Publications by authors named "Omer Gorgun"

25 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

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

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

Testis Involvement in Neuroblastoma: Report of 3 Cases in the Turkish Pediatric Oncology Group-Neuroblastoma Study and Review of the Literature.

J Pediatr Hematol Oncol 2019 01;41(1):13-16

Division of Pediatric Oncology, Oncology Institute, Dokuz Eylul University, Izmir, Turkey.

Background: Neuroblastoma (NB) is the most common extracranial solid tumor of childhood. Primary and secondary testicular involvement is extremely uncommon in neuroblastoma.

Procedure: All children with neuroblastoma treated with the Turkish Pediatric Oncology Group (TPOG)-Neuroblastoma (NB) Study and who had testis involvement either at diagnosis or at relapse were retrospectively evaluated. A review of all cases with neuroblastoma and testis involvement in the literature was done.

Results: There were 3 children with NB documented to have involvement of the testis, 2 at diagnosis, 1 at recurrence, within the 559 cases (0.5%) treated with the Turkish Pediatric Oncology Group (TPOG)-Neuroblastoma Protocol. All had advanced stage. Two were infants. A total of 57 cases of testicular or paratesticular neuroblastoma have been reported in children, and most cases represent metastases as in the 3 cases in our series.

Conclusions: Neuroblastoma should be considered in the differential diagnosis of testicular mass and work-up for neuroblastoma should be done before orchiectomy. Scrotal ultrasonography should be used as the first diagnostic tool and abdominal ultrasonography shall be done additionally. Testis examination should be performed at diagnosis and regularly during follow-up for boys diagnosed with neuroblastoma. Testes may be sanctuary sites when neuroblastoma is metastatic, as is the case in leukemia.
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http://dx.doi.org/10.1097/MPH.0000000000001316DOI Listing
January 2019

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

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

Breast Metastases in Children and Adolescents With Rhabdomyosarcoma: A Large Single-Institution Experience and Literature Review.

J Pediatr Hematol Oncol 2017 01;39(1):67-71

Departments of *Pediatric Hematology and Oncology, Cerrahpasa Faculty of Medicine †Pediatric Hematology and Oncology ∥Radiation Oncology, Oncology Institute ‡Pediatric Surgery, Istanbul School of Medicine, Istanbul University §Department of Surgery and Surgical Oncology, School of Medicine, Maltepe University, Istanbul, Turkey.

Introduction: Breast metastasis is rare in childhood malignancies. Soft tissue sarcomas, especially rhabdomyosarcomas (RMS), and hematologic neoplasms, such as lymphomas, are the most common tumors that metastasize to the breast, albeit rare.

Materials And Methods: All cases with breast metastasis within a cohort of 200 RMS patients followed in our institution during 1990 to 2014 were assessed retrospectively and the literature was reviewed.

Results: There were 3 adolescent female patients with breast metastasis. All had alveolar histology. The primary tumors were in the parameningeal sites, extremities, and the perineum, respectively. Two patients had breast metastasis at diagnosis, and 1 during follow-up. In 1 breast lesion, there was a complete response to chemotherapy, and in another there was no response to chemotherapy, and the patient underwent radical mastectomy. In the third patient, there was partial response, and lesions progressed. All patients died with recurrent/progressive disease, 2 with no recurrence in the breast. In the English literature, there are 70 cases including our cases. All but 1 involve female patients, all adolescents, most have alveolar histology and poor prognosis. All had chemotherapy, whereas some had surgery and/or radiotherapy for local treatment.

Conclusion: Breast metastasis should be considered in adolescent female patients with RMS. Optimal management is not clear. Besides chemotherapy, mastectomy and radiotherapy should be considered on a case basis.
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http://dx.doi.org/10.1097/MPH.0000000000000680DOI Listing
January 2017

Malignant transformation of aggressive osteoblastoma to ostesarcoma.

Eklem Hastalik Cerrahisi 2016 Aug;27(2):108-12

Division of Pediatric Oncology, İstanbul University, Oncology Institute, 34390 Çapa, Fatih, İstanbul, Turkey.

