Raguin J M, Wendling D, Montcuquet P, Guidet M
Service de Rhumatologie, CHU de Besançon.
Rev Rhum Mal Osteoartic. 1987 Jul-Sep;54(7-9):575-82.
The authors report on two cases of sacrococcygeal chordoma, with complications involving visceral organ metastases and distal bony metastases. Chordomas are malignant tumors which develop in adult subjects which originate from remnants of the embryonic notochord. Sacrococcygeal localization is found in 50% of the 1,300 cases reported in the medical literature, which represents 20% of sacrococcygeal tumors observed. The difficulty and the delay in diagnosing these tumors should decrease by routine CAT scan examination. Diagnostic certainty is based on histological examination, often suggestive of the diagnosis (physaliphore-like cells), possibly combined with ultrastructure and immunohistological study. The clinical course involves local recurrences, but there is a real risk of metastatic development, notably in the case of sacrococcygeal chordomas, with their incidence estimated at 17.5% of cases. Sometimes developing in later stages of their course, histological findings are similar to those of the initial lesion. Organs which are frequently the sites of metastases are the lung (48% of cases) and bone (26%), then the lymphatic organs, the liver, and subcutaneous tissue... Therapeutic management is unsatisfactory, with ideally, complete surgical excision of the initial tumor of its size permits and hence early diagnosis of this condition is a prerequisite for good results.
作者报告了两例骶尾部脊索瘤病例，伴有内脏器官转移和远端骨转移等并发症。脊索瘤是发生于成人的恶性肿瘤，起源于胚胎脊索的残余组织。在医学文献报道的1300例病例中，50%为骶尾部定位，占所观察到的骶尾部肿瘤的20%。通过常规计算机断层扫描（CAT）检查，这些肿瘤诊断的难度和延迟应会降低。诊断的确切性基于组织学检查，通常提示诊断（泡状核细胞样细胞），可能还需结合超微结构和免疫组织学研究。临床病程包括局部复发，但存在转移发展的实际风险，尤其是骶尾部脊索瘤，其转移发生率估计为病例的17.5%。转移有时在病程后期发生，组织学表现与初始病变相似。常见的转移部位是肺（48%的病例）和骨（26%），其次是淋巴器官、肝脏和皮下组织……治疗处理并不理想，理想情况下，应在肿瘤大小允许的情况下完全手术切除初始肿瘤，因此早期诊断这种疾病是取得良好治疗效果的前提条件。
