Mallick Supriya, Gandhi Ajeet Kumar, Sharma Daya Nand, Gupta Subhash, Haresh Kunhi P, Rath Goura Kishor, Julka Pramod Kumar
Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, India.
Childs Nerv Syst. 2015 Dec;31(12):2341-4. doi: 10.1007/s00381-015-2919-8. Epub 2015 Oct 5.
Primary pediatric gliosarcoma (pPGS) is an extremely rare entity with only 25 cases reported in the English literature. The value of concurrent and adjuvant temozolomide is not known in this group of patient.
Five patients of pPGS treated from 2006 to 2011 were included in this retrospective analysis. All patients underwent maximal safe surgical resection. Adjuvant therapy included conformal radiation 60 Gy in 30 fractions (2 Gy daily for 5 days in a week) with concurrent temozolomide 75 mg/m(2) daily followed by six cycles of maintenance temozolomide 150-200 mg/m(2) (day 1 to day 5) every 4 weeks. We combined the survival data of 25 patients (already published) and five of our patients and analyzed them in terms of progression free survival (PFS) and overall survival (OS) using Kaplan-Meier method.
Male to female ratio was 1:4 and median age was 12 years (range, 7-19 years). All but one patient underwent gross total resection and four patients completed adjuvant radiotherapy as well as concurrent and adjuvant temozolomide. At a median follow up of 22.6 months (range, 0 to 45.3 months), two patients were dead and two were alive without disease while one was lost to follow up. For the pooled data, estimated median PFS and OS of all 30 patients reported in literature were 12 and 43 months, respectively. Two years PFS and OS rate for all patients was 44.2 and 62.9%, respectively.
Adjuvant radiotherapy and temozolomide is well tolerated and show an encouraging survival in pPGS.
原发性儿童胶质肉瘤（pPGS）是一种极其罕见的疾病，英文文献中仅报道了25例。在这类患者中，同步放化疗及辅助使用替莫唑胺的价值尚不清楚。
本回顾性分析纳入了2006年至2011年期间治疗的5例pPGS患者。所有患者均接受了最大安全限度的手术切除。辅助治疗包括适形放疗，剂量为60 Gy，分30次（每周5天，每天2 Gy），同时每日口服替莫唑胺75 mg/m²，随后每4周进行6个周期的维持治疗，剂量为150 - 200 mg/m²（第1天至第5天）。我们将25例已发表患者及5例本研究患者的生存数据合并，并采用Kaplan-Meier法分析无进展生存期（PFS）和总生存期（OS）。
男女比例为1:4，中位年龄为12岁（范围7 - 19岁）。除1例患者外，所有患者均接受了全切除，4例患者完成了辅助放疗以及同步和辅助替莫唑胺治疗。中位随访22.6个月（范围0至45.3个月），2例患者死亡，2例患者无疾病存活，1例失访。对于汇总数据，文献报道的所有30例患者的估计中位PFS和OS分别为12个月和43个月。所有患者的2年PFS率和OS率分别为44.2%和62.9%。
辅助放疗和替莫唑胺耐受性良好，在pPGS中显示出令人鼓舞的生存率。