Osteoblastoma is a rare, bone-forming tumor, characterized by osteoid and woven bone production. A 13-year-old boy patient presented to our clinic with complaint of pain in his left proximal tibia. We performed curettage and bone grafting for the lesion diagnosed as osteoblastoma. Two years later, the patient admitted to the hospital with a mass in the same region which was diagnosed by biopsy to be osteosarcoma. Patient was performed reconstruction operation with local resection and mega prosthesis. Fourteen months after termination of chemotherapy, lung metastasis developed and the patient died consequently. In this article, we reported a patient with aggressive osteoblastoma of the left proximal tibia which recurred as an osteosarcoma and discussed the difficulties in the histopathological diagnosis and management of these patients. As some other cases in the literature, our case indicates that osteoblastomas may undergo malignant transformation.
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http://dx.doi.org/10.5606/ehc.2016.23DOI Listing
August 2016

A modified protocol with vincristine, topotecan, and cyclophosphamide for recurrent/progressive ewing sarcoma family tumors.

Pediatr Hematol Oncol 2013 Apr;30(3):170-7

Department of Pediatric Hematology-Oncology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.

Purpose: Topotecan has recently been used in the treatment of pediatric cancer. We evaluated our experience with the modified combination of vincristine, topotecan, and cyclophosphamide (VTC) given in 3 days, in children with recurrent Ewing sarcoma.

Method: Children received vincristine (1.5 mg/m(2)/1st day), cyclophosphamide (600 mg/m(2)/day × 2 days) + mesna, and topotecan (1 mg/m(2)/day × 3 days) every 21 days.

Result: A total of 118 courses of VTC were given to 13 patients. One patient received VTC both at first and at second relapse. Thus, 14 relapse episodes in 13 patients were evaluated. After three courses of VTC chemotherapy (CT), two achieved complete response (CR), five achieved partial response, thus an objective response was attained in 7/14 (50%) episodes. Two patients had stable disease and two patients progressed. In three episodes, CR was achieved by surgery before CT. One of them had a second relapse and attained CR with VTC. Median time from diagnosis to relapse was 23 months (5-45 months). Site of relapse was local in four patients, and metastatic in 10 episodes of nine patients. Seven patients are alive, three with no evidence of disease and four alive with disease; six have died of disease. Local treatment was used in 11 episodes. The toxicity of the VTC combination was limited mainly to the hematopoietic system.

Conclusion: In conclusion, the modified VTC protocol in 3 days every 3 weeks seems to be effective and tolerable in children and adolescents with recurrent/progressive Ewing sarcoma.
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http://dx.doi.org/10.3109/08880018.2013.767868DOI Listing
April 2013

Pediatric diffuse intrinsic pontine glioma patients from a single center.

Childs Nerv Syst 2013 Apr 8;29(4):583-8. Epub 2012 Dec 8.

Pediatric Hematology-Oncology, Cerrahpasa Medical Faculty and Oncology Institute, Istanbul University, Istanbul, Turkey.

Background: The prognosis of children with diffuse intrinsic pontine gliomas (DIPG) is dismal. This study aims to evaluate the characteristics and treatment outcome of children with DIPG in a single center.

Methods: We reviewed the outcome of children with DIPG treated at the Oncology Institute of Istanbul University from February 1999 to May 2012.

Results: Fifty children (26 female, 24 male) with the median age of 7 years were analyzed. The median duration of symptoms was 30 days. All patients received radiotherapy (RT). Before the year 2000, 12 patients received only RT. Thirty-eight had concomitant and/or adjuvant chemotherapy with RT. Between 2000 and 2004, 17 patients received cis-platinum or vincristine as sensitizers during RT and CCNU + vincristine combination after RT. Since 2004, 21 patients received temozolomide (TMZ) concomitantly during RT and as adjuvant chemotherapy after RT. The median survival time of all patients was 13 months (1-160 months). Patients receiving RT + TMZ had a significantly higher overall survival than patients with only RT (p = 0.018). Patients receiving RT + chemotherapy other than TMZ also had a significantly higher overall survival than patients receiving only RT (p = 0.013). Patients receiving RT + TMZ + and chemotherapy other than TMZ had a significantly higher survival than patients receiving only RT (p = 0.005).

Conclusion: In our series, patients receiving RT + TMZ and also patients receiving RT + chemotherapy other than TMZ had a significantly higher overall survival than patients treated with only RT. Hence, administering chemotherapy during and after RT seems to prolong survival in some DIPG patients.
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http://dx.doi.org/10.1007/s00381-012-1986-3DOI Listing
April 2013

Interferon-α for unresectable progressive and symptomatic plexiform neurofibromas.

