levels were studied by ELISA.

Results: At initial diagnosis, median serum bFGF levels were 11.1 ng/ml (4.8-16.6) in patients with IH (n=34) and 2.6 ng/ml (1.7-4.7) in controls (p < 0.001), and, median serum VEGF levels for same groups were 58.5 ng/ml (25.3-190.2) and 11.4 ng/ml (8.2-19.8) (p < 0.001), respectively. Serum VEGF and bFGF levels were not correlated. In 18 infants who were treated with propranolol with serial measurements, median serum bFGF levels were 10.7 ng/ml, 9.8 ng/ml and 10.5 ng/ml (p= 0.8), and median serum VEGF levels were 68.6 ng/ml, 63.5 ng/ml and 45.1 ng/ml (p < 0.001) at initial diagnosis, at first and third months, respectively. Median regression rates of the hemangiomas at the first and third months were -%47.3 and -%58.3 (p < 0.001), respectively.

Conclusions: Serum bFGF levels didn`t change in time. Serum VEGF levels seemed to follow the natural course of IH and might be a marker for follow-up. The contribution of propranolol treatment should also be considered.
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http://dx.doi.org/10.24953/turkjped.2020.06.009DOI Listing
January 2020

Primary intracranial germ cell tumors in children 36-year experience of a single center.

J Cancer Res Ther 2020 Oct-Dec;16(6):1459-1465

Department of Pediatric Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Purpose: Intracranial germ cell tumors (ICGCTs) comprise approximately 0.4%-3% of all brain tumors. In this study, we aim to evaluate clinical characteristics, treatment and outcomes of patients with ICGCT.

Patients And Methods: All patients with ICGCT diagnosed in Hacettepe University's Pediatric Oncology Department between January 1980 and January 2016 were evaluated, retrospectively.

Results: We identified 52 patients (male/female: 2.46) diagnosed with ICGT. Median age was 140 months. The median duration of symptoms was 3 months. Patients with endocrine symptoms were diagnosed later than others (P = 0.028). The primary site was pineal region in 20 patients, nonpineal region in 32 which included six bifocal involvements. Pineal location was more common in boys than girls (P = 0.02). Histopathological diagnosis was germinoma in 28 patients, nongerminomatous malignant germ cell tumors in 14 and immature teratoma in 4. The mean age for germinoma was higher than that of nongerminomatous tumors (P = 0.032). Patients treated with surgery and radiotherapy and chemotherapy. Median follow-up time was 52.5 months. Thirty-six patients were alive for 12-228 months. Relapsed/progressive disease was observed in 11 patients. Nongerminomatous tumors more frequently showed relapse/progression than germinoma (P = 0.06). Five-year overall and event-free survival rates for the whole group were 72.6% and 57.2%, respectively. Overall and event-free survival rates for germinoma were better than malignant nongerminomatous tumors.

Conclusion: Although the ratio of ICGCTs to central nervous system tumors in our series was similar to western countries, some clinical features such as tumor location were similar to cases from East Asian countries. Although similar protocols were used survival rates lower than developed western and eastern developed countries.
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http://dx.doi.org/10.4103/jcrt.JCRT_314_18DOI Listing
December 2020

Primary gastric lymphoma: A report of 16 pediatric cases treated at a single institute and review of the literature.

Pediatr Hematol Oncol 2020 Nov 24;37(8):656-664. Epub 2020 Jul 24.

Department of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Gastrointestinal tract is the most common extranodal site for childhood non-Hodgkin lymphomas (NHLs). However, primary gastric lymphoma (PGL) is very rare. We report our experience with PGL. Between 1972 and 2019, patients with PGL among 1696 NHL cases were evaluated retrospectively. Patient characteristics, treatments, and survival rates were recorded. We also reviewed the cases reported in literature. There were 16 PGL (11 males, five females) cases with a median age of 10 years. Most frequent complaints, similarly to the literature, were pain and vomiting. Hematemesis/melena and anemia were present in 20% of patients. Most common tumor location was antrum. Histopathological subtypes were Burkitt and non-Burkitt B-cell lymphoma in 43.75% and marginal zone lymphoma (MZL) in 6.25% of cases while mucosa-associated lymphoid tissue (MALT) and low-grade lymphomas constitute 15.3% of cases reported in the literature. In our series, Helicobacter pylori (H. pylori) was analyzed in only the case with MZL and found to be positive. However, H. pylori positivity was reported in 75% of the cases in the literature. H. pylori eradication, chemotherapy, and radiotherapy were applied in one, 14, and five patients. Subtotal gastrectomy with gastroduodenostomy/jejunostomy was performed in three patients. Gastrojejunostomy was done without tumor resection in two patients. Nine patients lived without disease for a median of 59 (12-252) months. Five-year EFS and OS were 69.6% and 64.3%, respectively. PGL constitutes 0.94% of our NHL cases. Interestingly, most of the cases in the literature were from Turkey. While adult PGL is mostly MALT lymphoma, most pediatric cases had high-grade histopathology. Although surgery and radiotherapy were applied earlier, chemotherapy alone is sufficient.
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http://dx.doi.org/10.1080/08880018.2020.1779884DOI Listing
November 2020

Primary Paraspinal and Spinal Epidural Non-Hodgkin Lymphoma in Childhood.

