Coppens Jeroen R, Ric Harnsberger H, Finn Michael A, Sharma Pramod, Couldwell William T
Department of Neurosurgery, University of Utah, Salt Lake City, UT 84132, USA.
Acta Neurochir (Wien). 2009 Aug;151(8):901-7. doi: 10.1007/s00701-009-0253-1. Epub 2009 Mar 18.
Chordomas are rare tumors derived from notochordal remnants. The authors report on a series of three cases of primary familial oronasopharyngeal chordomas treated at our institution.
A retrospective chart review was completed of the three cases of primary familial oronasopharyngeal chordoma treated at the University of Utah.
All three patients (100%) were neurologically intact and presented with nasal obstruction. The patients ranged in age from 5 to 65 years and were first-degree relatives. None of the patients had bony erosion of the skull base on imaging, and all of the patients' tumors connected with the skull base via a tract. All three patients were treated with a wide excision combined with drilling of the involved skull base. They all tolerated the procedure without any complications and remain tumor free with a follow-up of 12 months to 4.5 years.
Primary oronasopharyngeal chordomas are rare tumors that may present without bony erosion of the skull base. A wide excision with drilling of the involved bony structures may offer an oncologic cure.
脊索瘤是源自脊索残余组织的罕见肿瘤。作者报告了在本机构治疗的一系列3例原发性家族性口咽脊索瘤病例。
对犹他大学治疗的3例原发性家族性口咽脊索瘤病例进行了回顾性病历审查。
所有3例患者（100%）神经功能完好，均表现为鼻塞。患者年龄在5至65岁之间，均为一级亲属。影像学检查中，所有患者均无颅底骨质侵蚀，且所有患者的肿瘤均通过一条通道与颅底相连。所有3例患者均接受了广泛切除并联合对受累颅底进行钻孔。他们均耐受该手术，无任何并发症，随访12个月至4.5年，均无肿瘤复发。
原发性口咽脊索瘤是罕见肿瘤，可能在无颅底骨质侵蚀的情况下出现。对受累骨结构进行广泛切除并钻孔可能实现肿瘤根治。
