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000182533 041__ $$aEnglish
000182533 082__ $$a570
000182533 1001_ $$0P:(DE-He78)a975a4d7d3d59cc6ecc829e98bfe0c15$$aGojo, Johannes$$b0$$eFirst author$$udkfz
000182533 245__ $$aRare embryonal and sarcomatous central nervous system tumours: State-of-the art and future directions.
000182533 260__ $$aNew York, NY [u.a.]$$bElsevier$$c2023
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000182533 500__ $$a#EA:B062# / 2023 Nov 7;66(1):104660
000182533 520__ $$aThe introduction of molecular methods into the diagnostics of central nervous system (CNS) tumours and the subsequent deciphering of their molecular heterogeneity has resulted in a significant impact on paediatric neurooncology. Particularly in the field of rare embryonal and sarcomatous CNS tumours, novel tumour types have been delineated and introduced in the recent 5th edition of the WHO classification of CNS tumours. The rarity and novelty of these tumour types result in diagnostic and therapeutic challenges. Apart from distinct histopathological and molecular features, these tumour types exhibit characteristic clinical properties and require different therapeutic approaches for optimal patient management. However, based on the limited availability of clinical data, current therapeutic recommendations have to be based on data from small, predominantly retrospective patient cohorts. Within this article, we provide guidance for diagnostic work-up and clinical management of rare embryonal CNS tumours ('embryonal tumour with multi-layered rosettes', ETMR; 'CNS neuroblastoma, FOXR2-activated', CNS NB-FOXR2; 'CNS tumour with BCOR-ITD, CNS BCOR-ITD) and rare sarcomatous CNS tumours ('primary intracranial sarcoma, DICER1-mutant', CNS DICER1; 'CIC-rearranged sarcoma', CNS CIC). By emphasizing the significant consequences on patient management in paediatric CNS tumours, we want to encourage wide implementation of comprehensive molecular diagnostics and stress the importance for joint international efforts to further collect and study these rare tumour types.
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000182533 650_7 $$2Other$$aCNS tumour
000182533 650_7 $$2Other$$aClinical management
000182533 650_7 $$2Other$$aEmbryonal
000182533 650_7 $$2Other$$aMolecular diagnostics
000182533 650_7 $$2Other$$aPaediatric
000182533 650_7 $$2Other$$aSarcoma
000182533 7001_ $$aKjaersgaard, Mimi$$b1
000182533 7001_ $$aZezschwitz, Barbara V$$b2
000182533 7001_ $$0P:(DE-He78)51bf9ae9cb5771b30c483e5597ef606c$$aCapper, David$$b3$$udkfz
000182533 7001_ $$aTietze, Anna$$b4
000182533 7001_ $$0P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aKool, Marcel$$b5$$udkfz
000182533 7001_ $$aHaberler, Christine$$b6
000182533 7001_ $$aPizer, Barry$$b7
000182533 7001_ $$aHoff, Katja V$$b8
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