Approaches for prevention of tumors in patients with rhabdoid tumor predisposition syndrome.

IF 3.7 Q1 CLINICAL NEUROLOGY Neuro-oncology advances Pub Date : 2024-09-12 eCollection Date: 2024-01-01 DOI:10.1093/noajnl/vdae158
Anupa Geethadevi, Eric H Raabe
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Abstract

Patients with rhabdoid tumor predisposition syndrome (RTPS) harbor germline alterations in the epigenetic regulator genes SMARCB1 or SMARCA4. Patients usually present with atypical teratoid/rhabdoid tumor (AT/RT) of the brain or malignant rhabdoid tumor (MRT) arising outside the central nervous system. Intensive treatment can lead to remissions, however tumors frequently recur or synchronous or metachronous tumors appear. A maintenance or secondary prevention regimen may prevent these aggressive tumors. Potential maintenance regimens may include low-dose traditional chemotherapy or different epigenetic therapies designed to target the epigenetic imbalance that drives RTs. We here review several potential maintenance regimens that may be useful in RTPS.

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预防横纹肌瘤易感综合征患者罹患肿瘤的方法。
横纹肌瘤易感综合征(RTPS)患者的表观遗传调节基因 SMARCB1 或 SMARCA4 存在种系改变。患者通常表现为脑部非典型畸胎瘤/横纹肌瘤(AT/RT)或中枢神经系统外的恶性横纹肌瘤(MRT)。强化治疗可使病情缓解,但肿瘤经常复发,或出现同步或并发肿瘤。维持或二级预防方案可预防这些侵袭性肿瘤。潜在的维持治疗方案可能包括低剂量传统化疗或不同的表观遗传学疗法,这些疗法的目的是针对导致 RTs 的表观遗传学失衡。我们在此回顾几种可能适用于 RTPS 的潜在维持治疗方案。
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CiteScore
6.20
自引率
0.00%
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0
审稿时长
12 weeks
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