FABP4 is a candidate marker of cerebellar liponeurocytomas
FABP4 is a candidate marker of cerebellar liponeurocytomas
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DOI：
10.1007/s11060-012-0853-0
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发表时间：
2012-07-01
影响因子：
3.9
通讯作者：
Finocchiaro, Gaetano
中科院分区：
文献类型：
--
作者：
Anghileri, Elena;Eoli, Marica;Finocchiaro, Gaetano
小脑脂质神经细胞瘤（cLPN）是一种非常罕见的中枢神经系统（CNS）肿瘤，最近被认为是一种不同于髓母细胞瘤（MB）的临床和病理实体，并被列入WHO中枢神经系统肿瘤分类的“胶质神经元肿瘤”标题下。与MB相比，cLPN通常发生在成人年龄，预后良好。在这项工作中，我们回顾了在我们机构诊断的两例新型成人cLPN的临床和神经影像学资料；1例患者发生远处转移。我们试图为这种恶性肿瘤寻找新的分子标记。我们发现，与正常成人小脑不同，转录因子NEUROG1（而不是ATOH1）在cLPN中表达，脂肪酸结合蛋白4 （FABP4）通常在脂肪细胞中发现，与正常成人小脑和人MB相比，cLPN明显过表达。这些发现表明，cLPN是小脑祖细胞转化的结果，与小脑颗粒祖细胞不同，并异常分化为脂肪细胞样肿瘤细胞。他们还建议分析FABP4的表达有助于区分cLPN和MB。
Cerebellar liponeurocytoma (cLPN) is a very rare central nervous system (CNS) tumour recently recognized as a clinical and pathological entity distinct from medulloblastoma (MB), and included in the WHO classification of CNS tumours under the heading "glioneuronal tumours". cLPN typically develop in adult age and have a favourable prognosis compared with MB. In this work, we reviewed the clinical and neuroradiological data of two novel cases of adult cLPN diagnosed at our institution; one patient developed distant metastases. We tried to identify novel molecular markers for this malignancy. We found that the transcription factor NEUROG1 (but not ATOH1) is expressed in cLPN, unlike normal adult cerebellum, and that fatty acid binding protein 4 (FABP4), typically found in adipocytes, is significantly overexpressed compared with both normal adult cerebellum and human MB. These findings suggest cLPN occur as a result of transformation of cerebellar progenitors, which are distinct from cerebellar granule progenitors, and aberrantly differentiate into adipocyte-like tumour cells. They also suggest that analysis of FABP4 expression is of help to differentiate cLPN from MB.