Neuroblastoma in children

Neuroblastom je najčešći ekstrakranijalni solidni tumor dječje dobi i četvrti po redu od svih pedijatrijskih tumora. Potječe od neuroblasta, pluripotentnih stanica simpatičkog živčanog sustava. Najčešće se primarni tumor nađe u abdomenu, zatim u prsnom košu, a vrat i zdjelica su rjeđe lokalizacije. Bolest se u većine djece prezentira s palpabilnom abdominalnom masom kao prvim znakom bolesti. Ostali znakovi i simptomi bolesti su: kašalj, dispneja, Hornerov sindrom, disfunkcija mokraćnog mjehura i crijeva, neurološki ispadi, bolovi u kostima, orbitalne ekhimoze, povremene temperature, anemija, generalizirana bol itd. Nažalost se, otprilike 70-80% pacijenata starijih od 18 mjeseci prezentira s metastatskom bolešću u limfnim čvorovima, jetri, koži, kostima i koštanoj srži. U dijagnostici neuroblastoma koriste se laboratorijski testovi i slikovne metode za evaluaciju primarne bolesti i njene diseminacije. Dob, stadij i biološke karakteristike tumora su važni prognostički čimbenici koji se koriste u stratifikaciji rizika i u odabiru odgovarajućeg liječenja. Razlike u ishodu bolesti među pacijentima s neuroblastomom su upečatljive. Pacijenti s niskim i intermedijarnim rizikom bolesti imaju odličnu prognozu i ishod. Međutim, oni s visokim rizikom bolesti i dalje imaju vrlo lošu prognozu usprkos intenzivnoj terapiji. Čak i s primjenom visokih doza kemoterapije i autolognom transplantacijom matičnih stanica, manje od polovice pacijenata se izliječi.Neuroblastoma is the most common extracranial solid tumor in children and fourth of all pediatric cancers. It originates from neuroblasts, pluripotent cells of the sympathetic nervous system. The majority of primary tumors occur in the abdomen, then in thorax, seldom in neck and pelvis. Neuroblastoma usually presents with palpable abdominal mass as the first sign of the disease. Other signs and symptoms include: cough, dyspnea, Horner's syndrome, bladder and bowel dysfunction, neurologic disorders, bone pain, orbital ecchymosis, occasional temperature, anemia, generalized pain, etc. Unfortunately, approximately 70-80% of patients older than 18 months present with metastatic disease, usually in the lymph nodes, liver, bone, and bone marrow. The diagnosis of neuroblastoma consists of lab tests and imaging techniques for the evaluation of the primary tumor and its extension. Age, stage and biological features encountered in tumor are important prognostic factors and are used for risk stratification and treatment assignment. The differences in outcome for patients with neuroblastoma are striking. Patients with low- and intermediate-risk neuroblastoma have excellent prognosis and outcome. However, those with high-risk disease continue to have very poor outcomes despite intensive therapy. Less than half of these patients are cured, even with the use of high-dose therapy followed by autologous bone marrow and stem cell rescue