Pediatrik Olgularda Mesane, Adrenal ve Renal Tümörlere Yaklaşım
Özet
Çocukluk çağı kanserleri dünya genelinde önemli bir mortalite nedenidir, ancak erken tanı ve güncel multimodal tedavilerle sağkalım oranları artmaktadır. Pediatrik mesane tümörleri nadir görülür ve en sık saptanan tipi, üzüm salkımı görünümüyle karakterize embriyonel rabdomyosarkomdur (RMS); tedavisinde organ koruyucu yaklaşımlar ön planda olup, kemoterapi, radyoterapi ve gerektiğinde cerrahi rezeksiyon uygulanır. Çocuklarda en sık görülen ekstrakraniyal solid tümör olan nöroblastom (NB), sıklıkla adrenal medulladan köken alır ve idrarda katekolamin metabolitleri (VMA, HVA) artışıyla tanı alır; risk gruplarına göre tedavisi cerrahi gözlemden yoğun indüksiyon kemoterapisi, kök hücre nakli ve immünoterapiye kadar değişkenlik gösterir. Çocukluk çağının en sık böbrek tümörü olan Wilms tümörü (WT) ise genellikle ebeveynler tarafından fark edilen ağrısız bir karın kitlesiyle klinik verir; COG ve SIOP protokolleri doğrultusunda nefrektomi ve kemoterapi kombinasyonuyla başarıyla yönetilir. Ayrıca konjenital mezoblastik nefrom, berrak hücreli sarkom, rabdoid tümör ve renal hücreli karsinom gibi diğer böbrek tümörleri de farklı prognostik özelliklerle çocuklarda görülebilmektedir. Her üç tümör grubunda da erken ve doğru evreleme ile multidisipliner tedavi yaklaşımları hayat kurtarıcı rol oynamaktadır.
Childhood cancers are a major cause of mortality worldwide, but survival rates are increasing with early diagnosis and modern multimodal treatments. Pediatric bladder tumors are rare, and the most frequently detected type is embryonal rhabdomyosarcoma (RMS), characterized by a cluster-of-grapes appearance; its treatment focuses on organ-preserving approaches, combining chemotherapy, radiotherapy, and surgical resection when necessary. Neuroblastoma (NB), the most common extracranial solid tumor in children, frequently originates from the adrenal medulla and is diagnosed by elevated urinary catecholamine metabolites (VMA, HVA); its treatment varies based on risk groups, ranging from surgical observation to intensive induction chemotherapy, stem cell transplantation, and immunotherapy. Wilms tumor (WT), the most frequent renal tumor in childhood, typically presents as a painless abdominal mass often noticed by parents, and is successfully managed through a combination of nephrectomy and chemotherapy in line with COG and SIOP protocols. Additionally, other renal tumors such as congenital mesoblastic nephroma, clear cell sarcoma, rhabdoid tumor, and renal cell carcinoma can occur in children with distinct prognostic characteristics. In all three tumor groups, early and accurate staging along with multidisciplinary treatment approaches play a life-saving role.
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