Kumar Raj, Singhal Namit, Jaiswal Shishir K, Mahapatra A K
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Pediatr Neurosurg. 2007;43(5):364-8. doi: 10.1159/000106384.
To study the outcome and recurrence in supratentorial anaplastic ependymoma.
Sixteen cases of supratentorial anaplastic ependymoma were reviewed. The average age of presentation was 8.2 years ranging from 1 to 16 years of age. The mean duration between the onset of first symptoms to time of presentation was 4.2 months. Follow-up ranged from 5 to 58 months with a mean of 16.8 months.
Gross total excision of tumor was achieved in 14 cases, as judged on the basis of intraoperative impression and confirmed with postoperative contrast MR or CT scan. There were 2 unfortunate deaths in the series, one as result of disseminated intravascular coagulation in view of massive blood loss and the other child had evidence of central transtentorial herniation preoperatively which failed to recover. Postoperative cranial radiotherapy was offered to all the 14 remaining cases. Twelve of 14 cases showed evidence of recurrence at follow-up. Recurrence occurred as early at 6 months and as late as 58 months. Only 2 children were recurrence free at follow-up of 6 months and 14 months, respectively. Two operated and irradiated cases of differentiated ependymomas (grade II) developed anaplastic recurrence at follow-up of 5 years and 9 years, respectively, suggesting a malignant transformation of tumor at follow-up. Four out of 12 cases were reoperated for their recurrence in view of localized nature of recurrence and good Karnofsky's performance status.
It is obvious that anaplastic ependymomas of the supratentorial compartment are aggressive tumors with high rates of recurrence even after gross total excision and irradiation. Gross total excision and postoperative irradiation are not effective in preventing early recurrence in anaplastic ependymomas, and other factors affecting the outcome need to be analyzed.
研究幕上间变性室管膜瘤的治疗结果及复发情况。
回顾性分析16例幕上间变性室管膜瘤患者。患者就诊时的平均年龄为8.2岁，年龄范围为1至16岁。首发症状出现至就诊的平均时间为4.2个月。随访时间为5至58个月，平均为16.8个月。
根据术中情况判断，14例患者实现了肿瘤全切，并经术后增强磁共振成像（MR）或计算机断层扫描（CT）证实。该系列中有2例不幸死亡，1例因大量失血导致弥散性血管内凝血死亡，另1例患儿术前有中央幕下疝的证据，术后未能恢复。其余14例患者均接受了术后颅脑放疗。14例患者中有12例在随访时出现复发迹象。复发最早在6个月出现，最晚在58个月出现。只有2例患儿在分别随访6个月和14个月时未复发。2例接受手术及放疗的分化型室管膜瘤（II级）患者在随访5年和9年时分别出现间变复发，提示随访期间肿瘤发生了恶性转化。12例复发患者中有4例因复发部位局限且卡氏功能状态良好而接受了再次手术。
显然，幕上间变性室管膜瘤是侵袭性肿瘤，即使在肿瘤全切及放疗后仍有较高的复发率。肿瘤全切及术后放疗对预防间变性室管膜瘤的早期复发无效，需要分析影响治疗结果的其他因素。
