Rapidis A D, Archondakis G, Anteriotis D, Skouteris C A
Department of Maxillofacial Surgery, Greek Anticancer Institute, Saint Savvas Hospital, Athens.
J Craniomaxillofac Surg. 1997 Dec;25(6):322-7. doi: 10.1016/s1010-5182(97)80034-0.
Chondrosarcomas are malignant mesenchymal tumours occurring only rarely in the bones of the cranium. Less than 5% of all chondrosarcomas are located in the head and neck area and their commonest location is the ethmoids and the sphenoid sinus. They are slow-growing tumours with low malignancy rate and unclear histopathogenesis. The prevailing hypothesis is that they arise from cartilaginous remnants in the petro-clival, spheno-occipital and fronto-nasal synchondroses. Diagnosis is only made after biopsy since clinical signs and symptoms and radiological findings are not pathognomonic. Symptomatology mainly derives from tumour encroachment and infiltration of adjacent intracranial structures. Surgery is the treatment of choice, while radiotherapy has an adjunctive role. Chemotherapy is not effective. Partial tumour excision to alleviate symptoms is an acceptable surgical technique since diagnosis is usually late, but treatment can be repeated when recurrence occurs. During the last year, two cases of chondrosarcoma of the skull base were treated in our institution. Both patients were female, aged 62 and 73, respectively. Computerized Tomography and Magnetic Resonance Imaging were inconclusive and diagnosis was established after biopsy. Treatment for both cases was surgical, with partial excision due to intracranial involvement of the internal carotid artery. In one case, a temporal approach was used, whereas the other patient was operated on via a naso-orbital approach. One patient received postoperative radiotherapy. The postoperative course was uneventful in both patients and marked clinical improvement was noted 18 months and 1 year after surgery. We support the view that partial resection of chondrosarcomas of the base of the skull to alleviate symptoms is an acceptable surgical treatment.
软骨肉瘤是一种恶性间充质肿瘤，仅罕见发生于颅骨。所有软骨肉瘤中不足5%位于头颈部，其最常见的部位是筛骨和蝶窦。它们是生长缓慢、恶性率低且组织发病机制不明的肿瘤。目前流行的假说是，它们起源于岩枕、蝶枕和额鼻软骨结合处的软骨残余。仅在活检后才能确诊，因为临床体征和症状以及影像学表现并无特异性。症状主要源于肿瘤对相邻颅内结构的侵犯和浸润。手术是首选治疗方法，放疗起辅助作用。化疗无效。由于通常诊断较晚，部分肿瘤切除以缓解症状是一种可接受的手术技术，但复发时可重复治疗。去年，我们机构治疗了2例颅底软骨肉瘤。两名患者均为女性，年龄分别为62岁和73岁。计算机断层扫描和磁共振成像结果不明确，活检后确诊。两例患者均接受手术治疗，由于颈内动脉颅内受累而进行了部分切除。1例采用颞部入路，另1例患者通过鼻眶入路手术。1例患者接受了术后放疗。两名患者术后过程均顺利，术后18个月和1年时临床症状明显改善。我们支持这样的观点，即颅骨底部软骨肉瘤的部分切除以缓解症状是一种可接受的外科治疗方法。