J Pediatr Hematol Oncol 2021 04;43(3):e395-e400

Department of Pediatric Oncology, Hacettepe University Faculty of Medicine.

Primary paraspinal/spinal epidural lymphoma (PPSEL) is rare in childhood. Here, we retrospectively evaluated patients with PPSEL treated in our department. We also reviewed the cases reported in the literature. Fifteen of 1354 non-Hodgkin lymphoma cases diagnosed over a 38-year period were PPSEPL. There were 11 male individuals and 4 female individuals with a median age of 13 years. Most common symptoms were pain and limb weakness. Physical examination revealed spinal cord compression in 80% of patients. The most common tumor location was the lumbar region. Histopathologic subtypes were lymphoblastic lymphoma in 6 and Burkitt lymphom in 5 patients. Subtotal or near-total excision of the tumor with laminectomy was performed in 6 patients. Thirteen and 9 patients received chemotherapy and radiotherapy, respectively. Neurologic recovery was observed in 70% of patients. Seven patients were alive without disease at a median of 88 months. Overall and event-free survival rates were 61.7% and 50.1%, respectively. We reviewed clinical features, treatment, and outcome of 69 PPSEL cases reported in the literature. Neurologic recovery and long-term survival was achieved in 66.7% of them. Heterogeneity in diagnostic methods and treatment have made it difficult to establish the prognostic indicators for neurologic outcome and survival. Multicenter prospective studies with more cases are necessary to determine the prognostic factors.
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http://dx.doi.org/10.1097/MPH.0000000000001858DOI Listing
April 2021

Hepatocyte-specific contrast-enhanced MRI findings of focal nodular hyperplasia-like nodules in the liver following chemotherapy in pediatric cancer patients.

Diagn Interv Radiol 2020 Jul;26(4):370-376

Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey.

Purpose: We aimed to assess the MRI findings and follow-up of multiple focal nodular hyperplasia (FNH)- like lesions in pediatric cancer patients diagnosed by imaging findings.

Methods: We retrospectively analyzed clinical data and MRI examinations of 16 pediatric patients, who had been scanned using gadoxetate disodium (n=13) and gadobenate dimeglumine (n=3). Hepatic nodules were reviewed according to their number, size, contour, T1- and T2-weighted signal intensities, arterial, portal, delayed and hepatobiliary phase enhancement patterns. Follow-up images were evaluated for nodule size, number, and appearance.

Results: All 16 patients received chemotherapy in due course. Time interval between the initial diagnosis of cancer and detection of the hepatic nodule was 2-14 years. Three patients had a single lesion, 13 patients had multiple nodules. The median size of the largest nodules was 19.5 mm (range, 8-41 mm). Among 16 patients that received hepatocyte-specific agents, FNH-like nodules appeared hyperintense in 11 and isointense in 5 on the hepatobiliary phase. During follow-up, increased number and size of the nodules were seen in 4 patients. The nodules showed growth between 6-15 mm.

Conclusion: Liver MRI using hepatocyte-specific agents is a significant imaging method for the diagnosis of FNH-like lesions, which can occur in a variety of diseases. Lesions can increase in size and number in pediatric patients.
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http://dx.doi.org/10.5152/dir.2019.19398DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7360070PMC
July 2020

Comparison of intravitreal melphalan with and without topotecan in the management of vitreous disease in retinoblastoma.

Jpn J Ophthalmol 2020 Jul 23;64(4):351-358. Epub 2020 May 23.

Department of Pediatric Oncology, Hacettepe University School of Medicine, Ankara, Turkey.

Purpose: To evaluate clinical outcomes and enucleation rates after intravitreal melphalan (IVM) alone and after IVM combined with intravitreal topotecan (IVT) for the treatment of vitreous disease, and to a lesser extent subretinal and retrohyaloid seeds, in patients with retinoblastoma.

Study Design: A retrospective analysis of 77 eyes of 72 consecutive patients.

Methods: Demographic data, classification of tumors, seed type (dust, sphere or cloud) before injection and at the end of follow-up, injection type (IVM or IVM+IVT), doses of IVM and IVT, number of injections, follow-up time, enucleation status and side effects were recorded. Cox regression analysis and log-rank test for Kaplan-Meier curves were performed.

Results: Of 77 eyes, 40 received IVM alone (group 1) and 37 received IVM+IVT (group 2). Enucleation rates were 62.5% (n=25) in group 1 and 10.8% (n=4) in group 2 (p=0.001). Median eye survival was 23.6 months in group 1 and 25.6 months in group 2. Mantel-Cox test revealed statistically significant differences between Kaplan-Meier curves of group 1 and 2 (p=0.022). Multiple Cox regression analysis showed a significantly elevated enucleation rate associated with: IVM only treatment group (p=0.019) and pre-injection cloud type of seeding (p=0.014).

Conclusion: The combined use of intravitreal melphalan and topotecan provides significantly better results in terms of avoiding enucleation and vitreal and subretinal seed control.
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http://dx.doi.org/10.1007/s10384-020-00743-2DOI Listing
July 2020

Cardiac complications in patients who underwent to hematopoietic stem cell transplantation.

