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儿童弥漫性和局灶性脑干肿瘤:预后因素及手术结果。单机构经验。

Diffuse and focal brain stem tumors in childhood: prognostic factors and surgical outcome. Experience in a single institution.

作者信息

Sandri A, Sardi N, Genitori L, Giordano F, Peretta P, Basso M E, Bertin D, Mastrodicasa L, Todisco L, Mussa F, Forni M, Ricardi U, Cordero di Montezemolo L, Madon E

机构信息

Pediatric Oncology Department, University of Turin, Torino, Piazza Polonia 94, 10124 Turin, Italy.

出版信息

Childs Nerv Syst. 2006 Sep;22(9):1127-35. doi: 10.1007/s00381-006-0083-x. Epub 2006 Mar 28.

DOI:10.1007/s00381-006-0083-x
PMID:16568342
Abstract

OBJECTIVE

Brainstem tumors (BSTs) are usually gliomas and are divided into diffuse BSTs (DBSTs) and focal BSTs (FBSTs). The aim of this study is to investigate the different outcomes of these two entities.

METHODS

Thirty-one patients with BSTs were admitted to our institution from 1995 to 2003. Patients with DBSTs were treated with locoregional radiotherapy (1.8 Gy/day for 54 Gy) and weekly vincristine for radiosensitization (1.5 mg/sm for six total doses). Patients with FBSTs underwent surgical resection. Chemotherapy and/or radiotherapy were considered in progression.

RESULTS AND CONCLUSIONS

Fourteen patients were diagnosed as having DBSTs. The responses to treatment were ten cases of partial response, three of stable disease, and one of progressive disease. General and/or neurological symptoms improved in more than 80% of patients. The median time from diagnosis to progression and to death were, nonetheless, 8 (range of 3-13) and 13 (range of 4-25) months, respectively, with a 2-year overall survival rate of 12.3% [standard error (SE) 11.2]. Seventeen patients were diagnosed as having FBSTs. Gross total removal was achieved in 4/17 cases, subtotal removal in 7/17, and partial removal in 6/17. There was one surgery-related death. Eight out of 17 patients had adjuvant chemo- and/or radiotherapy after progression: 6/8 are without neurological symptoms and 2/8 have died due to tumor progression. The 4-year overall and disease-free survival rates are 87.4 (SE 8.4) and 58.8% (SE 11.9), respectively, the extent of resection being the most important prognostic factor (p=0.012). DBSTs continue to carry a dismal prognosis, thus demanding new treatment modalities; FBSTs can be treated surgically and patients benefit from a better prognosis.

摘要

目的

脑干肿瘤(BSTs)通常为胶质瘤,分为弥漫性脑干肿瘤(DBSTs)和局灶性脑干肿瘤(FBSTs)。本研究旨在探讨这两种类型肿瘤的不同预后情况。

方法

1995年至2003年期间,31例脑干肿瘤患者入住我院。弥漫性脑干肿瘤患者接受局部区域放疗(1.8 Gy/天,共54 Gy),并每周使用长春新碱进行放射增敏(总量1.5 mg/sm,共6次剂量)。局灶性脑干肿瘤患者接受手术切除。疾病进展时考虑化疗和/或放疗。

结果与结论

14例患者被诊断为弥漫性脑干肿瘤。治疗反应为部分缓解10例、病情稳定3例、病情进展1例。超过80%的患者全身和/或神经症状有所改善。然而,从诊断到病情进展和死亡的中位时间分别为8个月(范围3 - 13个月)和13个月(范围4 - 25个月),2年总生存率为12.3%[标准误(SE)11.2]。17例患者被诊断为局灶性脑干肿瘤。17例中有4例实现了全切,7例次全切,6例部分切除。有1例手术相关死亡。17例患者中有8例在病情进展后接受了辅助化疗和/或放疗:8例中有6例无神经症状,2例因肿瘤进展死亡。4年总生存率和无病生存率分别为87.4%(SE 8.4)和58.8%(SE 11.9),切除范围是最重要的预后因素(p = 0.012)。弥漫性脑干肿瘤的预后仍然很差,因此需要新的治疗方式;局灶性脑干肿瘤可以通过手术治疗,患者预后较好。

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Brainstem gliomas.脑干胶质瘤
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