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[关于所谓施密克淋巴上皮瘤的一个特定病例描述以及与爱泼斯坦-巴尔病毒相关性的研究]

[Description of a particular case of the so-called Schmincke lymphoepithelioma and study of the correlation with Epstein-Barr virus].

作者信息

Torre V, Cavallari V, Bucolo S, Abbate G, Romano G, Fera G, Galletti B

机构信息

Istituto Policattedra di Otorinolaringoiatria, Università di Messina.

出版信息

Acta Otorhinolaryngol Ital. 2000 Oct;20(5):347-53.

Abstract

For poorly differentiated rhinopharyngeal carcinomas, the clinical presentation (association with the Epstein-Barr virus, paraneoplastic syndromes, onset of lymphoma) and the histopathological features can be polymorphous and they can confound or delay diagnosis and preparation of an adequate treatment plan (radio-chemotherapy). Often these neoplasms arise as clinically primitive laterocervical metastases, masked by clinical findings and a history that can lead to the mistaken diagnosis of systemic lymphoproliferative processes such as Hodgkin's disease. Here an observation of this type is presented in a young patient (19 years old) who came under observation for a laterocervical tumefaction recurrent from a previous exeresis performed at another hospital and symptoms of serotine febricula, dysphagia and serology positive for the Epstein-Barr virus (EBV). The patient underwent surgery and then radiotherapy and has been under close post-operative follow-up for two years. To date the patient's condition--both local and general--is good. The particular histology of the neoformation lies in the abundant infiltration of plasma cell and lymphocyte eosinophils, at times in blastic form. Moreover, elements with a large clear nucleus and evident nucleolus (Hodgkin-like) and scattered multinucleate Langhans-type giant cells can be seen. Immunohistologically the tumor cells markedly express for cytokeratin and the latent membrane protein (LMP1) of the Epstein-Barr virus (EBV) and show a high growth fraction. Under the electron microscope, the plurinucleate giant cells present large nuclei with morphology similar to that of tumor cells. The clear cytokeratin-positivity of the tumor elements and the histological and ultrastructural features mentioned led to the diagnosis of a massive metastasis from lymphoepithelial carcinoma, the Schmincke variant, plus EBV infection of the neoplastic cells. The authors conclude assuming that the particular granulomatous reaction is due to the host's reaction to the tumor cells, but also to the reaction to the viral antigens. In the former case we find an attempt to limit the carcinomatous process; in the latter it is a response caused by the EBV and is not, apparently, aimed at protecting against the neoplasm rather it facilitates the neoplastic process.

摘要

对于低分化鼻咽癌,其临床表现(与爱泼斯坦-巴尔病毒相关、副肿瘤综合征、淋巴瘤的发生)和组织病理学特征可能具有多态性,这可能会混淆或延误诊断以及制定适当的治疗方案(放化疗)。这些肿瘤通常以临床上原发性颈侧转移灶的形式出现,被临床症状和病史所掩盖,可能导致误诊为系统性淋巴增殖性疾病,如霍奇金病。本文介绍了一名年轻患者(19岁)的观察病例,该患者因先前在另一家医院进行手术切除后复发的颈侧肿胀以及低热、吞咽困难症状和爱泼斯坦-巴尔病毒(EBV)血清学阳性而接受观察。患者接受了手术,随后进行了放疗,并在术后进行了两年的密切随访。迄今为止,患者的局部和全身状况良好。新形成肿瘤的特殊组织学表现为浆细胞和淋巴细胞嗜酸性粒细胞大量浸润,有时呈母细胞样形态。此外,可见具有大而清晰核仁和明显核仁的细胞成分(霍奇金样)以及散在的多核朗汉斯型巨细胞。免疫组织化学显示肿瘤细胞显著表达细胞角蛋白和爱泼斯坦-巴尔病毒(EBV)的潜伏膜蛋白(LMP1),且增殖分数较高。在电子显微镜下,多核巨细胞呈现出与肿瘤细胞形态相似的大核。肿瘤成分明确的细胞角蛋白阳性以及上述组织学和超微结构特征导致诊断为淋巴上皮癌施密克变型的大量转移,外加肿瘤细胞的EBV感染。作者推断,这种特殊的肉芽肿反应既是宿主对肿瘤细胞的反应,也是对病毒抗原的反应。在前一种情况下,我们看到了限制癌性过程的尝试;在后一种情况下,这是由EBV引起的反应,显然不是旨在预防肿瘤,而是促进肿瘤过程。

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