Boccardo M, Telera S, Vitali A
U.O. di Neurochirurgia-Neurotraumatologia, Azienda Ospedaliera Santa Corona, Pietra Ligure, Italy.
Neurochirurgie. 2003 Dec;49(6):605-10.
We report a rare case of the tanycytic variant of intramedullary ependymoma. Tanycytes are the common progenitor cells of both ependymal cells and astrocytes. These particular elongate unipolar and bipolar ependymal cells extend from the ventricular lumen to the surface of the nervous system. It is extremely important, in terms of both management and prognosis, to distinguish intramedullary tanycytic ependymomas from intramedullary astrocytomas although a correct histological diagnosis may be difficult since tanycytes resemble astrocytes.
A 39-year-old woman underwent surgical treatment for a cervical intramedullary tumor in our department. Although pathological examination of frozen sections was suggestive of low-grade astrocytoma, the definitive histological diagnosis was "tanycytic" ependymoma, a tumor characterized by poor cellularity, elongated elements mixed with fibrillary components, rare pseudo-rosettes and mixed astro-ependymal aspects. Since a complete resection was performed at surgery, no further treatment was proposed. After a follow-up period of two years the patient is free from recurrence.
Tanycytic ependymomas should be managed in the same way as "ordinary" ependymomas, since there is no current evidence suggesting that these morphologically distinct tumors differ in terms of biological behavior. Increased awareness of this transitional form of intramedullary ependymoma among neurosurgeons and pathologists may avoid incorrect surgical approaches and postoperative treatment.
我们报告一例罕见的髓内室管膜瘤的伸长细胞变体病例。伸长细胞是室管膜细胞和星形胶质细胞的共同祖细胞。这些特殊的细长单极和双极室管膜细胞从脑室腔延伸至神经系统表面。尽管由于伸长细胞与星形胶质细胞相似，正确的组织学诊断可能困难，但从治疗和预后角度而言，将髓内伸长细胞型室管膜瘤与髓内星形细胞瘤区分开来极为重要。
一名39岁女性在我科接受了颈椎髓内肿瘤的手术治疗。尽管冰冻切片的病理检查提示为低级别星形细胞瘤，但最终组织学诊断为“伸长细胞型”室管膜瘤，该肿瘤的特征为细胞密度低、伸长成分与纤维成分混合、罕见假菊形团以及星形-室管膜混合表现。由于手术时进行了完整切除，未建议进一步治疗。经过两年的随访，患者无复发。
伸长细胞型室管膜瘤的治疗方式应与“普通”室管膜瘤相同，因为目前没有证据表明这些形态学上不同的肿瘤在生物学行为方面存在差异。神经外科医生和病理学家提高对这种髓内室管膜瘤过渡形式的认识，可能避免不正确的手术方法和术后治疗。
