Rivierez M, Oueslati S, Philippon J, Pradat P, Foncin J F, Muckensturm B, Dorwling-Carter D, Cornu P
Service de Neurochirurgie, Hôpital de la Salpêtrière, Paris.
Neurochirurgie. 1990;36(2):96-107.
Twenty cases of ependymomas of the intradural filum terminale in adults have been reviewed. Their pathology was quite uniform, of a myxopapillary type, similar to the low grade ependymoma described by Kernohan, which represent about 23% of the tumours of cauda equina. Mean age of the patients was 35.7 years. Mean time between the first symptom and the diagnosis was 46 months. Clinical symptoms were often non specific, with low back pain and radiculalgias. At the time of operation, clinical signs were essentially motor deficits usually moderate (11 cases), sphincter disturbances (10 cases), and sensory loss (9 cases). In 3 patients with rapid worsening, an intratumoral haemorrhage was found. In 2 other cases, intracranial hypertension was the main symptom: in the first, it was related to hydrocephalus probably caused by spinal subarachnoid haemorrhage; in the second, there was no ventricular dilatation. In this series, neuroradiological examinations had consisted mainly in myelographies. C.T. scan has been performed in 3 patients; in only one case it has allowed to visualize a presacral extension. One patient had preoperative M.R.I.: the association of an expansive lesion with upper cyst in conus medullaris and presence of blood in the sacral area permitted the diagnosis of ependymoma of the filum terminalis. The average size of the tumours was 8 cm. Total removal has been possible in 15 cases (and in 2 of the 5 giant tumours), subtotal removal in 2 cases, and partial removal in 3 cases. In 4 patients where existed an intraspinal cord extension above the conus, it has been resected completely, except for one case with recurrence. Patients with a total removal had a good functional recovery (13/15). No recurrence has been observed in this group. In conclusion, with M.R.I., one may hope an earlier diagnosis, condition of radical surgery. So, radiotherapy which is not without risk, could be avoided.
回顾了20例成人硬脊膜内终丝室管膜瘤病例。其病理表现较为一致,为黏液乳头型,类似于克诺汉描述的低级别室管膜瘤,约占马尾肿瘤的23%。患者的平均年龄为35.7岁。首发症状至诊断的平均时间为46个月。临床症状通常不具特异性,表现为腰痛和神经根痛。手术时,临床体征主要为运动功能障碍(通常为中度,11例)、括约肌功能障碍(10例)和感觉丧失(9例)。3例病情迅速恶化的患者发现肿瘤内出血。另外2例中,颅内高压为主要症状:第一例与可能由脊髓蛛网膜下腔出血引起的脑积水有关;第二例无脑室扩张。在该系列病例中,神经放射学检查主要为脊髓造影。3例患者进行了CT扫描;仅1例显示有骶前延伸。1例患者术前行MRI检查:髓内圆锥部有一膨胀性病变并伴有上囊肿,骶部有血液,据此诊断为终丝室管膜瘤。肿瘤的平均大小为8 cm。15例(5例巨大肿瘤中的2例)实现了全切,2例次全切除,3例部分切除。4例脊髓圆锥上方存在脊髓内延伸的患者,除1例复发外均完全切除。全切患者功能恢复良好(13/15)。该组未观察到复发。总之,借助MRI有望实现早期诊断,这是根治性手术的条件。因此,可以避免有风险的放疗。