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CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2013; 34(04): 264-269
DOI: 10.4103/0971-5851.125241
ORIGINAL ARTICLE

Rare childhood tumors in a Turkish pediatric oncology center

Nurdan Tacyildiz
Department of Pediatric Oncology, Ankara University, Ankara, Turkey
,
Derya Özyörük
Department of Pediatric Oncology, Ankara University, Ankara, Turkey
,
Gülsan Yavuz
Department of Pediatric Oncology, Ankara University, Ankara, Turkey
,
Emel Cabı Ünal
Department of Pediatric Oncology, Ankara University, Ankara, Turkey
,
Handan Dinçaslan
Department of Pediatric Oncology, Ankara University, Ankara, Turkey
,
Gülşah Oktay Tanyıldız
Department of Pediatric Oncology, Ankara University, Ankara, Turkey
,
Zülfikar Gördü
Department of Pediatric Oncology, Ankara University, Ankara, Turkey
,
Zeynep Şıklar
Department of Pediatric Endocrinology, Ankara University, Ankara, Turkey
,
Merih Berberoğlu
Department of Pediatric Endocrinology, Ankara University, Ankara, Turkey
,
Gönül Öcal
Department of Pediatric Endocrinology, Ankara University, Ankara, Turkey
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Abstract

Background: It has been estimated that rare tumor rate is about 15% of all childhood cancer in United States. According to Turkish Pediatric Oncology Group (TPOG) datas, 8889 children were diagnosed between 2002 and 2008 in our country and 3.7% of them were diagnosed as rare tumors. Aim: To investigate the frequency and clinical features of rare tumors in our pediatric oncology center. Materials and Methods: A total of 43 cases that have diagnosed as rare tumor in 574 cancer patients between the yaer 2002 and 2012 were reviewed retrospectively. All cases definitive diagnosis were established by histopathological and immunohistochemical studies. Results: Frequency of rare tumors was 7.4% in our center. Benign and border line rare tumors were 27 (62.7%) cases, malignant rare tumor were 16 (37.2%) cases. Median follow-up period was 48 months (between 1 and 110 months). Six of the malignant rare tumors were died with progressive disease (synovial sarcoma, mixed malignant mesenchymal tumor, undifferentiated sarcoma, plexus choroideus carcinoma, renal peripheral primitive neuroectodermal tumor, adrenocortical carcinoma). Malignant rare tumor mortality rate was found 37.5% in our clinic. Conclusion: We have found that our rare tumor rate (7.4%) was higher than Turkish rare tumor rate (3.7%) according to TPOG′s datas. However, it was still lower than rare tumor rates of western countries (15%), probably due to difficulties of diagnosis and referral problems.

Keywords

Benign rare tumors - border-line rare tumors - malignant rare tumors


Publication History

Article published online:
19 July 2021

© 2013. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/.)

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