J Cancer Res Ther 2020 Jan-Mar;16(1):53-59

Department of Pediatrics, Division of Pediatric Oncology, Hacettepe University Faculty of Medicine and Cancer Institute, Ankara, Turkey.

Aim Of Study: Cardiac complications may be observed after hematopoietic stem cell transplantation (HSCT). Despite significant improvement in supportive care, HSCT may be associated with significant morbidity and mortality. In this study, the aim was to evaluate the frequency of clinically serious cardiac complications after HSCT in our patients.

Materials And Methods: This is a retrospective study. Cardiac complications were analyzed in 75 patients undergone to HSCT with physical examination, electrocardiography, echocardiography, and cardiac monitorization.

Results: The median age was 12 years (range 11-16) and M/F ratio was 2/3 = 0.66. There are five patients with six complications among the retrospective cohort of 75. These were pericardial effusion in three patients, and sinus bradycardia in two patients and ventricular tachycardia in one patient. The incidence of cardiac complications among 75 patients with HSCT was 6.7%. The mortality rates of patients with and without cardiac complication were 40% and 34%, and both of them with cardiac complication had pericardial effusion. However, the cardiac complication was not found statistically significant factor on survival ( P = 0.82).

Conclusion: Poor risk factors of patients, myocarditis, pericarditis, and heart failure owing to cumulative doses of anthracycline, cyclophosphamide, cytomegalovirus infection or other infections, mediastinal irradiation, and cryopreserved stem cell product with dimethyl sulfoxide may be effective on the development of cardiac complications individually. Early intervention can prevent death related to this complication.
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http://dx.doi.org/10.4103/jcrt.JCRT_314_17DOI Listing
October 2020

Ewing sarcoma in an infant and review of the literature.

Turk J Pediatr 2019 ;61(5):760-764

Departments of Radiation Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Bedük Esen ÇS, Gültekin M, Aydın GB, Akyüz C, Karlı Oğuz K, Orhan D, Cengiz M, Gürkaynak M, Yıldız F. Ewing sarcoma in an infant and review of the literature. Turk J Pediatr 2019; 61: 760-764. Ewing sarcoma (ES) is a rare tumor in infants and prognosis is controversial. There are no standard recommendations for treatment in such very young patients. Generally, radiotherapy (RT) is not a part of treatment in infants due to the risk of severe late side effects. In this case report, we report a 7-month-old boy with diagnosis of left mastoid bone ES with lung metastases at diagnosis, showing a rapidly fatal outcome despite aggressive systemic chemotherapy and RT without surgery.
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http://dx.doi.org/10.24953/turkjped.2019.05.016DOI Listing
August 2020

Investigation of the effect of task-orientated rehabilitation program on motor skills of children with childhood cancer: a randomized-controlled trial.

Int J Rehabil Res 2020 Jun;43(2):167-174

Department of Pediatric Oncology, Hacettepe University, Ankara, Turkey.

Problems have been reported in the execution and development of motor skills and its treatment as a cause of cancer in children. The purpose of this study was to examine the effect of the task-orientated rehabilitation program (ToRP) on motor skills of children with childhood cancer. Following the consort guideline, 93 children (49 males and 44 females) with pediatric cancer were randomized to either study (n = 52; 12.35 ± 3.43 years) or control (n = 41; 11.89 ± 3.56 years) groups. The study group received ToRP, and the control group received a home-based therapeutic strategies program for 20 sessions. Motor skill outcomes were assessed with Bruininks-Oseretsky Test of Motor Proficiency, Short Form (BOTMP-SF) by assessor who was blind to group allocation and interventions. The groups were homogenous in terms of demographic characteristics and motor skills. The study group showed a significant increase in both gross and fine motor skills (P values for all subtests: P < 0.001), whereas running speed, bilateral coordination, strength, response speed and visual motor control did not show significant improvement with the control group (P > 0.05). The ToRP appears to provide beneficial effects in improving motor skills at the early stages of rehabilitation for children with childhood cancer.
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http://dx.doi.org/10.1097/MRR.0000000000000400DOI Listing
June 2020

Coexistence or a related condition: an infant with retinoblastoma and Gaucher disease.

Turk J Pediatr 2019 ;61(3):449-452

Departments of Pediatric Gastroenterology, Hepatology and Nutrition Hacettepe University Faculty of Medicine, Ankara, Turkey.

Berberoğlu-Ateş B, Varan A, Demir H, Akyüz C, Yüce A. Coexistence or a related condition: an infant with retinoblastoma and Gaucher disease. Turk J Pediatr 2019; 61: 449-452. Gaucher disease (GD) is the most prevalant lysosomal lipid storage disease that results from loss of function of acid β-glucosidase due to mutations in the glucocerebrosidase gene. Common features of all types of GD include hepatosplenomegaly, cytopenia, and various patterns of bone and lung involvement. Retinoblastoma is a malignant tumor of the developing retina that occurs in children, typically before the age of five. Retinoblastoma develops from cells that have cancer-predisposing variants in both copies of RB1. The association between GD and retinoblastoma has not been reported until now. Here we report the case that was diagnosed with, retinoblastoma at the age of 2 months and then GD at the age of 11 months. Although there are controversies concerning the association between GD and cancer; malignancies should be kept in mind during GD patients follow up.
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http://dx.doi.org/10.24953/turkjped.2019.03.022DOI Listing
July 2020

Outcome of 102 patients under 5 years of age with Hodgkin lymphoma.

