Expanding the Spectrum of GLI1-rearranged Neoplasms of the Gastrointestinal Tract to Include Monophasic Keratin-positive Epithelial Neoplasms.

IF 4.5 1区 医学 Q1 PATHOLOGY
American Journal of Surgical Pathology Pub Date : 2024-11-01 Epub Date: 2024-09-18 DOI:10.1097/PAS.0000000000002303
Dorukhan Bahceci, Grace E Kim, Sanjay Kakar, Dana J Balitzer, Eric D Nguyen, Rageshree Ramachandran, Sarah E Umetsu, Nancy M Joseph
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引用次数: 0

Abstract

GLI1-altered tumors form a diverse group occurring in various anatomic locations. In the alimentary tract, the most established are gastroblastoma, a biphasic epithelial-mesenchymal neoplasm of the stomach, and plexiform fibromyxoma, a pure spindle cell neoplasm. The spectrum of GLI1-rearranged gastrointestinal tumors has recently expanded with reports of cases in other parts of the GI tract, some exhibiting gastroblastoma-like features and others being pure mesenchymal neoplasms. These tumors often display a nonspecific immunophenotype, with only CD56 and cyclin D1 expression being common. Biphasic GLI1-altered tumors show diffuse keratin positivity in the epithelial component only, and GLI1-altered mesenchymal tumors typically lack or show only focal keratin expression. This study details 2 GLI1-rearranged gastrointestinal tract tumors with diffuse keratin and CD56 expression, composed entirely of epithelial cells with a nested growth pattern and finely stippled monotonous nuclei, leading to an initial suspicion of neuroendocrine tumor in both cases, despite lack of synaptophysin and chromogranin expression. Diffuse strong nuclear cyclin D1 expression was seen in both cases, and conversely, strong cyclin D1 staining was only seen in 5.4% (4/74) of well-differentiated neuroendocrine tumors tested. These 2 GI tract neoplasms highlight a widened spectrum of GLI1-rearranged tumors, now including monophasic epithelial neoplasms with diffuse keratin expression.

将胃肠道 GLI1 重组肿瘤的范围扩大到单相角蛋白阳性上皮肿瘤
GLI1改变的肿瘤种类繁多,发生在不同的解剖部位。在消化道,最常见的是胃母细胞瘤(一种胃部上皮-间质双相肿瘤)和丛状纤维瘤(一种纯纺锤形细胞肿瘤)。最近,GLI1 重组胃肠道肿瘤的范围有所扩大,有报告称胃肠道其他部位也出现了病例,其中一些表现出胃母细胞瘤样特征,另一些则是纯间叶肿瘤。这些肿瘤通常表现为非特异性免疫表型,常见的只有 CD56 和细胞周期蛋白 D1 表达。双相 GLI1 改变的肿瘤仅在上皮成分中显示弥漫性角蛋白阳性,而 GLI1 改变的间质肿瘤通常缺乏或仅显示局灶性角蛋白表达。本研究详细描述了 2 例 GLI1 重组的胃肠道肿瘤,这些肿瘤具有弥漫性角蛋白和 CD56 表达,完全由上皮细胞组成,具有巢状生长模式和细条纹单核细胞,尽管缺乏突触素和嗜铬粒蛋白表达,但最初怀疑这两例肿瘤为神经内分泌肿瘤。两例病例均可见弥漫性强细胞周期蛋白 D1 核表达,相反,强细胞周期蛋白 D1 染色仅见于 5.4%(4/74)的分化良好的神经内分泌肿瘤。这两种消化道肿瘤凸显了 GLI1 重组肿瘤的范围扩大,现在包括具有弥漫角蛋白表达的单相上皮肿瘤。
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来源期刊
CiteScore
10.30
自引率
5.40%
发文量
295
审稿时长
1 months
期刊介绍: The American Journal of Surgical Pathology has achieved worldwide recognition for its outstanding coverage of the state of the art in human surgical pathology. In each monthly issue, experts present original articles, review articles, detailed case reports, and special features, enhanced by superb illustrations. Coverage encompasses technical methods, diagnostic aids, and frozen-section diagnosis, in addition to detailed pathologic studies of a wide range of disease entities. Official Journal of The Arthur Purdy Stout Society of Surgical Pathologists and The Gastrointestinal Pathology Society.
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