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表现为牙龈肿物的口腔血管免疫母细胞性T细胞淋巴瘤:1例经聚合酶链反应检测显示克隆性T细胞受体γ基因重排的罕见病例的组织病理学及分子特征报告

Angioimmunoblastic T-cell lymphoma of the oral cavity presenting as gingival mass: report of the histopathologic and molecular characteristics of an unusual case featuring clonal T-cell receptor γ gene rearrangement by polymerase chain reaction.

作者信息

Argyris Prokopios P, Koutlas Ioannis G, Cooley Sarah, Yohe Sophia L, Bhakta Keta, Gopalakrishnan Rajaram

机构信息

Division of Oral and Maxillofacial Pathology, School of Dentistry, University of Minnesota, Minneapolis, MN, USA.

Department of Medicine, University of Minnesota Medical School, Minneapolis, MN, USA.

出版信息

Oral Surg Oral Med Oral Pathol Oral Radiol. 2014 Dec;118(6):e198-204. doi: 10.1016/j.oooo.2014.07.006. Epub 2014 Jul 25.

Abstract

Angioimmunoblastic T-cell lymphoma (AITL) is a rare neoplastic process constituting 15% to 20% of peripheral T-cell lymphomas. We report the clinicopathologic and molecular characteristics of an unusual intraoral manifestation of AITL. A 35-year-old white man with a history of AITL presented with a 2.5-cm, poorly circumscribed, erythematous, exophytic lesion occupying the free and attached buccal gingiva of the right maxillary lateral incisor and canine. Histopathologically, the tumor showed diffuse and intense polymorphic infiltration by small to medium-sized lymphocytes admixed with numerous eosinophils. The neoplastic cells showed strong and diffuse reactivity for CD2, CD3, CD4, CD10, and PD-1 (programmed cell death 1 [PDCD1]). Rare immunopositivity was seen with BCL6 (B-cell CLL/lymphoma 6) and CXCL13 (chemokine [C-X-C motif] ligand 13). Neoplastic cells were negative for CD7 and EBER ISH (Epstein-Barr virus-encoded small RNA in situ hybridization). CD21 did not show any increased follicular dendritic cell component. Polymerase chain reaction-based assay found monoclonal T-cell receptor γ (TRG) gene rearrangements. Diagnosis of recurrent/residual AITL was rendered. Chemotherapy was administered, with the intraoral tumor resolving completely 3 months later.

摘要

血管免疫母细胞性T细胞淋巴瘤(AITL)是一种罕见的肿瘤性病变,占外周T细胞淋巴瘤的15%至20%。我们报告了一例AITL罕见的口腔内表现的临床病理和分子特征。一名35岁有AITL病史的白人男性,右侧上颌侧切牙和尖牙的游离和附着颊侧牙龈处出现一个2.5厘米、边界不清、红斑样、外生性病变。组织病理学上,肿瘤表现为小至中等大小淋巴细胞的弥漫性和强烈多形性浸润,并混有大量嗜酸性粒细胞。肿瘤细胞对CD2、CD3、CD4、CD10和PD-1(程序性细胞死亡1 [PDCD1])呈强阳性和弥漫性反应。BCL6(B细胞慢性淋巴细胞白血病/淋巴瘤6)和CXCL13(趋化因子[C-X-C基序]配体13)可见罕见的免疫阳性。肿瘤细胞CD7和EBER原位杂交(爱泼斯坦-巴尔病毒编码的小RNA原位杂交)阴性。CD21未显示滤泡树突状细胞成分增加。基于聚合酶链反应的检测发现单克隆T细胞受体γ(TRG)基因重排。诊断为复发性/残留性AITL。给予化疗,3个月后口腔内肿瘤完全消退。

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