Arch Argent Pediatr 2019 10;117(5):e459-e465

Department of Pediatric Oncology, Hacettepe University, Cancer Institute, Ankara, Turkey.

Background: Hodgkin's lymphoma (HL) is one of the most curable pediatric cancers, however it is rare among children under five years of age and prognostic factors for survival rate are still unknown due to low frequency in this age group.

Objectives: The aim of this study was to evaluate clinical characteristics, treatment regimens, and outcome of patients under five years of age with HL.

Methods: Patients diagnosed with HL between 1972 and 2013 were retrospectively evaluated. All patients were treated with chemotherapy with or without radiotherapy.

Results: There were 102 patients with a median age of 4 years (range: 2 to 4.9). The median followup time was 13 years. Twenty-three patients had B symptoms, 15 patients had 'bulky disease' and the most common stages were stage I and II. Overall survival (OS) rates were significantly different according to the stage of the cancer (p = 0.008). Although there were no statistically significant differences; the positivity of 'bulky disease' and B symptoms were associated with poor prognosis.

Conclusion: Our single-center study included the largest number of patients under five years of age with HL. The stage was the main predictor for OS; on the other hand, the presence of B symptoms and bulky disease has also affected the prognosis.
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http://dx.doi.org/10.5546/aap.2019.eng.e459DOI Listing
October 2019

Bilateral Wilms tumors: Treatment results from a single center.

Turk J Pediatr 2019 ;61(1):44-51

Department of Pediatric Oncology, Hacettepe University Cancer Institute, Ankara, Turkey.

Aydın B, Akyüz C, Yalçın B, Ekinci S, Oğuz B, Akçören Z, Yıldız F, Varan A, Kurucu N, Büyükpamukçu M, Kutluk T. Bilateral Wilms tumors: Treatment results from a single center. Turk J Pediatr 2019; 61: 44-51. The management of bilateral Wilms tumor (BWT) is challenging, particularly due to its presentation at a younger age, rarity, and difficulty for treatment decisions and surgical evaluation comparing to unilateral WT. In this study, the outcome of BWT patients from a single center who were treated by the Turkish Pediatric Oncology Group (TPOG) Wilms Tumor Regimen were retrospectively reviewed. From 1990 to 2016, 30 patients with synchronous BWT were treated with a preoperative chemotherapy of vincristine and actinomycin-D (VA). Chemotherapy was continued until safe nephron sparing surgery (NSS) could be performed for as long as radiological tumor response continued; otherwise, the chemotherapy was intensified by adding doxorubicin (D) alternating with VA every 6 weeks. The median followup of patients was 59 months (4-297 months). The median duration of preoperative chemotherapy was 81 days and ranged between 14 days and 198 days. Preoperative chemotherapy was modified in seven patients (23%) to the VAD regimen. Twenty-two patients (73%) had a radical nephrectomy on the larger tumor and NSS on the contralateral kidney, and 6 patients (20%) had bilateral NSS. Postoperative tumor stages for stage I, II and III were 60%, 22% and 14%, respectively. The 5-year event free survival (EFS) rates were 100%, 90% and 51% for stages I, II and III (p=0.02), respectively. Unfavorable histology and nephrogenic rests were reported in 20% and 20% of patients, respectively. The 5-year overall survival (OS) and EFS rates were 50% and 25%, respectively, in patients with anaplasia, while the same rates were 96% and 96% in patients with favorable histology tumors (p=0.05 and p < 0.001). The 10-year EFS and OS rates for all patients were 82% and 86%, respectively. Our results are comparable with the literature. VA is effective as initial preoperative treatment of BWT and allows for safe resection.
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http://dx.doi.org/10.24953/turkjped.2019.01.008DOI Listing
January 2020

Maternal Pain Management at Home in Children with Cancer: A Turkish Sample.

J Pediatr Nurs 2020 Jan - Feb;50:e99-e106. Epub 2019 Aug 19.

Professor Pediatric Oncology Department, Hacettepe University Institute of Oncology, Hacettepe University, Sıhhiye, Ankara, Turkey.

Purpose: The purpose of the present study was to examine maternal pain management in children with cancer and the associated factors.

Design And Methods: The present work is a descriptive and cross-sectional study. Data for the study were obtained from mothers of children in the age group of 0 to 18 years undergoing treatment for solid tumors in Pediatric Oncology Service and Outpatient Clinics (n = 112). We used a questionnaire on parental pain management practices at home, the knowledge about pain and analgesic drugs, Spielberger State-Trait Anxiety Inventory (STAI), and Pain Catastrophizing Scale (PCS) to collect the data.

Results: Several mothers taking part in the study reported various misconceptions about the assessment of children's pain, analgesic drugs, and usage of limited non-pharmacological methods for managing pain in children with cancer. No significant relationships were found between mothers' pain management practices, knowledge of pain assessment and analgesic drugs, and mothers' and children's sociodemographic characteristics or mothers' pain catastrophizing and anxiety about their own pain.

