Çocuk onkoloji hastalarında konjenital anomalilerin sıklığının araştırılması
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Date
2017
Authors
Turan, Cansu
Journal Title
Journal ISSN
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Publisher
Uludağ Üniversitesi
Abstract
Çocukluk çağı kanserlerinin birçoğunun etyolojisi net açıklanamamıştır. Son çalışmalar, anomalilerin genetik ve çevresel faktörlerin de etkisi ile kanser gelişiminde rol alabileceğini göstermiştir. Bu çalışmanın amacı, maligniteler ve konjenital anomaliler arasındaki ilişkinin araştırılması ve çeşitli kanserlere en sık eşlik eden anomalilerin belirlenmesidir. Çalışmamızda Uludağ Üniversitesi Tıp Fakültesi Çocuk Onkoloji Bilim Dalı'nda takipli, solid tümör veya lenfoması olan 506 hasta incelenmiştir. Hastalardaki malignite dağılımına bakıldığında 135 (%27) hasta sayısı ile lenfomalar birinci sıradadır. Bunu 65 (%13) hasta ile santral sinir sistemi tümörleri ve 63 (%12) hasta ile germ hücreli tümörler takip etmektedir. Hastaların %33.5'inde (n=170) anomali olduğu görülmüştür. Bu 170 hastada, 241 farklı anomali tanımlanmıştır. Bu anomalilerin %30.7'sinin göz, kulak, yüz, boyun anomalileri, %18.25'inin kardiyovasküler sistem anomalileri, %10.78'inin ürogenital sistem anomalileri, %9.12'sinin SSS anomalileri, %7.05'inin kas iskelet sistemi anomalileri, %6.27'sinin sindirim sistemi anomalileri, %2.48'inin solunum sistemi anomalileri, %15.35'inin ise diğer anomaliler grubunun anomalileri olduğu görülmüştür. Hastaların demografik özelliklerine bakıldığında; hastaların %44.6'sı (n=226) kız, %55.4'ü (n=280) erkektir. Hastaların ortalama yaşı 7.34±5.46 yıldır. Doğumdaki ortalama anne yaşı 26.83±5.17, ortalama baba yaşı ise 30.02±5.41'tir. Soygeçmişlerine bakıldığında %20.4'ünde (n=103) annede düşük veya ölü doğum öyküsü olduğu, %28.1'inde (n=142) akrabalarında kanser öyküsü olduğu ve %14.2'sinin (n=72) anne ve babası arasında akrabalık olduğu görülmüştür. Anomalisi olan hastaların %25'inin doğumdaki baba yaşının 35 yaş ve üzeri olduğu görülmüştür (p:0.004). Malignitelerde anomali görülme sıklığı incelendiğinde; SSS tümörü olan hastalarda anomali görülme sıklığı %61.5 (n=40) olarak saptanmıştır (p<0.001). Böbrek tümörlü hastaların %32.7'sinde, sarkomlu hastaların %32.2'sinde, nöroblastomlu hastaların %31.6'sında ve lenfomalı hastaların %27.4'ünde anomali olduğu görülmüştür. SSS tümörü olan hastaların %32.3'ünde (n=21) göz, kulak, yüz, boyun anomalisi saptanmış ve diğer malignite gruplarına göre istatistiki olarak anlamlı oranda sık bulunmuştur (p<0.001). SSS anomalisi olan hastaların %36.3'ünde SSS tümörü mevcuttur ve bu gruptaki en sık görülen malignite grubudur. Göz, kulak, yüz, boyun anomalisi olan 58 hastanın %28.3'ünde (n=21) de SSS tümörü olduğu görülmüştür. Ekokardiyografisi olan hastaların %20.5'sinde (n=44) kardiyak anomali tespit edilmiştir. Malignite grupları arasında kardiyovasküler sistem anomalisi görülme sıklığı açısından fark görülmemiştir. Germ hücreli tümörü olan hastaların ekokardiyografisi olanlar içerinde %35.7'sinde, böbrek tümörü olanların %31'inde, yumuşak doku sarkomu olanların %26.7'sinde kardiyovasküler anomali saptanmıştır. Sonuç olarak; anomaliler ve maligniteler arasında bir ilişki olduğu açıktır. Kanser gelişiminde ve özellikle embriyolojik gelişim evreleri sırasında gelişen konjenital anomalilerde rol alan genlerle ilgili yapılacak olan ileri çalışmalar değerli bilgiler sağlayabilir. Bu alanda yapılacak çok daha geniş kapsamlı çalışmalara ihtiyaç vardır.
