Genomic Characterization of Radiation-Induced Intracranial Undifferentiated Pleomorphic Sarcoma.

Case Reports in Genetics Pub Date : 2021-03-08 eCollection Date: 2021-01-01 DOI:10.1155/2021/5586072
Christopher S Hong, Edwin Partovi, James Clune, Anita Huttner, Henry S Park, Sacit Bulent Omay
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Abstract

Intracranial undifferentiated pleomorphic sarcoma remains a rare pathology within the sarcoma literature that may arise primarily or secondary after radiation therapy. Despite first-line treatment with maximal surgical resection, followed by nonstandardized adjuvant chemotherapy/radiation regimens, clinical prognosis remains exceedingly poor. Furthermore, there is a lack of genetic or molecular characterization to guide potential for targeted therapies. We present genomic analysis of a radiation-induced intracranial undifferentiated pleomorphic sarcoma in an 83-year-old woman with notable KIT and PDGFRA alterations. Further similar genomic studies of intracranial pleomorphic sarcoma are needed to develop better therapies for this rare but challenging disease entity.

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辐射诱导颅内未分化多形性肉瘤的基因组特征。
颅内未分化多形性肉瘤在肿瘤文献中仍然是一种罕见的病理,可能在放射治疗后主要或继发发生。尽管在一线进行了最大限度的手术切除,随后进行了非标准化的辅助化疗/放疗方案,但临床预后仍然非常差。此外,缺乏遗传或分子特征来指导靶向治疗的潜力。我们报告了一名83岁女性放射诱导的颅内未分化多形性肉瘤的基因组分析,其KIT和PDGFRA明显改变。颅内多形性肉瘤需要进一步类似的基因组研究,以开发更好的治疗这种罕见但具有挑战性的疾病实体。
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