外周型原始神经外胚层肿瘤1例

原始神经外胚叶肿瘤是一种罕见的、起源于原始神经上皮的恶性小圆形细胞肿瘤,具有多方向分化能力,可发展为神经元性的、胶质细胞性的、甚至间叶组织性质的肿瘤.根据发病部位将其分为中枢型和外周型两种,中枢型多发于脑和脊髓,外周型多见于胸壁、四肢,发生于脊柱者少见.本文就外周型原始神经外胚层肿瘤1例做病例报道,总结该类肿瘤的特点,肿瘤密度/信号不均匀,轻至中度强化,预后差,临床治疗手段主要为外科手术辅以放化疗,术前行增强CT对手术可切除性评估非常有价值.

One Case of Peripheral Primitive Neuroectodermal Tumors

Primitive neuroectodermal tumors （PNET） are rare malignant small round cell tumors which originated in primitive neuroepithelial. It can develop into the nature of neuron, glial cell, and even mesenchymal tissue tumors. PNET can be divided into two types, which are the central type PNET （cPNET） and the peripheral type （pPNET） according to the location of tumor. The central type mainly develop in the brain and spinal cord, and the peripheral-type is common in the chest, limbs, but is rare in the spine. One case of pPNET will be reported. This case report is about the PNET, summarizing the characteristic of the PNET, the dense/signal is nonuniform, mild or medium enhanced, and the prognosis is poor, surgery assisted with radiotherapy and chemotherapy is the main clinical treatment, the enhanced CT is valuable in assessing the surgical resection.