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喉部炎性肌纤维母细胞瘤:同一实体的不同临床表现和组织形态学特征。

Laryngeal inflammatory myofibroblastic tumors: Different clinical appearance and histomorphologic presentation of one entity.

机构信息

Institute of Pathology, University Würzburg, Würzburg, Germany.

出版信息

Head Neck. 2010 Nov;32(11):1573-8. doi: 10.1002/hed.21232.

DOI:10.1002/hed.21232
PMID:19787794
Abstract

BACKGROUND

Inflammatory myofibroblastic tumors (IMFTs) of the larynx are rare. We report the clinical presentation, histomorphology, and new molecular findings of 2 cases.

METHODS

Paraffin-embedded specimens were stained immunohistochemically (eg, vimentin, AE1/3, Alk-1, smooth muscle [sm-]actin, p53, Rb1, immunoglobulin G4 [IgG4]). Epstein-Barr virus-encoded RNA (EBER) in situ hybridization and HHV8-polymerase chain reaction (PCR) were done. Comparative genomic hybridization (CGH) was performed.

RESULTS

Case 1 was that of a 56-year-old man with an infiltrating plasma-cell-rich tumor (Alk-1-, sm-actin+). Plasma cells were strongly positive for IgG4. CGH was unsuspicious. Case 2 was that of a 34-year-old woman with an exophytic tumor (Alk-1+). CGH revealed losses on 13q14-22. The few plasma cells were negative for IgG4. The proliferation (Ki67) was low in both cases.

CONCLUSION

Different types of IMFTs may exist and could indicate different therapeutic strategies. Alk-1-positive cases with only scattered inflammatory cells could represent the neoplastic variant, whereas cases rich in plasma cells could be associated with IgG4 sclerosing diseases.

摘要

背景

喉部炎性肌纤维母细胞瘤(IMFT)较为罕见。我们报告了 2 例病例的临床表现、组织形态学和新的分子发现。

方法

石蜡包埋标本行免疫组织化学染色(例如,波形蛋白、AE1/3、ALK-1、平滑肌肌动蛋白、p53、Rb1、免疫球蛋白 G4)。进行 EBV 编码 RNA(EBER)原位杂交和 HHV8-聚合酶链反应(PCR)。进行比较基因组杂交(CGH)。

结果

病例 1 为 56 岁男性,浸润性富含浆细胞的肿瘤(ALK-1-,sm-肌动蛋白+)。浆细胞 IgG4 染色阳性。CGH 无异常。病例 2 为 34 岁女性,外生性肿瘤(ALK-1+)。CGH 显示 13q14-22 缺失。少数浆细胞 IgG4 染色阴性。两种情况下增殖(Ki67)均较低。

结论

可能存在不同类型的 IMFT,这可能提示不同的治疗策略。仅存在散在炎症细胞的ALK-1 阳性病例可能代表肿瘤性变体,而富含浆细胞的病例可能与 IgG4 硬化性疾病相关。

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