Conclusions: The findings of the study revealed that the majority of mothers of children with cancer had misconceptions regarding knowledge of pain assessment and analgesic drugs; these misconceptions potentially lead to manage children's pain associated with cancer ineffectively. Findings indicate mothers' information and support needs for children's cancer pain management in the home settings.

Practice Implications: A further understanding of barriers to parental pain management in children with cancer in the home setting will contribute immensely in developing appropriate management practices.
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http://dx.doi.org/10.1016/j.pedn.2019.08.007DOI Listing
November 2020

Bilateral Pulmonary Langerhans's Cell Histiocytosis is Surgical Challenge in Children: A Case Report.

European J Pediatr Surg Rep 2019 Jan 23;7(1):e8-e11. Epub 2019 May 23.

Department of Pediatric Oncology, Hacettepe University, Faculty of Medicine, Ankara, Turkey.

 Pulmonary Langerhans's cell histiocytosis (PLCH) is a rare cause of interstitial lung disease in children and more than half of the cases are bilateral. Persistent respiratory distress due to spontaneous pneumothorax (SP) in bilateral PLCH may refractory to conservative treatment and posed a great challenge to surgical modalities. A 3-year-old boy with SP due to bilateral PLCH is presented to discuss the surgical options of recurrent and refractory PLCH cases in children.  The patient was admitted to the emergency department with severe respiratory distress and SP. After chest tube insertion, biopsy from neck mass revealed Langerhans's cell histiocytosis. Chemotherapy including vinblastine and prednisone was initiated. Due to persistent respiratory difficulty and air leaks, talc pleurodesis and thoracoscopic bullae excision with pleural decortication were performed. Two months after the admission, due to nosocomial infection and severe respiratory distress, extracorporeal membranous oxygenation (ECMO) support was initiated. The patient was died of ECMO complications on 24th day of ECMO.  Despite the use of chemotherapy and surgical excision of cystic lesions, bilateral PLCH in children may have lethal outcome. Other treatment options including respiratory support with ECMO and lung transplantation should be considered as last resort of treatment alternative in persistent cases.
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http://dx.doi.org/10.1055/s-0039-1688771DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6533102PMC
January 2019

Solid pseudopapillary neoplasm of the pancreas in children: Hacettepe experience.

ANZ J Surg 2019 Jun 29;89(6):E236-E240. Epub 2019 Apr 29.

Department of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Background: Solid pseudopapillary neoplasm of the pancreas (SPNP) is mostly seen in young women in the second and third decades of life; it is quite uncommon in children. We aimed to review our institutional experience with SPNP in children.

Methods: Hospital charts of children <18 years of age diagnosed to have SPNP were reviewed for demographic characteristics, presenting symptoms, diagnostic interventions, physical examination findings, radiological data, extent of disease, diagnostic and management strategies and final outcome.

Results: Nineteen cases were diagnosed as SPNP between 1992 and 2017 (female: male, 16:3; median age 13 years, range 8.5-16.5). The most common symptom was abdominal pain. Physical examination was normal in 12/19 cases. Three cases were diagnosed incidentally. The most common tumour localization was the head of the pancreas. Median tumour diameter was 5 cm (1.4-15). One patient had abdominal disseminated disease, and others had localized disease. Surgical interventions were enucleations in nine, distal pancreatectomies in four and total resection in two patients; three underwent no surgery following diagnosis. Only one patient received adjuvant chemotherapy and radiotherapy. One patient died, one was lost to follow-up at 164 months and 17 were under follow-up with no events at a median of 60 months (20-308 months).

Conclusion: In childen, SPNP demonstrates different clinical features. Complete resection is curative in most patients. In children, the optimal surgical strategy for SPNP is still debatable. Instead of radical resections, limited pancreatic resections, such as enucleations, with negative surgical margins should be attempted. For unresectable or recurrent tumours, cisplatin and 5-FU-based chemotherapy might be considered.
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http://dx.doi.org/10.1111/ans.15111DOI Listing
June 2019

Cognitive rehabilitation is advantageous in terms of fatigue and independence in pediatric cancer treatment: a randomized-controlled study.

Int J Rehabil Res 2019 Jun;42(2):145-151

Department of Pediatric Oncology, Faculty of Medicine Sciences, Hacettepe University, Ankara, Turkey.

This study aimed to investigate the effect of task-oriented inpatient cognitive rehabilitation on fatigue perception and independence in daily activities. Forty (22 males, 18 females) children with pediatric cancer were randomized to either study [n = 22; 10.81 (1.33) years] or control [n = 18; 10.16 (1.24) years] groups. The study group received a cognitive rehabilitation intervention in addition to a routine therapy program, and the control group received a routine therapy program for 15 sessions. Cognitive status, fatigue, and daily life activity outcomes were assessed by blinded assessors in before and after interventions in the hospital setting. The study group showed a significant decrease in fatigue (P values for fatigue before, during, and after activity: P < 0.001) and in the control group (P values for fatigue only in during and post activity: P < 0.05). A statistically significant improvement was found in functional independence for the study group in all activities of daily living areas areas (P < 0.001), whereas no differences were found in the control group. Cognitive rehabilitation appears to provide beneficial effects in decreasing fatigue, improving functional status in activities of daily living, and increasing cognitive skills at the early stages in inpatient rehabilitation of pediatric cancer.
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http://dx.doi.org/10.1097/MRR.0000000000000340DOI Listing
June 2019

Radiation for ETMR: Literature review and case series of patients treated with proton therapy.

