Souvirón Encabo R, Arenas Britez O, Gil R, Pérez-Luengo E, Scola Yurrita B
Hospital General Universitario Gregorio Marañón, Madrid.
Acta Otorrinolaringol Esp. 2005 Jan;56(1):38-40. doi: 10.1016/s0001-6519(05)78568-x.
The term primitive neuroectodermal tumor (PNET) should be included in the differential diagnosis of small round blue cell tumors, which come from the neural crest and explain their neuroectodermal character. This is one of the most indifferentiated histological types of malignant tumors. In the PNET family, we high light the Ewing's Sarcoma, that is frecuently located in bony sites and has a less frequent localization in soft tissues. We focus on one atypic case of PNET/Extraosseus Ewing's Sarcoma that involves the external auditory canal and infiltrates the temporal bone. Surgery followed by postoperative chemotherapy and radiation offers the best local control of the disease.
原始神经外胚层肿瘤（PNET）这一术语应纳入小圆蓝细胞肿瘤的鉴别诊断中，这类肿瘤起源于神经嵴并解释了其神经外胚层特征。这是恶性肿瘤中最未分化的组织学类型之一。在PNET家族中，我们重点关注尤因肉瘤，其常位于骨部位，在软组织中的定位较少见。我们聚焦于一例累及外耳道并浸润颞骨的非典型PNET/骨外尤因肉瘤病例。手术联合术后化疗和放疗可实现对该疾病的最佳局部控制。
