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胸膜弥漫性恶性间皮瘤:6例活检后症状缓解期延长或生存期延长患者的临床病理研究及长期生存文献综述

Diffuse malignant mesothelioma of the pleura: a clinicopathological study of six patients with a prolonged symptom-free interval or extended survival after biopsy and a review of the literature of long-term survival.

作者信息

Mark E J, Shin D H

机构信息

Department of Pathology, Massachusetts General Hospital, Boston 02114.

出版信息

Virchows Arch A Pathol Anat Histopathol. 1993;422(6):445-51. doi: 10.1007/BF01606452.

Abstract

Diffuse malignant mesothelioma of the pleura generally proceeds rapidly with clinical deterioration soon after the initial histopathological diagnosis of the tumor. We encountered six patients in whom the symptom-free interval after biopsy was surprisingly prolonged. Histopathologically, the tumor in each case was epithelioid with prominent myxoid stroma and formation of tubules and micropapillae. Cytologically, the malignant cells had relatively regular nuclei with bland chromatin. As individual findings, these histological and cytological features are common in diffuse malignant mesothelioma. However, the combination of (1) bland cytology of epithelioid cells, (2) formation of tubular and micropapillary patterns, (3) a myxoid stroma and (4) an absence of sclerosing desmoplasia, as observed in these six cases, might indicate indolent disease at the outset in a few patients. It is possible that these features are related to early stage of disease rather than having discriminatory value in their own right. We do not conclude that initially restrained disease necessarily presages a longer mean survival because the follow-up in these patients has not been long enough. We present these observations because, to date, this malignancy has proven so refractory to clinical management.

摘要

胸膜弥漫性恶性间皮瘤通常进展迅速,在肿瘤初次组织病理学诊断后不久临床症状就会恶化。我们遇到了6例患者,其活检后的无症状期出人意料地延长。组织病理学上,每例肿瘤均为上皮样,伴有显著的黏液样间质以及小管和微乳头形成。细胞学上,恶性细胞的核相对规则,染色质平淡。作为个体表现,这些组织学和细胞学特征在弥漫性恶性间皮瘤中很常见。然而,如这6例中所观察到的,(1)上皮样细胞的平淡细胞学表现、(2)小管和微乳头模式的形成、(3)黏液样间质以及(4)无硬化性促纤维增生,这些特征的组合可能表明少数患者在疾病初期进展缓慢。这些特征有可能与疾病的早期阶段有关,而并非其本身具有鉴别价值。我们并未得出疾病初期进展缓慢必然预示着更长平均生存期的结论,因为对这些患者的随访时间还不够长。我们展示这些观察结果是因为,迄今为止,这种恶性肿瘤已被证明对临床治疗如此难治。

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