Brainstem tumors in children

Primarni tumori mozga i leđne moždine čine oko 20% svih tumora u djece te predstavljaju drugu najčešću dječju novotvorinu nakon hematoloških. U djece između dvije i sedam godina starosti najčešći su tumori stražnje lubanjske jame, uključujući i moždano deblo. Tumori moždanog debla definirani su prije svega svojom anatomskom lokalizacijom, a manje patohistološkim fenotipom. Astrocitomi i glioblastomi čine oko 50% svih novotvorina dok su rjeđi meduloblastomi, ependimomi, kraniofaringeomi, oligodendrogliomi te tumori zametnih stanica. Iako se u predjelu moždanog debla mogu pojaviti svi patohistološki oblici tumora, sama pojava u toj regiji, tipična neuroradiološka slika te izraženi klinički simptomi važniji su prognostički čimbenik nego patohistološki fenotip i stupanj diferenciranosti. Ukupna prognoza tumora moždanog debla u djece je loša, srednje preživljenje oboljele djece iznosi 8 – 10 mjeseci. U ovu studiju uključeno je 10 djece u dobi od 1 – 7 godina s dijagnozom potvrđenom patohistološki ili neuroradiološki tumora moždanog debla koja su liječena u razdoblju od 2008. do 2012. godine u Klinici za dječje bolesti u Klaićevoj te u Klinici za pedijatriju, KBC Zagreb. Cilj ove studije je određivanje objektivnog odgovora pacijenata na provedeno liječenje i ukupnog preživljenja bolesnika i određivanje toksičnosti provedenog liječenja. Svi su bolesnici liječeni kemoterapijom i radioterapijom. Kemoterapija je provođena po protokolu HIT-GBM ili temozolomidom. Radioterapija se provodila zračenjem područja stražnje lubanjske jame i sijela primarnog procesa ukupnom dozom do 54 Gy u 27 frakcija. Primjenjivana je trodimenzionalna konformalna radioterapija radi maksimalne poštede zdravih tkiva. Objektivni odgovor pratio se kliničkim neurološkim pregledom te neuroradiološkim praćenjem (MR mozga). Toksičnost, prvenstveno hematološka, praćena je redovitim kontrolama hematopoetskih i biokemijskih parametara. Usprkos napretku u kemoterapijskom liječenju i radioterapiji, rezultati liječenja tumora moždanog debla još uvijek ostaju nezadovoljavajući i zahtijevaju primjenu novih terapijskih postupaka.Primary tumors of the brain and spinal cord make up about 20% of all tumors in children and are the second most common types of pediatric neoplasia after hematology. In children between two and seven years of age the most common types of tumors are tumors of the posterior cranial fossa, including the brainstem tumors. Brain stem tumors are defined primarily due to its anatomic localization, rather than the grade of histological phenotype. Astrocytomas and glioblastomas make up about 50% of all neoplasms while medulloblastomas, ependymomas, kraniofaringeomi, oligodendrogliomas, and germ cell tumors are the rarest. Although in the area of the brainstem can occur all histopathological types of tumors, the occurrence in this region, the typical neuroradiological images and expressed clinical symptoms are more important prognostic factor than histopathological phenotype and degree of differentiation. Total prognosis for brainstem tumors in children is poor, average survival of affected children is 8-10 months. This study includes 10 children in age of 1-7 years with confirmed diagnosis of brain stem tumors by neuroradiology or patohistology in the Children's Hospital in Klaićeva and the Department of Pediatrics, KBC Zagreb who were treated in the period of 2008. - 2012.The primary goal of this study is to determine the objective response to conducted treatment and overall survival of patients. The goal of this study is to determine the toxicity after completion of treatment. All patients were treated with chemotherapy and radiotherapy. Chemotherapy was conducted according to the HIT-GBM protocol or with temozolomide. Radiation therapy was conducted in the area of posterior fossa and the seat of the primary process with total dose of 54 Gy in 27 fractions. Three-dimensional conformal radiotherapy was applied for maximum protection of healthy tissue. Objective response was monitored by clinical neurological examination and neuroradiological monitoring (MRI). Toxicity, primarily hematologic, was monitored by regular controls of hematopoietic and biochemical parameters. Despite advances in chemotherapy treatment and radiotherapy, the results of treatment of brain stem tumors are still poor and application of new therapeutic procedures is required