Clin Transl Radiat Oncol 2019 Feb 7;15:31-37. Epub 2018 Nov 7.

Department of Radiation Oncology, UT MD Anderson Cancer Center, Houston, TX, United States.

Background And Purpose: Embryonal tumors with multilayered rosettes (ETMRs) are aggressive tumors that typically occur in young children. Radiation is often deferred or delayed for these patients due to late effects; proton therapy may mitigate some of these concerns. This study reviews the role of radiation in ETMR and describes initial results with proton therapy.

Materials And Methods: Records of patients with embryonal tumor with abundant neuropil and true rosettes (ETANTR), medulloepithelioma (MEP), and ependymoblastoma (EPL) treated with proton therapy at our institution were retrospectively reviewed. A literature review of cases of CNS ETANTR, MEP, and EPL published since 1990 was also conducted.

Results: Seven patients were treated with proton therapy. Their median age at diagnosis was 33 months (range 10-57 months) and their median age at radiation start was 42 months (range 17-58 months). Their median overall survival (OS) was 16 months (range 8-64 months), with three patients surviving 36 months or longer. Five patients had disease progression prior to starting radiation; all 5 of these patients failed in the tumor bed. A search of the literature identified 204 cases of ETMR with a median OS of 10 months (range 0.03-161 months). Median OS of 18 long-term survivors (≥36 months) in the literature was 77 months (range 37-184 months). Of these 18 long-term survivors, 17 (94%) received radiotherapy as part of their initial treatment; 14 of them were treated with craniospinal irradiation.

Conclusions: Outcomes of patients with ETMR treated with proton therapy are encouraging compared to historical results. Further study of this rare tumor is warranted to better define the role of radiotherapy.
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http://dx.doi.org/10.1016/j.ctro.2018.11.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6297264PMC
February 2019

Cervical Extraneural Lymph Node Metastasis in a Patient with Medulloblastoma.

Pediatr Neurosurg 2019 13;54(1):71-73. Epub 2018 Dec 13.

Department of Pediatric Oncology, Hacettepe University, Ankara, Turkey.

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http://dx.doi.org/10.1159/000494930DOI Listing
April 2019

FAB LMB 96 Regimen for Newly Diagnosed Burkitt Lymphoma in Children: Single-center Experience.

J Pediatr Hematol Oncol 2019 01;41(1):e7-e11

Department of Pediatric Oncology, Hacettepe University Cancer Institute, Ankara, Turkey.

Background/objectives: The outcome of Burkitt lymphoma has improved by over 90%. In this study, demographic characteristics and outcomes of 57 patients with Burkitt lymphoma treated with FAB LMB 96 regimen are presented.

Materials And Methods: Standard intensity arms were selected for all risk groups. Clinical characteristics, response to treatment, and outcome were evaluated.

Results: The median age of 48 boys and 9 girls was 8.2 years. There were 2 (3%), 41 (72%), and 14 (25%) patients in the low, intermediate, and high-risk groups, respectively. Patients were followed-up for a median of 50 months. The 5-year overall survival and event-free survival rates for the study group were 90.8% and 87.4%, respectively. Two patients in the low-risk group are still alive without evidence of disease for 17 and 57 months. The 5-year overall survival rates were 95% and 78% for intermediate and high-risk patients, and the 5-year event-free survival rates were 93% and 62%, respectively. At the end of induction, 58% of patients had complete response, and 42% (n=24) had residual tumors. A total of 16 of 24 patient biopsies showed no viable tumors.

Conclusions: Our results confirm the previously published survival results of FAB LMB 96. The regimen is an effective regimen for mature B-cell non-Hodgkin lymphoma without radiotherapy or surgery even in central nervous system-positive patients. The most important prognostic factor is the complete response after induction chemotherapy. However, residual tumor at the end of consolidation might not always be a sign for unresponsive tumor. Tumoral residue should be radiologically well evaluated and biopsied if possible for viability before intensifying treatment.
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http://dx.doi.org/10.1097/MPH.0000000000001270DOI Listing
January 2019

Retrospective analysis of primarily treated group D retinoblastoma.

Graefes Arch Clin Exp Ophthalmol 2018 Nov 30;256(11):2225-2231. Epub 2018 Jun 30.

Department of Pediatric Oncology, Hacettepe University School of Medicine, Ankara, Turkey.

Purpose: To assess the effectiveness of intra-arterial chemotherapy (IAC), intravenous chemotherapy (IVC), and their combination in the management of group D retinoblastoma.

