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.
Gardner-Diamond syndrome (GDS) is a rare disease often seen in young women involving painful localized inflammation and ecchymosis. Ecchymosis usually develops spontaneously after emotional stress. The pathophysiology of the disease is not fully understood, and little is known about management modalities for this syndrome. The primary approach of health professionals in the evaluation of this rare condition should involve identification of cases and investigation of potential accompanying psychiatric pathologies. The case presented here highlights the importance of assessing for GDS and reviews descriptions of GDS in the context of the existing literature.
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