Germinatívne nádory pineálnej oblasti: prehľad

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

Autoři: 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
Působiště autorů: 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
Vyšlo v časopise: Klin Onkol 2013; 26(1): 19-24
Kategorie: Přehledy

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Obdrženo: 25. 11. 2012

Přijato: 2. 12. 2012

Souhrn

Kľúčové slová:
pinealóm – germinóm – mozgové nádory – diagnóza – liečba

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