Dysembryoplastic neuroepithelial tumors in childhood
Dysembryoplastic neuroepithelial tumors in childhood
复制标题
儿童期胚胎发育不良性神经上皮肿瘤
作者：
M. Nolan;R. Sakuta;N. Chuang;H. Otsubo;J. Rutka;O. Snead;C. Hawkins;S. Weiss
背景资料：胚胎发育不良性神经上皮肿瘤（DNT）与药物难治性癫痫相关，手术切除后预后良好。作者描述了经病理证实的DNT手术切除后儿童的临床、影像学和病理学特征和结局，以确定术后癫痫复发的预后特征。方法：我们检索了1993年至2002年儿童医院的神经病学、神经外科和病理学数据库，以回顾性地识别确诊为DNT和癫痫发作的儿童。术后癫痫复发的风险因素在12个月和长期随访时进行了检查。结果：在确定的26名儿童中（手术时平均年龄10.0岁），22名儿童（85%）在12个月时癫痫发作结局良好（1级）。在较长的随访期（平均4.3，范围1.0至11.0年），只有16例（62%）保持无复发。在24名术后MRI可用的儿童中，有15名明显存在残留DNT。3例患儿肿瘤复发。在12个月的随访中，年龄较大（>10岁）和癫痫持续时间较长（>2年）与癫痫复发相关。残留肿瘤的存在是长期随访时癫痫复发的风险因素（p = 0.02）。结论：患有DNT和癫痫的儿童可能受益于手术治疗;然而，癫痫发作的结果并不总是有利的。虽然大多数儿童在手术切除DNT后仍然没有癫痫发作，但相当多的儿童有复发性癫痫发作。手术时年龄较大和癫痫持续时间较长会影响短期结果。肿瘤残留是癫痫预后不良的重要危险因素。肿瘤可以复发。
Background: Dysembryoplastic neuroepithelial tumors (DNTs) are associated with medically intractable epilepsy and a favorable prognosis after surgical resection. The authors describe the clinical, radiologic, and pathologic characteristics and outcomes in children after surgical resection of pathologically confirmed DNT to ascertain prognostic features for seizure recurrence following surgery. Methods: Neurology, neurosurgery, and pathology databases from 1993 to 2002 at the Hospital for Sick Children were searched to retrospectively identify children with confirmed DNT and presentation with seizures. Risk factors for postoperative seizure recurrence were examined with respect to seizure outcome at 12 months and long-term follow-up. Results: Of the 26 children identified (mean age at surgery 10.0 years) seizure outcome was good in 22 children (85%) at 12 months (Class 1). At longer follow-up (mean 4.3, range 1.0 to 11.0 years) only 16 (62%) remained seizure-free. Residual DNT was evident in 15 of the 24 children with available postoperative MRI. Three children demonstrated recurrence of tumor. At 12 months follow-up, older age (>10 years) and longer duration of epilepsy (>2 years) were associated with seizure recurrence. The presence of residual tumor was a risk factor for seizure recurrence at long-term follow-up (p = 0.02). Conclusions: Children with DNT and epilepsy may benefit from surgical management; however, seizure outcome is not always favorable. Although the majority of children remain seizure free after surgical excision of DNT, a considerable number have recurrent seizures. Short-term outcome is influenced by older age at surgery and longer duration of epilepsy. Residual tumor is a significant risk factor for poor seizure outcome. Recurrent tumor can occur.