Methods: This was a retrospective, interventional, comparative, and non-randomized clinical study, including all eyes with primarily treated group D retinoblastoma managed in a single institution from February 2010 to July 2016. Patient demographics, treatment modality (primary enucleation, intravenous chemotherapy, intra-arterial chemotherapy alone or intravenous, and intra-arterial chemotherapy), additional need for consolidation treatments or intravitreal melphalan (IVM) injections, and follow-up time were recorded. The main outcome measure was ocular survival rate after various treatment modalities.

Results: Of 87 eyes of 83 consecutive cases, 9 eyes (10.3%) were primarily enucleated, 37 (42.6%) eyes received IVC, 30 (34.5%) eyes had IAC, and 11 (12.6%) eyes were treated with IVC followed by IAC. After a mean 81.1 ± 75.2 weeks of follow-up, enucleation rates were 56.8% in the IVC group, 23.3% in the IAC group, and 36.6% in the IVC + IAC group (p = 0.021). There was no significant difference of ocular survival estimates among these 3 groups at 2 or 5 years (p = 0.998, p = 0.986). With logistic regression analysis, age; gender; disease laterality; administration of IVM, TTT, or cryotherapy; mean dose of IVM; follow-up time; and number of IAC or IVC cycles were not significantly related to the enucleation rate (p > 0.05 for all variables).

Conclusions: Our experience showed that in group D patients, primary IAC achieved ocular survival in 76.7% of eyes, and less need for local consolidation treatments, whereas following primary IVC ocular survival was 43.2%.
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http://dx.doi.org/10.1007/s00417-018-4051-4DOI Listing
November 2018

Cleft Palate Repair Using Single Flap Palatoplasty in Patient With Associated Palatal Hemangioma.

J Craniofac Surg 2018 Jul;29(5):1332-1333

Department of Plastic Reconstructive and Aesthetic Surgery.

Hemangiomas are the most common tumors of childhood with an average incidence of 10%. It is unusual for hemangiomas, which are already rarely seen isolated on the palate, to coexist with cleft palate as in the authors' patient. Four months old baby was admitted with isolated Veau 2 cleft palate and a red-purple colored raised lesion involving almost the whole right side of the hard palate. Magnetic resonance imaging revealed intensive contrasting solid nodule with a lobulated contour that was covering the right half of the hard palate, measuring 2.2×1.3 cm. To reduce the size of the vascular lesion 7 mg/d oral propranolol treatment was initiated. Following reduction in the size of hemangioma, the cleft palate was repaired at 11 months of age. Single mucoperiosteal flap from the left side of the palate preserving the major palatine artery was elevated whereas right mucoperisteal flap was minimally dissected not to interfere with the hemangioma. No intraoperative and postoperative complications in both the early and late term were experienced. As a result, since early palatal repair is important to obtain ideal speech outcomes in cleft patients, repair should be performed in similar patients with hemangiomas without delaying the timing.
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http://dx.doi.org/10.1097/SCS.0000000000004547DOI Listing
July 2018

A Rare Cause of Secondary Immunodeficiency: Generalized Lymphatic Anomaly.

J Pediatr Hematol Oncol 2018 04;40(3):248-251

Departments of Pediatrics Division of Immunology.

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http://dx.doi.org/10.1097/MPH.0000000000001101DOI Listing
April 2018

ICE Regimen for Relapsed/Refractory Bone and Soft Tissue Sarcomas in Children.

Rev Recent Clin Trials 2018;13(2):126-131

Hacettepe University Cancer Institute, Department of Pediatric Oncology, Ankara, Turkey.

Background: The outcome of bone and soft tissue sarcomas (BST) after relapse has very poor prognosis with survival rates less than 39%. Unfortunately there are not many treatment options, but promising responses have been reported with ifosfamide, etoposide and carboplatin (ICE).

Objective: Therefore, we planned a study for children with recurrent/refractory BST treated with ICE regimen to evaluate their demographic features, responses to treatment and outcome.

Method: Patients with primary diagnosis of BST and treated with ICE regimen at the time of first or subsequent relapse, progression or unresponsive disease were selected for study. The files were retrospectively evaluated.

Results: Thirty of sixty-six patients had metastatic disease at relapse. Patients received median 5 cycles of ICE and were followed-up median 16.3 months. Overall survival rates were 83% and 62% at 1st and 2nd year. The ORR to the regimen was 43%. Survival rates were significantly higher in good responders (3-year EFS and OS rates: 50% 8% and 78% 14%; p<0.0001 and p<0.0001). Survival rates for non-metastatic disease were 42% and 75% for EFS and OS and higher than patients with metastases at relapse (p<0.001 and p<0.0001). EFS and OS rates of patients with rhabdomyosarcoma were 42% and 69%. No grade 3 or 4 renal toxicity was documented.

Conclusion: ICE combination improves the outcome of relapsed or refractory sarcomas and it might serve as second line treatment. Patients with no metastases at relapse would benefit more than others with metastatic disease.
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http://dx.doi.org/10.2174/1574887113666180124114818DOI Listing
December 2018

Denosumab treatment in aneurysmal bone cyst: Evaluation of nine cases.

Pediatr Blood Cancer 2018 Apr 29;65(4). Epub 2017 Dec 29.

