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成人髓母细胞瘤的预后因素。一项临床病理研究。

Prognostic factors in adult medulloblastoma. A clinico-pathologic study.

作者信息

Giordana M T, Cavalla P, Chiò A, Marino S, Soffietti R, Vigliani M C, Schiffer D

机构信息

Clinica Neurologica II, Università di Torino, Italy.

出版信息

Tumori. 1995 Sep-Oct;81(5):338-46. doi: 10.1177/030089169508100507.

Abstract

AIMS AND BACKGROUND

Medulloblastoma in adults is a rare tumor. The small number of cases in the reported series has not permitted a definite assessment of the prognostic role of clinical, pathologic and cell kinetics factors. The largest series of medulloblastoma in adults treated in a single institution is herein reported.

METHODS

The clinical, therapeutic, pathologic and proliferation features of medulloblastoma in 44 adult patients (> 18 years) were analyzed retrospectively with regard to postoperative survival. The proliferation potential of each tumor was evaluated by the immunohistochemical demonstration of proliferating cell nuclear antigen (PCNA) and Ki-67, clone MIB-1, in paraffin sections.

RESULTS

The overall 5- and 10-year survival rates were 40% and 35.6%, respectively. Significant factors in predicting a longer postoperative survival were: age < 37 years, decade of management (1977-1990), radiotherapy (50-55 Gy on the posterior fossa and 30-35 Gy on the spinal cord) and nuclear isomorphism. When corrected for adequacy of radiotreatment, desmoplastic type and differentiation were significantly correlated with a shorter survival. The PCNA-labelling index (LI) ranged from 34.5 to 82.2%, the MIB-1-LI ranged from 9.6 to 64.7%. No association was found between PCNA- or MIB-1-LI values and microscopic features, or between LI values and prognosis.

CONCLUSIONS

Contrary to a general assumption, desmoplastic medulloblastoma and differentiated medulloblastoma are negative prognostic factors in adequately radiotreated adult patients. This may possibly be referred to lower radiosensitivity of these tumor variants. The LI with PCNA or Ki-67 is of no help in identifying aggressive tumors.

摘要

目的与背景

成人髓母细胞瘤是一种罕见肿瘤。已报道系列中的病例数量较少,无法对临床、病理和细胞动力学因素的预后作用进行明确评估。本文报道了在单一机构治疗的最大系列成人髓母细胞瘤病例。

方法

回顾性分析44例成年患者(>18岁)髓母细胞瘤的临床、治疗、病理和增殖特征,以评估术后生存率。通过免疫组织化学方法在石蜡切片中检测增殖细胞核抗原(PCNA)和Ki-67克隆MIB-1,评估每个肿瘤的增殖潜能。

结果

总体5年和10年生存率分别为40%和35.6%。预测术后生存期较长的显著因素包括:年龄<37岁、治疗年代(1977 - 1990年)、放疗(后颅窝50 - 55 Gy,脊髓30 - 35 Gy)和核同型性。校正放疗充分性后,促纤维增生型和分化程度与较短生存期显著相关。PCNA标记指数(LI)范围为34.5%至82.2%,MIB-1-LI范围为9.6%至64.7%。未发现PCNA或MIB-1-LI值与微观特征之间、LI值与预后之间存在关联。

结论

与一般假设相反,在接受充分放疗的成年患者中,促纤维增生性髓母细胞瘤和分化型髓母细胞瘤是不良预后因素。这可能是由于这些肿瘤变体的放射敏感性较低。PCNA或Ki-67的LI对识别侵袭性肿瘤无帮助。

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