J Pediatr Hematol Oncol 2013 Apr;35(3):e115-7

Division of Pediatric Hematology-Oncology, Cerrahpaşa Medical Faculty, and Oncology Institute, Istanbul University, Istanbul, Turkey.

Neurofibromas are the most common manifestations of neurofibromatosis type-1. They occasionally cause pain or progressive loss of function due to nerve compression. Optimal treatment approach is still challenging and the current treatment results are not satisfactory. Four cases of plexiform neurofibromas with various clinical presentations and an addendum to a previously published report on a patient who had relief from pain and/or regression of tumor volume after treatment with interferon-α 2a are presented.
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http://dx.doi.org/10.1097/MPH.0b013e318270cd24DOI Listing
April 2013

Intra-abdominal metastasis in osteosarcoma: survey and literature review.

Pediatr Hematol Oncol 2011 Oct 29;28(7):609-15. Epub 2011 Aug 29.

Department of Pediatric Hematology and Oncology, Cerrahpaşa Medical Faculty and Oncology Institute, Istanbul University, Istanbul, Turkey.

Extrapulmonary metastasis, particularly abdominal metastasis from osteogenic sarcoma, are rare and generally appear as a solid mass of calcification as the primary tumor. The aim of this case report is to document the incidence, characteristics, treatment, and prognosis of abdominal metastasis in osteosarcomas in a single institution and to review the literature. From September 1989 to December 2002, 94 children ≤16 years of age with osteosarcomas were diagnosed and treated in the Division of Pediatric Oncology, Oncology Institute, Istanbul University. Patients with abdominal metastasis were assessed. Two girls of 94 patients (2.1%) with osteosarcoma developed abdominal metastasis. One had pulmonary metastasis at diagnosis and the other had developed lung metastasis 15 months after diagnosis. They developed abdominal metastasis 4 and 3 years after diagnosis during therapy or relapse at a median duration of 16 months (1-70 months) from initial diagnosis. All patients had metastasis to various sites, mostly lung, at the time the abdominal metastasis were detected. Treatment included surgery, chemotherapy, and radiotherapy in one and only surgery in the other patient. Both patients died at a median time of 4 months (2-6 months) from the time of abdominal metastasis with progressive disease. Abdominal metastasis in osteosarcoma is a rare event, but abdomen should be investigated in case of recurrence from osteosarcoma. The outcome for these patients is dismal in this series and in the literature.
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http://dx.doi.org/10.3109/08880018.2011.590959DOI Listing
October 2011

Metastatic mandibular neuroblastoma: a rare cause of tooth mobility.

J Clin Pediatr Dent 2011 ;36(2):203-6

Istanbul University, Faculty of Dentistry, Department of Oral Medicine and Surgery, Istanbul, Turkey.

Neuroblastoma (NBL), a malignant embryonic tumor derived from neural crest cells, is the most common tumor worldwide among children less than 1 year of age. Metastasis to the mandible is uncommon. This article reports the case of a 15-month-old male diagnosed with NBL with bone metastasis including the mandible which resulted in severe tooth mobility. Dentists or pediatricians should consider the primary or metastatic tumors of the maxillofacial region in the differential diagnosis in children presenting with premature loss of teeth related to tooth mobility.
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http://dx.doi.org/10.17796/jcpd.36.2.qm4064n5751541j3DOI Listing
May 2012

Malignant ovarian germ cell tumors: a single-institution experience.

Am J Clin Oncol 2009 Apr;32(2):191-6

Department of Medical Oncology, Faculty of Medicine, Trakya University, Edirne; Turkey.

Objective: To evaluate the clinicopathologic prognostic factors in malignant ovarian germ cell tumors.

Methods: We reviewed the medical records of 70 patients treated from 1990 to 2006 at our center. Clinical data including demographics, stage, surgery, chemotherapy, survival, menses status, and fertility were collected from patients' charts.