Department of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Background: Aneurysmal bone cyst (ABC) is a benign bone tumor. Curettage and bone grafting is the common treatment. Here, we retrospectively evaluate nine patients treated with denosumab.

Procedure: Nine patients with ABC, mostly pelvic and vertebral, treated with denosumab were analyzed retrospectively. A 70 mg/m denosumab dose was used weekly in the first month, and then monthly. Clinical and radiological responses to treatment were evaluated.

Results: In all patients, clinical symptoms including pain and limping regressed completely within 3 months. Radiological evaluation revealed changes in lesion size and content. In six patients, overall volume reduction in the range of 18-82% was detected. Decreases in the size and number of cysts were detected in eight patients. In five patients, fat signal appeared on follow-up imaging. No major side effects were observed during treatment. Median follow-up time after treatment was 15 months. At 5 months, severe hypercalcemia was observed in two patients due to rebound increase in osteoclastic activity. Subsequent to denosumab treatment, three patients underwent surgery for clinical or radiological recurrence.

Conclusions: Our results showed that denosumab provided a meaningful clinical and radiological improvement in ABC. It may be a treatment option, especially in spinal and pelvic tumors with potentially high surgical morbidity. However, late rebound hypercalcemia may restrict its use. Studies with more cases are required for routine use of denosumab in ABC.
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http://dx.doi.org/10.1002/pbc.26926DOI Listing
April 2018

Single institution experience on cancer among adolescents 15-19 years of age.

Turk J Pediatr 2017 ;59(1):1-5

Department of Pediatric Oncology, Hacettepe University Institute of Oncology, Ankara, Turkey.

Yağcı-Küpeli B, Akyüz C, Yalçın B, Varan A, Kutluk T, Büyükpamukçu M. Single institution experience on cancer among adolescents 15-19 years of age. Turk J Pediatr 2017; 59: 1-5. Adolescent cancers differ from other age groups in terms of cancer types, treatment and outcome. We aimed to present our institutional data on survival of certain types of cancer in adolescents. Hospital files were retrospectively evaluated for distribution of tumor types, clinical features, and overall and event-free survival (OS and EFS) rates in adolescents with malignant tumors. Two hundred ninety-three cases between 15-19 years who were diagnosed with malignant tumor at our department in the last 38 years were included in the study. Mean age was 15.3 and median age was 15 years at time of diagnosis. Male/female ratio was found to be 1.8/1. The most common cancers were non-Hodgkin lymphoma (NHL) (20.5%), Hodgkin's lymphoma (HL) (19.8%), central nervous system (CNS) tumors (10.9%), osteosarcoma (10.6%), Ewing sarcoma/primitive neuroectodermal tumors (EWN/PNET (9.9%) nasopharyngeal carcinoma (NPC) (9.6%). Non-compliance to medical treatment was observed in 10.9% of cases with significantly high non-compliance in NHL and osteosarcoma (p=0.02). Overall survival was 57.6% and EFS was 45.7% at a median follow-up time of 8.75 years. In adolescents, lower OS and EFS rates than the younger age group are observed. The most appropriate management plan should be made according to the physical and psychosocial needs of patients in this age group.
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http://dx.doi.org/10.24953/turkjped.2017.01.001DOI Listing
December 2017

Primary Osteosarcoma of the Rib: A Case Report and Review of the Literature.

J Pediatr Hematol Oncol 2018 01;40(1):48-50

Departments of Pediatrics, Division of Pediatric Oncology.

Osteosarcoma is the most common primary malignant tumor of the bone. The most common sites of osteosarcoma in children are the metaphysis of long bones, especially the distal femur, proximal tibia, and proximal humerus. It occurs very rarely in flat bones. Here we report a 14-year-old adolescent boy with primary osteosarcoma of the fifth rib and a review of literature.
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http://dx.doi.org/10.1097/MPH.0000000000000896DOI Listing
January 2018

Hepatoblastoma and Wolf-Hirschhorn syndrome: Coincidence or a new feature of a rare disease?

Pediatr Int 2017 Sep 7;59(9):1028-1029. Epub 2017 Aug 7.

Department of Pediatric Oncology, Cancer Institute, Hacettepe University, Ankara, Turkey.

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http://dx.doi.org/10.1111/ped.13345DOI Listing
September 2017

An orbital perivascular epithelioid cell tumor in a 7-year-old boy: case report and review of the literature.

J AAPOS 2017 Aug 30;21(4):325-328.e1. Epub 2017 May 30.

Department of Pediatric Oncology, Hacettepe University, Cancer Institute, Ankara, Turkey.

We report the case of a 7-year-old boy who presented with a swollen right eye. Magnetic resonance imaging revealed a right intraconal orbital mass with intense contrast enhancement. Incisional biopsy led to a diagnosis of perivascular epithelioid cell tumor (PEComa). Sirolimus was initiated but discontinued at the third week of treatment because the tumor had progressed. A minor regression of the tumor was seen after six cycles of systemic chemotherapy. Previously reported cases of PEComa were benign in nature, and full remission was achieved with surgical excision. In the present case the tumor was malignant and responded only slightly to systemic chemotherapy.
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http://dx.doi.org/10.1016/j.jaapos.2017.05.022DOI Listing
August 2017