背景：恶性杆状肿瘤（MRT）和他们的中枢神经系统(CNS)类似物非典型性畸胎样的/杆状肿瘤(ATRT)是很少见的小儿恶性肿瘤.尽管有报道说通过加强治疗能够长期生存,但是效果不好.方法：我们在Great Ormond Street医院对1989到2009年20年中的所有MRT/ATRT 诊断病人作了回顾性调查.所有的病例都经过了包括了INI-1免疫染色的病理学调查.结果：在最终的34例病例中总存活率为17.4%,平均存活10.1月.在小于3岁的病人中更加严重（平均存活率分别为6.6月和14.9月）,尽管根据病人年龄来分析有点混乱.有4个长期存活（大于30个月）的病例接受了化疗,有的接受了放疗和外科切除手术.在本研究中,c-Erb 或c-Met免疫组化分析并没有发现明显的染色,这表明将它们作为靶位点对治疗MRT/ATRT没有意义.结论：通过该调查,我们需要寻找新的分子靶位点和治疗方案来治疗小儿MRT/ATRT血癌.

1: Pediatr Blood Cancer. 2009 Aug 3. [Epub ahead of print]
Clinical and pathological features of paediatric malignant rhabdoid tumours.
Morgenstern DA, Gibson S, Brown T, Sebire NJ, Anderson J.
Unit of Molecular Haematology and Cancer Biology, UCL Institute of Child Health, London, UK.
  BACKGROUND: Malignant rhabdoid tumours (MRT) and their central nervous system (CNS) counterparts atypical teratoid/rhabdoid tumours (ATRT) are rare, highly aggressive malignant neoplasms of childhood. Although there are isolated reports of long-term survival with intensive, multimodal therapy, outcomes are generally poor. PROCEDURE: We conducted a retrospective review of all patients diagnosed with MRT/ATRT at Great Ormond Street Hospital over the 20 years from 1989 to 2009. All cases were subjected to expert pathological review including INI-1 immunostaining. RESULTS: In a final cohort of 34 cases, overall survival was 17.4%, with median survival 10.1 months. Outcome in patients aged <3 years was significantly worse (median survival 6.2 months vs. 19.2 months). Data demonstrated a statistically significant benefit of radiotherapy (median survival 14.9 months vs. 6.6 months), although this analysis is confounded by the impact of patient age. There were four long-term survivors (>30 months), all of whom received chemotherapy with or without surgical resection or radiotherapy. In the present study, immunohistochemistry revealed no significant staining for either c-Erb or c-Met in any case, suggesting that targeting these molecules is unlikely to be of benefit in treating MRT/ATRT. CONCLUSIONS: In view of poor outcomes, there is a clear need for new treatment strategies and the identification of novel molecular targets for MRT/ATRT. Pediatr Blood Cancer (c) 2009 Wiley-Liss, Inc.

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