Results: Median age was 22 years (range, 9-68). The histologic subtypes included 36 dysgerminomas, 11 yolk sac tumors, 3 immature teratomas, 1 embryonal carcinomas, and 19 mixed types. The most striking clinicopathologic finding was a history of concomitant immunosuppressant therapy, which was observed in 2 patients. Two patients had contralateral sex-cord tumors at presentation and follow-up. During a median follow-up period of 4.6 years, 11 patients had recurrence. The median time to recurrence was 8 months (6-28 months). Recurrences appeared in the abdominopelvic cavity in 9 out of 11 patients. Only one could be salvaged with second-line chemotherapy. Cumulative survival rate was 97% and 60% in patients with dysgerminoma and nondysgerminoma, respectively. Nondysgerminoma histology and residual tumor after surgery were unfavorable prognostic factors (P < 0.001 and P = 0.015). Fertility-sparing surgery was as effective as radical surgery among all eligible patients. Of patients with known menstrual status, 96% had regular menses. Of the 8 patients who opted for conception among these patients, 7 delivered healthy infants.

Conclusions: Nondysgerminomas have an aggressive clinical course. New treatment strategies are needed for eradication of abdominopelvic disease at initial diagnosis and recurrent setting. Occurrence of malignant ovarian germ cell tumors may be associated with immunosuppression in some patients. Sex-cord stromal tumors may present with bilateral involvement. It is possible to maintain fertility after fertility-sparing surgery followed by chemotherapy.
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http://dx.doi.org/10.1097/COC.0b013e3181841f2eDOI Listing
April 2009

Hypertrophic osteoarthropathy and intrathoracic Hodgkin's disease in children.

Leuk Res 2006 Jul 28;30(7):899-902. Epub 2005 Nov 28.

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

Background: Hypertrophic osteoarthropathy (HOA) is a syndrome characterized by clubbing of the fingers and toes, periosteal new bone formation of the long bones and polyarthritis.

Case Report: In this report, two children with intrathoracic Hodgkin's disease and HOA are presented.

Conclusions: Intrathoracic neoplasms are one of the major causes of HOA in adults; however HOA is rarely associated with intrathoracic malignancies in children. HOA associated with intrathoracic Hodgkin's disease is even more rare, but should be kept in mind.
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http://dx.doi.org/10.1016/j.leukres.2005.10.019DOI Listing
July 2006

Are serum levels of CD44 relevant in children with pediatric sarcomas?

Pediatr Blood Cancer 2006 Jan;46(1):62-5

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

Background: Variation in serum levels of CD44, which acts as an adhesion receptor involved in lymphocyte migration and binding, have been reported in some malignancies. The aim of this study is to compare serum levels of CD44 in children with sarcomas with those in healthy children.

Procedure: CD44 levels were measured by enzyme-linked immunosorbent assay (ELISA) in serum samples taken at diagnosis from 55 children with sarcomas and from 27 healthy children of similar age, sex, and socioeconomic status.

Results: There was no statistically significant difference between CD44 serum levels of children with sarcomas and those of healthy children. No significant difference was observed between CD44 serum levels of each patient group and those of control group (P > 0.05). There was no significant difference among CD44 serum levels of patient groups according to stage or outcome.

Conclusions: In this study, serum CD44 levels were not found to be of value in the diagnosis or prognosis in children with sarcomas.
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http://dx.doi.org/10.1002/pbc.20554DOI Listing
January 2006

Ependymal tumors in childhood.

Pediatr Blood Cancer 2005 Sep;45(3):298-303

Department of Radiation Oncology, Istanbul University-Istanbul Medical Faculty, 34390 Capa Istanbul, Turkey.

Background: Ependymal tumors are classified as ependymoma (benign or low grade) versus anaplastic ependymoma (malignant or high grade). Ependymomas represent 5-10% of intracranial neoplasm in children. In this study, demographic data and the treatment results of pediatric patients with ependymal tumors, treated in a single institute, is reported.

