Department of Radiological, Pathological and Oncological Sciences, Sapienza University, Rome, Italy.
Neuropathology. 2011 Oct;31(5):549-55. doi: 10.1111/j.1440-1789.2010.01196.x. Epub 2011 Feb 1.
Peripheral primitive neuroectodermal tumor/Ewing's sarcoma (ES) (pPNET/ES) of intracranial origin are very rare. These tumors are characterized by specific translocations involving a gene on chromosome 22q12, the most common being t(11;22) (q24;q12). We report a case of 37-year-old man with pPNET/ES arising in the meninges and bearing the rare translocation t(21;22) (q22;q12). The tumor was composed of sheets and nests of monotonous small cells with round to oval nuclei, finely dispersed chromatin, small nucleolus and scant cytoplasm. We discuss the importance of the differential diagnosis with central primitive neuroectodermal tumors (cPNET).
颅内起源的外周原始神经外胚层肿瘤/尤文肉瘤（pPNET/ES）非常罕见。这些肿瘤的特征是涉及染色体 22q12 上基因的特定易位，最常见的是 t(11;22)（q24;q12）。我们报告了一例 37 岁男性，脑膜起源的 pPNET/ES 伴罕见的易位 t(21;22)（q22;q12）。肿瘤由片状和巢状排列的单调小圆形至卵圆形细胞组成，染色质细颗粒状，核仁小，细胞质稀少。我们讨论了与中枢原始神经外胚层肿瘤（cPNET）的鉴别诊断的重要性。
