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000177381 041__ $$aEnglish
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000177381 1001_ $$0P:(DE-He78)8e1d81963d2c9b963058050651d55485$$aJenseit, Anne Lena$$b0$$eFirst author$$udkfz
000177381 245__ $$aEZHIP: a new piece of the puzzle towards understanding pediatric posterior fossa ependymoma.
000177381 260__ $$aHeidelberg$$bSpringer$$c2022
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000177381 500__ $$a#EA:B062#LA:B062# / 2022 Jan;143(1):1-13
000177381 520__ $$aEpendymomas (EPN) are tumors of the central nervous system (CNS) that can arise in the supratentorial brain (ST-EPN), hindbrain or posterior fossa (PF-EPN) or anywhere in the spinal cord (SP-EPN), both in children and adults. Molecular profiling studies have identified distinct groups and subtypes in each of these anatomical compartments. In this review, we give an overview on recent findings and new insights what is driving PFA ependymomas, which is the most common group. PFA ependymomas are characterized by a young median age at diagnosis, an overall balanced genome and a bad clinical outcome (56% 10-year overall survival). Sequencing studies revealed no fusion genes or other highly recurrently mutated genes, suggesting that the disease is epigenetically driven. Indeed, recent findings have shown that the characteristic global loss of the repressive histone 3 lysine 27 trimethylation (H3K27me3) mark in PFA ependymoma is caused by aberrant expression of the enhancer of zeste homolog inhibitory protein (EZHIP) or in rare cases by H3K27M mutations, which both inhibit EZH2 thereby preventing the polycomb repressive complex 2 (PRC2) from spreading H3K27me3. We present the current status of the ongoing work on EZHIP and its essential role in the epigenetic disturbance of PFA biology. Comparisons to the oncohistone H3K27M and its role in diffuse midline glioma (DMG) are drawn, highlighting similarities but also differences between the tumor entities and underlying mechanisms. A strong focus is to point out missing information and to present directions of further research that may result in new and improved therapies for PFA ependymoma patients.
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000177381 650_7 $$2Other$$aDMG
000177381 650_7 $$2Other$$aEZHIP
000177381 650_7 $$2Other$$aH3K27M
000177381 650_7 $$2Other$$aH3K27me3
000177381 650_7 $$2Other$$aPFA ependymoma
000177381 650_7 $$2Other$$aPRC2
000177381 7001_ $$0P:(DE-He78)dd60ad1c45db7fcacc5be5238ad17e7d$$aCamgoz, Aylin$$b1$$eFirst author$$udkfz
000177381 7001_ $$0P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aPfister, Stefan M$$b2$$udkfz
000177381 7001_ $$0P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aKool, Marcel$$b3$$eLast author$$udkfz
000177381 773__ $$0PERI:(DE-600)1458410-4$$a10.1007/s00401-021-02382-4$$n1$$p1-13$$tActa neuropathologica$$v143$$x1432-0533$$y2022
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