Patients And Methods: Between 1989 and 2001, 40 (22 M/18 F) previously untreated patients with a median age of 5.5 years (3 months-15 years), of histologically proven ependymal tumors (except ependymoblastomas) were referred to the Institute of Oncology, University of Istanbul. The localization was supratentorial in 18, infratentorial in 20, both supra and infratentorial in two patients. Histologic subgroups were 18 ependymomas (43.6%), and 22 anaplastic ependymomas (56.4%). Total tumor resection was performed in 20 patients (50%), subtotal in 18 patients (45%), and biopsy only in 2 patients (5%). Postoperative treatment consisted of regional (8 patients) or craniospinal (CSI) (9 patients) radiotherapy (RT) in patients with ependymoma; regional (7 patients) or CSI RT (14 patients) with chemotherapy (ChT) in patients with anaplastic ependymoma; ChT only (1 patient) in patients less than 3 years of age. The standard technique for posterior fossa irradiation was parallel-opposed lateral fields and total dose was 45-54 Gy. Between September 1989 and May 1991 patients received regimen A, which consisted of RT followed by eight-in-one ChT, given every 4 weeks for eight courses. Patients who were treated between June 1991 and July 1994, received regimen B, which included two courses of postoperative "VEC" (vincristine, etoposide, cisplatin) ChT, administered every 3 weeks, followed by RT applied with low dose concomitant cisplatin used as a radiosensitizer. Patients with objective response to postoperative "VEC" continued to have "VEC" after completion of RT for six more courses. From August 1994 on, patients received regimen C, consisting of RT and concomitant infusion of cisplatin followed by "VCPCU" (vincristine, cyclophosphamide, procarbazine, lomustine) administered every 4 weeks for eight courses.

Results: A total of 40 patients were included in the outcome and survival data. The 5-year overall survival (OS) rate was 64.9%, and the 5-year progression-free survival rate was 50.8% for the whole series. Median time for progression or relapse was 24.3 months and there were 19 patients (43.6%) with relapse or progression. Non-metastatic patients (P = 0.0008, 5-year OS rate was 82% vs. 29%), and totally resected patients (P = 0.01, 5-year OS rate was 80% vs. 55%), and > or =3 years of age (P = 0.04, 5-year OS rate was 75% vs. 38%) had significantly better outcome.

Conclusions: The majority of complete responders were patients who had total tumor removal. Treatment failure occurred mainly within the first 2 years, and outcome was dismal for patients who relapsed or had progressive disease. The median age at diagnosis is 6 years in our patient group; younger children (less than 3 years old) have less favorable outcome. There was no significant difference in survival or progression-free survival between the two histologic subtypes.
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http://dx.doi.org/10.1002/pbc.20212DOI Listing
September 2005

Brain metastasis in pediatric extracranial solid tumors: survey and literature review.

J Neurooncol 2005 Jan;71(1):43-8

Division of Pediatric Oncology, Oncology Institute, Istanbul University, Capa, Istanbul, Turkey.

Objectives: Brain is a rare site of metastasis in most extracranial pediatric solid tumors. The aim of this study is to investigate the incidence, treatment, prognosis of brain metastasis in extracranial pediatric malignant tumors in a single institution and to review the literature.

Methods: From September 1989 to December 2002, 1100 children
Results: Sixteen (10 female, 6 male) of 1100 patients (1.45%) with extracranial solid tumors developed brain metastases. The median age of the patients was 10.5 (1-16) years. The diagnosis was sarcomas in 12 patients: 5 osteosarcomas, 4 Ewing's sarcoma family tumors, 1 rhabdomyosarcoma, 1 clear cell sarcoma of the soft tissue, 1 alveolar soft part sarcoma. Two patients had Wilms' tumor and two had germ cell tumors. Four patients (25%) had brain metastasis at diagnosis. Twelve (75%) developed brain metastasis during therapy or relapse at a median duration of 16 (1-70) months from initial diagnosis. All patients had metastases to various sites, mostly lung, at the time the brain metastases were detected. Treatment included surgery, followed by postoperative radiotherapy (RT) and chemotherapy (CT) in 1, S and RT in 1, S in 1, RT and CT in 6, RT in 1, CT in 1 and no treatment in 5. Only one patient with alveolar soft part sarcoma is alive with disease 20 months from diagnosis of brain metastasis. All other patients died at a median time of 2 months (2 days-6 months) from the time of brain metastasis.

Conclusions: Children with metastatic cancer who develop headaches or any other neurologic symptom should be investigated for possible brain metastasis. Although, the outcome for these patients is dismal in this series and in the literature; reports of long term survival in a few cases with Wilms' tumor, osteosarcoma and alveolar soft part sarcoma who had isolated brain metastasis, suggest that a subset of patients may benefit from therapy.
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http://dx.doi.org/10.1007/s11060-004-4840-yDOI Listing
January 2005

Congenital mesenchymal chondrosarcoma of the orbit: case report and review of the literature.

