Nengsu A, Bernat I, Brocheriou-Spelle I, Martin-Duverneuil N, Lamas G, Soudant J, Tankéré F
Service d'ORL et de Chirurgie Cervico-Faciale, Hôpital de la Pitié-Salpêtrière, 47 Boulevard de l'Hôpital, 75013 Paris, France.
Ann Otolaryngol Chir Cervicofac. 2002 Nov;119(5):287-92.
Ollier's disease is a constitutional pathology of unknown etiology. It is characterized by bone dysplasia generating numerous enchondromas. The malignant degeneration of this dysplasia is well known. The aim of this article is to study the diagnostic, therapeutic and prognostic characteristics of these lesions.
We report a case of parapharyngeal chondrosarcoma extended to the base of the skull in a patient with Ollier's disease. The treatment was a surgical removal by a cervicotransoral incision combined with a preauricular incision and with a mastoidectomy. It was therefore possible to control the skull base, the parapharyngeal space, the infratemporal fossa and the major neurovascular structures. The removal of the lesion was completed at the level of the clivus and sphenoid with optics (30 and 70 degrees ). We discuss this treatment and the follow up on the bases of literature data.
The neoplastic degeneration of enchondromas is estimated between 25 to 50% of cases. The most frequent location is the pelvic bones. Chondrosarcomas are slow growing tumors and their metastatic potential is less significant as we note it in our case report. Their diagnostic is essentially based on histological criteria's and their treatment is surgical.
Chondrosarcomas of the ENT area occurring with Ollier's disease is rare. Their prognostic is good if the surgical treatment is well done.
Ollier病是一种病因不明的先天性疾病。其特征为骨发育异常，产生大量内生软骨瘤。这种发育异常的恶性变是众所周知的。本文旨在研究这些病变的诊断、治疗和预后特征。
我们报告1例患有Ollier病的患者发生的咽旁软骨肉瘤，病变扩展至颅底。治疗方法是通过颈-经口切口联合耳前切口及乳突切除术进行手术切除。因此可以控制颅底、咽旁间隙、颞下窝及主要神经血管结构。在斜坡和蝶骨水平借助30度和70度光学设备完成病变切除。我们根据文献资料讨论了这种治疗方法及随访情况。
内生软骨瘤的恶变率估计在25%至50%之间。最常见的部位是骨盆骨。软骨肉瘤是生长缓慢的肿瘤，正如我们在病例报告中所注意到的，其转移潜能较小。其诊断主要基于组织学标准，治疗方法为手术治疗。
Ollier病伴发的耳鼻喉区域软骨肉瘤罕见。如果手术治疗得当，其预后良好。
