Verma Vivek, Johnson Catrina P, Bennion Nathan R, Bhirud Abhijeet R, Li Sicong, McComb Rodney D, Lin Chi
Department of Radiation Oncology, University of Nebraska Medical Center, 987521 Nebraska Medical Center, Omaha, NE, USA,
Childs Nerv Syst. 2015 Aug;31(8):1393-9. doi: 10.1007/s00381-015-2723-5. Epub 2015 May 5.
Atypical teratoid rhabdoid tumors (ATRTs) arise from the central nervous system largely in the pediatric population. They portend a very poor prognosis with few long-term survivors. We describe a series of five cases at our institution.
We conducted a retrospective chart review and clinical follow-up.
Three patients underwent chemoradiation after surgical resection; the two patients whose caretakers declined this therapy passed away soon after diagnosis. Chemoradiation included intravenous and intrathecal chemotherapy as well as intensity-modulated radiotherapy after resection. Of the patients receiving chemoradiation, two patients had infratentorial tumors, two had gross residual tumor after resection, and two were under the age of 3 years. The three patients receiving trimodality therapy remain clinically and symptomatically disease-free with follow-up times of 44, 46, and 55 months. Two of the patients have mild neuropsychiatric sequelae after therapy.
Long-term, high-volume trials of ATRT are currently not published. We offer experience in successful long-term survival of this tumor treated with chemoradiotherapy.
非典型畸胎样横纹肌样瘤（ATRT）主要发生于儿童中枢神经系统。其预后极差，长期存活者寥寥无几。我们报告了本机构的5例该类病例。
我们进行了回顾性病历审查和临床随访。
3例患者在手术切除后接受了放化疗；另外2例患者的监护人拒绝了该治疗，诊断后不久便去世。放化疗包括静脉和鞘内化疗以及切除术后的调强放疗。接受放化疗的患者中，2例为幕下肿瘤，2例术后有大体残留肿瘤，2例年龄小于3岁。接受三联疗法的3例患者在随访44、46和55个月时临床及症状上均无疾病复发。其中2例患者治疗后有轻度神经精神后遗症。
目前尚无关于ATRT的长期、大样本试验发表。我们提供了经放化疗成功实现该肿瘤长期存活的经验。
