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神经垂体的鞍区和鞍上颗粒细胞瘤:1例罕见病例报告及文献复习

Sellar and suprasellar granular cell tumor of the neurohypophysis: A rare case report and review of the literature.

作者信息

Jiang Biying, Shi Xiuying, Fan Chuifeng

机构信息

Department of Pathology, First Affiliated Hospital and College of Basic Medical Sciences of China Medical University, Shenyang, China.

出版信息

Neuropathology. 2018 Jun;38(3):293-299. doi: 10.1111/neup.12448. Epub 2017 Dec 21.

Abstract

Granular cell tumors of the neurohypophysis are rare tumors with a WHO grade of I. Symptomatic tumors are even more rare. In this case, we present a 50-year-old patient with a sellar and suprasellar granular cell tumor of the neurohypophysis, who reported headaches, blurred vision and unsteady gait. CT imaging showed a sellar and suprasellar mass approximately 2.9 cm in diameter with clear boundaries. Histologically, the tumor lacked any obvious atypia and contained densely arranged polygonal tumor cells with abundant granular eosinophilic cytoplasm. Staining for Alpha-1 AntiChymotrypsin (AACT), TTF-1 and PAS was diffusely positive, and S-100 staining was focally positive in the tumor cells. CD34, CK, EMA, GFAP and HMB45 staining were negative. The Ki-67 index was < 1%. According to these findings, the tumor was diagnosed as a symptomatic granular cell tumor of the neurohypophysis. We suggest that identifying the location of the tumor with imaging is helpful for understanding the granular cell tumor of the neurohypophysis. Prompt diagnosis and treatment are critical for patients.

摘要

神经垂体颗粒细胞瘤是一种罕见的肿瘤,世界卫生组织分级为I级。有症状的肿瘤更为罕见。在本病例中,我们报告了一名50岁的患者,患有鞍区及鞍上神经垂体颗粒细胞瘤,该患者主诉头痛、视力模糊和步态不稳。CT成像显示鞍区及鞍上有一直径约2.9厘米的肿块,边界清晰。组织学上,肿瘤无明显异型性,由密集排列的多边形肿瘤细胞组成,细胞质丰富,呈嗜酸性颗粒状。α1抗糜蛋白酶(AACT)、甲状腺转录因子-1(TTF-1)和过碘酸雪夫(PAS)染色呈弥漫性阳性,S-100染色在肿瘤细胞中呈局灶性阳性。CD34、细胞角蛋白(CK)、上皮膜抗原(EMA)、胶质纤维酸性蛋白(GFAP)和HMB45染色均为阴性。Ki-67指数<1%。根据这些发现,该肿瘤被诊断为有症状的神经垂体颗粒细胞瘤。我们认为,通过影像学确定肿瘤位置有助于了解神经垂体颗粒细胞瘤。对患者来说,及时诊断和治疗至关重要。

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