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一名60岁男性的原始神经外胚层肿瘤:病例报告及文献综述

Primitive neuroectodermal tumour in a 60-year-old man: a case report and literature review.

作者信息

Balafouta M J, Kouvaris J R, Miliadou A C, Papacharalampous X N, Kolokouris D V, Kouloulias V E, Mariolis A D, Vlahos L J

机构信息

Department of Radiology-Radiotherapy, Aretaieion Hospital, University of Athens, Greece.

出版信息

Br J Radiol. 2003 Jan;76(901):62-5. doi: 10.1259/bjr/47707206.

Abstract

Primitive neuroectodermal tumour (PNET) is very rare, especially in adults. We report a 60-year-old man presented with a PNET. The symptoms at the time of diagnosis were intense headache, Broca's aphasia and right hemiparesis. Only an open biopsy was performed. Irradiation of the primary tumour was the main treatment (total tumour dose 59.8 Gy) because of serious haematological side effects due to chemotherapy. The patient tolerated radiation therapy extremely well and his neurological symptoms were improved. 1 month after completion of radiotherapy, MRI showed no regression of the tumour. Clinical deterioration was observed 10 months after the initial diagnosis and the patient died 2 months later. In cases of PNET, initial therapy is surgical bulk reduction whenever possible. Irradiation of the cerebrospinal axis is justified as a routine treatment but, owing to the radioresistance of the tumour, the addition of multiregimen chemotherapy appears to improve survival, according to the literature.

摘要

原始神经外胚层肿瘤(PNET)非常罕见,尤其是在成年人中。我们报告了一名60岁患有PNET的男性。诊断时的症状为剧烈头痛、布罗卡失语症和右侧偏瘫。仅进行了开放性活检。由于化疗导致严重的血液学副作用,对原发肿瘤进行放射治疗是主要治疗方法(肿瘤总剂量59.8 Gy)。患者对放射治疗耐受性极佳,其神经症状得到改善。放疗结束1个月后,MRI显示肿瘤无退缩。初始诊断10个月后观察到临床病情恶化,患者2个月后死亡。对于PNET病例,初始治疗应尽可能进行手术减瘤。对脑脊髓轴进行放射治疗作为常规治疗是合理的,但根据文献,由于肿瘤的放射抗性,添加多方案化疗似乎可提高生存率。

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