Germinatívne nádory pineálnej oblasti: prehľad
:
V. Miskovska 1; V. Usakova 2; B. Vertakova-Krakovska 2; B. Mrinakova 2; V. Lehotská 3; M. Chorvath 4; B. Rychlý 5; J. Steno 6; D. Ondrus 1
:
1st Department of Oncology, Comenius University, Faculty of Medicine, St. Elisabeth Cancer Institute, Bratislava, Slovak Republic
1; Department of Medical Oncology, St. Elisabeth Cancer Institute, Bratislava, Slovak Republic
2; 2nd Department of Radiodiagnostics, Comenius University, Faculty of Medicine, St. Elisabeth Cancer Institute, Bratislava, Slovak Republic
3; Department of Radiation Oncology, Slovak Medical University, St. Elisabeth Cancer Institute, Bratislava, Slovak Republic
4; Cytopathos, Bratislava, Slovak Republic
5; Department of Neurosurgery, Comenius University, Faculty of Medicine, University Hospital, Bratislava, Slovak Republic
6
:
Klin Onkol 2013; 26(1): 19-24
:
Reviews
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Obdrženo: 25. 11. 2012
Přijato: 2. 12. 2012
Úvod:
Primárne intrakraniálne germinatívne tumory predstavujú zriedkavú skupinu nádorov, ktoré sa vyskytujú v detstve a u mladých dospelých. WHO klasifikácia delí intrakraniálne tumory na germinómy a negerminómy. Najčastejšia lokalita týchto nádorov je pineálna a supraselárna oblasť. Klinické príznaky a symptómy závisia od lokalizácie tumoru, najčastejšie sú to príznaky v dôsledku zvýšeného intrakraniálnho tlaku, Parinaudov syndróm, bitemporálna hemianopsia, príznaky z endokrinného deficitu. Zobrazovacím vyšetrením voľby pri diagnostike intrakraniálnych germinatívnych tumorov je MRI vyšetrenie mozgu s použitím kontrastnej látky gadolinium. Zobrazovacie vyšetrenia však neposkytnú dostatočnú informáciu o type nádoru, k tomu je potrebná bioptizácia. Výnimkou sú prípady s charakteristicky zvýšenými hladinami nádorových markerov (AFP a β-hCG) stanovenými v sére a cerebrospinálnom moku.
Klinický prípad:
U 26-ročného pacienta so zhoršeným zrakom s poruchou zaostriť a svetloplachosťou, výraznou únavou a ospalosťou, nevoľnosťou, občasným zvracaním, s intermitentnými bolesťami hlavy a klinicky prítomným Parinaudovým syndróm sa zistil germinatívny nádor pineálnej oblasti. Zrealizované MR vyšetrenie mozgu ukázalo tumoróznu expanziu v pineálnej oblasti a v pravej časti mezencefala. Bola vykonaná radikálna exstirpácia tumoru v pineálnej oblasti. Pri kontrolnom MR vyšetrení mozgu sa zistila recidíva ochorenia. Pacient absolvoval kraniospinálne ožiarenie s následnou pooperačnou chemoterapiou (schéma cisplatina a etoposid), celkovo tri cykly. V súčasnej dobe je pacient 30 mesiacov po ukončení úspešnej onkologickej liečby v klinickej remisii ochorenia.
Záver:
Liečba a prognóza tohto nádorového ochorenia je rozdielna v jednotlivých skupinách. Germinómy majú lepšie prežívanie než negerminómy. 5-ročné prežívanie germinómov po aplikácii samotnej rádioterapie bolo > 90 %. Pridanie chemoterapie viedlo k zníženiu dávky a zmenšeniu ožarovanej oblasti, s dosiahnutím menších nežiadúcich účinkov a bez zníženia kurability. Negerminómy sú menej rádiosenzitívne než germinómy, ale použitím adjuvantnej chemoterapie sa dosiahlo zlepšenie prežívania. Naďalej však menežment týchto nádorov ostáva kontroverzný.
Kľúčové slová:
pinealóm – germinóm – mozgové nádory – diagnóza – liečba
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Paediatric clinical oncology Surgery Clinical oncologyArticle was published in
Clinical Oncology
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