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成人髓母细胞瘤:手术结果与生存情况——16例患者的单中心分析

Medulloblastoma in Adults: Surgical Outcomes and Survival - A Single Center Analysis of 16 Patients.

作者信息

Yilmaz Hakan, Akcay Emrah, Benek Huseyin Berk

机构信息

Health Sciences University, Izmir Bozyaka Education and Research Hospital, Department of Neurosurgery, Izmir, Turkey.

出版信息

Turk Neurosurg. 2021;31(6):980-985. doi: 10.5137/1019-5149.JTN.32143-20.2.

DOI:10.5137/1019-5149.JTN.32143-20.2
PMID:34542903
Abstract

AIM

To look into the clinicopathological characteristics, surgical outcomes, and survival rates of adult patients with medulloblastoma.

MATERIAL AND METHODS

Adult patients (age > 17 years) who had surgery in our clinic with the diagnosis of cerebellar mass between 2009 and 2015 and whose pathological diagnosis was medulloblastoma were examined. The study was carried out retrospectively by analyzing the clinicopathological data, surgical outcomes, and complications of the patients. In the postoperative and follow-up periods, contrast-enhanced cranial magnetic resonance imaging (MRI) was used to assess the presence of recurrence or residual disease. Moreover, the overall 5-year survival rates of the patients were evaluated.

RESULTS

A total of 16 patients were diagnosed with medulloblastoma, with a mean age of 32.25 years old (age range 18?57 years). The tumor was found in the vermis in eight (50%) patients and the cerebellar hemispheres in the rest (50%) of them. Total excision was performed on 14 (87.5%) patients, near-total excision on 1 (6.25%) patient, and subtotal excision on 1 (6.25%) patient. The histopathological results were consistent with desmoplastic type medulloblastoma in nine (56.25%) patients, classical type in six (37.5%) patients, and anaplastic medulloblastoma in one (6.25%) patient. All patients received posterior fossa boost dose + craniospinal radiotherapy after the surgery. Recurrent lesions were found in six (40%) of the patients. A total of ten (62.5%) patients were still alive, and mortality rate was found to be 25% (4 patients) at 5 years.

CONCLUSION

After 5 years, 10 of the 16 patients in our study were still alive. Lateral localization of the tumor, desmoplastic histologic variant, and total excision were all good prognostic indicators. Total excision is difficult in patients with brainstem invasion, and even if total excision is performed, the prognosis is poor.

摘要

目的

探讨成年髓母细胞瘤患者的临床病理特征、手术结果及生存率。

材料与方法

对2009年至2015年间在我院接受手术、诊断为小脑肿块且病理诊断为髓母细胞瘤的成年患者(年龄>17岁)进行检查。通过分析患者的临床病理数据、手术结果及并发症,进行回顾性研究。在术后及随访期间,采用增强头颅磁共振成像(MRI)评估复发或残留疾病的存在情况。此外,评估患者的总体5年生存率。

结果

共16例患者被诊断为髓母细胞瘤,平均年龄32.25岁(年龄范围18 - 57岁)。8例(50%)患者肿瘤位于蚓部,其余8例(50%)位于小脑半球。14例(87.5%)患者进行了全切除,1例(6.25%)患者进行了近全切除,1例(6.25%)患者进行了次全切除。组织病理学结果显示,9例(56.25%)患者为促纤维增生型髓母细胞瘤,6例(37.5%)患者为经典型,1例(6.25%)患者为间变型髓母细胞瘤。所有患者术后均接受后颅窝增强剂量 + 全脑脊髓放疗。6例(40%)患者发现复发病变。共有10例(62.5%)患者仍存活,5年死亡率为25%(4例患者)。

结论

在我们的研究中,16例患者中有10例在5年后仍存活。肿瘤的外侧定位、促纤维增生组织学变异型及全切除均为良好的预后指标。脑干侵犯患者难以进行全切除,即使进行了全切除,预后也较差。

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