后颅凹FET-CREB融合阳性型间叶性肿瘤1例并文献复习
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【中图分类号】 R739.41 【文献标志码】 B 【文章编号】 1672-7770（2026）01-0114-04
Posterior cranial fossa mesenchymal tumor with FET-CREB fusion-positive：report of 1 case and literature review XU Yinhao，ZHANG Xulian， ZHENG Darui， WANG Juan， ZHANG Da， HU Xiao. Department of Radiology， Nanjing Brain Hospital Affiliated to Nanjing Medical University，Nanjing 21oo29，China Corresponding author： Hu Xiao
Abstract： ObjectiveTo investigate the imaging and pathological characteristics of intracranial mesenchymal tumor（IMT）harboring FET-CREB fusion-positive.MethodsThe CTand MRI imaging features，histomorphology， immunophenotype，and molecular genetic characteristicsof an IMT positive forFET-CREB fusion patient were analyzedretrospectively.Therelevantliteraturewerealsoreviewed.ResultsThetumorwaslocated in theright posteriorcranial fossa，exhibitingawell-defined shape.CTshowedarelativelyhomogeneous，ovaloid，isodensemass with significant peritumoral edema and mass effect.MRI revealeda tumor with heterogeneous signal intensity， demonstrating prominent enhancement post-contrastadministration，accompanied bypatchy non-enhancing areas.No dural tail sign was observed.Initial diagnosison imaging wasmeningioma orsolitary fibroustumor. Histopathologically，the tumorcelswerearranged ina lobularpattern withinamyxoid matrixrich inmucin.The tumortissue was highly vascularized and showed focalcollagenization.Immunohistochemistryshowed the tumorcells were positive for EMA，VIM，H3K27me3，and INI1.Focal positivity was observed for S1O，SSTR2，DES，CD99， CD68，CD63，and EGFR.The pathological findings were consistent with a myxoid tumor. Subsequent molecular genetictesting confirmedanintracranial mesenchymal tumor positiveforFET-CREB fusion.ConclusionsIMTs with FET-CREB fusion-positive can present with atypical imaging features.The definitive diagnosisrelieson comprehensive histopathological evaluation supplemented by molecular genetic testing.
Key words： intracranial mesenchymal tumor； FET-CREB fusion-positive；neuroimaging；pathology
伴有基因FET-CREB融合阳性型的颅内间叶性肿瘤（intracranialmesenchymal tumor，IMT）是2021年第五版WHO中枢神经系统肿瘤分类中分化不确定肿瘤的一个新亚类[1]。（剩余9260字）