The etiology of a large number of childhood cancers is not clear. Recent studies have shown that anomalies may play a role in the development of cancer by the effects of genetic and environmental factors. The aim of this study was to investigate the relationship between malignancies and congenital anomalies and to identify the most common anomalies associated with various cancers. In our study, 506 patients with solid tumors or lymphoma who were followed up at Uludag University Faculty of Medicine, Department of Pediatric Oncology were examined. The distribution of malignancies among the patients revealed that 135 (27%) patients had lymphoma, the leading malignancy in this category, followed by 65 (13%) patients with central nervous system (CNS) tumors and 60 (12%) with neuroblastoma. It was found that 33.5% (n = 170) of the patients had anomalies. In these 170 patients, 241 different anomalies were identified. Distribution of these anomalies is 30.7% eye, ear, face, neck anomalies and 18.25% cardiovascular system anomalies, 10.8% urogenital system anomalies, 9.12% CNS anomalies, 7.05% musculoskeletal system anomalies, 6.27% digestive system anomalies, 2.48% respiratory system anomalies and 15.35% other anomalies. Demographic characteristics of the patients are examined; 44.6% of the patients (n = 226) were female and 55.4% (n = 280) were male. The mean age of the patients was 7.34 ± 5.46 years. Mean maternal age at birth was 26.83 ± 5.17, and mean father age was 30.02 ± 5.41. When the patient's family history is examined, It was found that 20.4% (n = 103) had a history of low or stillbirth in the mother, 28.1% (n = 142) had cancer stories in their relatives and 14.2% (n = 72) had consanguineous marriage. It was observed that 25% of patients with anomalies had a father of age 35 years and older at birth. This result was statistically significant when compared to the group without anomalies (p:0.004). The frequency of anomalies in malignant groups were examined; the incidence of anomalies in patients with CNS tumors was 61.5% (n = 40) (p <0.001). It was found that, in 32.7% of renal tumor patients, in 32.2% of patients with sarcoma, in 31.6% of neuroblastoma and in 27.4% of lymphoma patients had an anomaly. Eye, ear, face and neck anomalies were found in 32.3% (n = 21) of patients with CNS tumors and statistically significant frequency was found to be statistically significant compared to other malignancy groups (p <0.001). CNS tumors are present in 36.3% of patients with CNS anomalies and this group is the most common malignancy group. Of the 58 patients with eye, ear, face, and neck anomalies, 36.2% (n = 21) had CNS tumors. Cardiac anomaly was detected in 20.5% (n = 44) who examined with echocardiography. There was no difference in the incidence of cardiovascular system anomalies between malignant groups; but the incidence of cardiovascular system anomalies in malignancy groups is significantly higher. Cardiovascular abnormalities were detected in 35.7% of patients with germ cell tumors, 31% of those with renal tumors, and 26.7% of those with soft tissue sarcomas. Consequently; it is clear that there is an association between anomalies and malignancies. Further studies on cancer development and genes involved in congenital anomalies that occur especially during embryological developmental stages may provide valuable information regarding this subject. There is a need for much more extensive work to be done on this topic. We suppose that our study will contribute to the malignancy-anomaly relationship to be investigated later on.
The etiology of a large number of childhood cancers is not clear. Recent studies have shown that anomalies may play a role in the development of cancer by the effects of genetic and environmental factors. The aim of this study was to investigate the relationship between malignancies and congenital anomalies and to identify the most common anomalies associated with various cancers. In our study, 506 patients with solid tumors or lymphoma who were followed up at Uludag University Faculty of Medicine, Department of Pediatric Oncology were examined. The distribution of malignancies among the patients revealed that 135 (27%) patients had lymphoma, the leading malignancy in this category, followed by 65 (13%) patients with central nervous system (CNS) tumors and 60 (12%) with neuroblastoma. It was found that 33.5% (n = 170) of the patients had anomalies. In these 170 patients, 241 different anomalies were identified. Distribution of these anomalies is 30.7% eye, ear, face, neck anomalies and 18.25% cardiovascular system anomalies, 10.8% urogenital system anomalies, 9.12% CNS anomalies, 7.05% musculoskeletal system anomalies, 6.27% digestive system anomalies, 2.48% respiratory system anomalies and 15.35% other anomalies. Demographic characteristics of the patients are examined; 44.6% of the patients (n = 226) were female and 55.4% (n = 280) were male. The mean age of the patients was 7.34 ± 5.46 years. Mean maternal age at birth was 26.83 ± 5.17, and mean father age was 30.02 ± 5.41. When the patient's family history is examined, It was found that 20.4% (n = 103) had a history of low or stillbirth in the mother, 28.1% (n = 142) had cancer stories in their relatives and 14.2% (n = 72) had consanguineous marriage. It was observed that 25% of patients with anomalies had a father of age 35 years and older at birth. This result was statistically significant when compared to the group without anomalies (p:0.004). The frequency of anomalies in malignant groups were examined; the incidence of anomalies in patients with CNS tumors was 61.5% (n = 40) (p <0.001). It was found that, in 32.7% of renal tumor patients, in 32.2% of patients with sarcoma, in 31.6% of neuroblastoma and in 27.4% of lymphoma patients had an anomaly. Eye, ear, face and neck anomalies were found in 32.3% (n = 21) of patients with CNS tumors and statistically significant frequency was found to be statistically significant compared to other malignancy groups (p <0.001). CNS tumors are present in 36.3% of patients with CNS anomalies and this group is the most common malignancy group. Of the 58 patients with eye, ear, face, and neck anomalies, 36.2% (n = 21) had CNS tumors. Cardiac anomaly was detected in 20.5% (n = 44) who examined with echocardiography. There was no difference in the incidence of cardiovascular system anomalies between malignant groups; but the incidence of cardiovascular system anomalies in malignancy groups is significantly higher. Cardiovascular abnormalities were detected in 35.7% of patients with germ cell tumors, 31% of those with renal tumors, and 26.7% of those with soft tissue sarcomas. Consequently; it is clear that there is an association between anomalies and malignancies. Further studies on cancer development and genes involved in congenital anomalies that occur especially during embryological developmental stages may provide valuable information regarding this subject. There is a need for much more extensive work to be done on this topic. We suppose that our study will contribute to the malignancy-anomaly relationship to be investigated later on.
Description
Keywords
Çocukluk çağı solid tümörleri ve lenfomaları, Konjenital anomaliler, Childhood solid tumors and lymphomas, Congenital anomalies
Citation
Turan, C. (2017). Çocuk onkoloji hastalarında konjenital anomalilerin sıklığının araştırılması. Yayınlanmamış uzmanlık tezi. Uludağ Üniversitesi Tıp Fakültesi.