Tumor neuroectodérmico primitivo periférico/sarcoma de Ewing extraóseo del hueso temporal

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 malig- nant tumors. In the PNET family, we high light the Ewings 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 Ewings Sarcoma that in- volves the external auditory canal and infiltrates the tempo- ral bone. Surgery followed by postoperative chemotherapy and radiation offers the best local control of the disease.