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[伯基特型恶性淋巴瘤。21例儿童病例研究]

[Malignant lymphoma of the Burkitt type. Study of 21 cases in childhood].

作者信息

Kodet R, Vanková R, Weinreb M, Zocková P

机构信息

Ustav patologické anatomie 2. lékarské fakulty University Karlovy, Praha.

出版信息

Cesk Pediatr. 1990 May;45(5):261-4.

PMID:2249289
Abstract

From a group of 151 malignant lymphomas in childhood the authors selected 21 cases of malignant Burkitt lymphoma. The histopathological diagnosis was checked using common paraffin material, and a basic clinicopathological correlations was made. Malignant lymphoma of the Burkitt type was characterized by a diffuse type of growth with striking cellular cohesion. From the cytological aspect it was uniform with minimal differences in cell size. The presence of macrophages forming a "starry-sky" phenomenon was constant in the bioptic material. It is, however, not specific for Burkitt's lymphoma. In necroptic material macrophages were often absent and the morphology of tumour cells was altered. In the differential diagnosis it is necessary to differentiate in particular malignant lymphoblastic lymphomas and monomorphous centroblastomas. The malignant of Burkitt lymphoma type was localized in the majority of patients in the abdominal cavity, less frequently on the neck and in Waldeyer's ring. The mediastinum was not primarily affected. Five patients survive for 16-52 months after termination of oncological therapy without a relapse. Twelve patients died from generalization of the process, or from complications. Eight of those who died had a primary tumour in the abdominal cavity. The mean interval between the bioptic diagnosis and death was 3.6 months. As to the primary localization of the tumour and some other clinical and pathological data, various authors differ--these differences are discussed in relation to possible variations in the distribution of the tumour under different geographical conditions.

摘要

作者从151例儿童恶性淋巴瘤中选出21例伯基特恶性淋巴瘤。采用普通石蜡材料检查组织病理学诊断,并进行基本的临床病理相关性分析。伯基特型恶性淋巴瘤的特征是弥漫性生长,细胞黏附性显著。从细胞学角度看,细胞形态一致,细胞大小差异极小。活检材料中始终存在形成“星空”现象的巨噬细胞。然而,这并非伯基特淋巴瘤所特有。在坏死材料中,巨噬细胞常缺失,肿瘤细胞形态也会改变。在鉴别诊断中,尤其需要区分恶性淋巴细胞性淋巴瘤和单形性中心母细胞性淋巴瘤。大多数伯基特淋巴瘤型恶性肿瘤患者的肿瘤位于腹腔,较少见于颈部和瓦尔代尔环。纵隔并非主要受累部位。5例患者在肿瘤治疗结束后存活16至52个月,无复发。12例患者死于病情播散或并发症。其中8例死亡患者的原发肿瘤位于腹腔。活检诊断至死亡的平均间隔时间为3.6个月。关于肿瘤的原发部位以及其他一些临床和病理数据,不同作者的观点存在差异——针对不同地理条件下肿瘤分布可能存在的差异,本文对这些差异进行了讨论。

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