Ophthalmology 2004 May;111(5):1016-22

Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Purpose: To report the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit.

Design: Interventional case report and review of the literature.

Methods: Ophthalmologic examination and computed tomography scan of the orbit were performed. Histologic and histochemical examination and immunohistochemistry of the biopsy specimen were evaluated.

Main Outcome Measures: Ocular and systemic disease control.

Results: A 5-month-old girl was seen with a papillomatous lesion in the right lower tarsal conjunctiva present from birth. Histologic examination demonstrated an admixture of undifferentiated mesenchymal cells and islands of mature hyaline cartilage, and immunohistochemistry studies revealing positivity for vimentin and S-100 were consistent with the diagnosis of mesenchymal chondrosarcoma. The patient was treated with combined chemotherapy (ifosfamide, epirubicin, and cisplatin), radiotherapy, and surgery (exenteration). She is alive with no evidence of disease after 4 years of follow-up. Facial asymmetry and dental malformations have developed as late effects. Further reconstructive surgery is planned for the malformations.

Conclusions: Extraskeletal mesenchymal chondrosarcoma in the orbit is extremely rare. This study reports the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit (also the youngest patient) both in skeletal and extraskeletal sites. Multimodality treatment (surgery, chemotherapy, and radiotherapy) may lead to long-term survival.
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http://dx.doi.org/10.1016/j.ophtha.2003.12.026DOI Listing
May 2004

Oral etoposide for recurrent/progressive sarcomas of childhood.

Pediatr Blood Cancer 2004 Apr;42(4):320-4

Istanbul University, Oncology Institute, Division of Pediatric Oncology, Capa, Istanbul, Turkey.

Background: Etoposide (VP-16) is a topoisomerase II inhibitor that is effective in a broad spectrum of pediatric and adult malignancies. Chronic, low-dose, oral VP-16 has also been shown to be active in some recurrent malignancies mostly in adults. The aim of this prospective, single institution study is to assess the efficacy and toxicity of oral VP-16 in children with progressive or recurrent (P/R) sarcomas.

Procedure: Twenty-one children (10 girls and 11 boys) with R/P sarcomas and a median age of 11 years (range 3-16 years) were enrolled in this study. The diagnosis was Ewing sarcoma family tumor (ESFT) in seven, osteosarcoma in eight, rhabdomyosarcoma in four, clear cell sarcoma of soft tissue in one, fibrosarcoma in one patient. Oral VP-16 was administered at a dose of 50 mg/m(2)/daily for 20 days. The next course was initiated after a 10 day rest. Response to oral VP-16 was assessed after two courses.

Results: There was an objective response (one complete response [CR], two partial responses [PR]) in three patients (14%) by two courses of oral VP-16 alone. One of these patients with PR achieved CR by the use of radiotherapy (RT) and further oral VP-16. Two more patients (9.5%) achieved CR by RT and oral VP-16. Eight (38%) patients had disease stabilization for 2-15 months. Two patients (9.5%) are long-term survivors. They are alive with no evidence of disease (NED) 79 and 94 months from time of relapse/progressive disease (PD). A patient developed acute myeloid leukemia and died. There was no major acute toxicity related to oral VP-16 in a total of 126 courses.

Conclusions: Oral VP-16 therapy is simple, relatively nontoxic, and does not necessitate hospitalization. The cure rate is small. Given the risk of second malignancy, especially in children with previous exposure to topoisomerase II inhibitors and alkylating agents, this regimen may be used as a palliative treatment or in patients with poor prognosis.
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http://dx.doi.org/10.1002/pbc.10393DOI Listing
April 2004

Rhabdomyosarcoma of the biliary tree.

Pediatr Int 2003 Aug;45(4):469-71

Divisions of Pediatric Oncology and Radiation Oncology, Oncology Institute, Istanbul University, Istanbul, Turkey.

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http://dx.doi.org/10.1046/j.1442-200x.2003.01763.xDOI Listing
August 2003

Is the Epstein Barr virus implicated in Ewing sarcoma?

Med Pediatr Oncol 2003 Apr;40(4):256-7

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

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http://dx.doi.org/10.1002/mpo.10162DOI Listing
April 2003