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原发性肝神经鞘瘤:病例报告与文献综述

Primary Hepatic Schwannoma: Case Report and Literature Review.

作者信息

Thi Khuyen Nguyen, Quoc Thanh Truong, Van Trung Hoang, Cong Thao Trinh, Nhu Tung Tran

机构信息

Center for Pathology and Molecular Biology, K Hospital, Ha Noi, Vietnam.

Medlatec Pathology Center, Ha Noi, Vietnam.

出版信息

Clin Med Insights Case Rep. 2023 Dec 6;16:11795476231215907. doi: 10.1177/11795476231215907. eCollection 2023.

Abstract

Primary hepatic schwannoma is an extremely rare tumor with a good prognosis. Preoperative diagnosis is often challenging due to nonspecific clinical symptoms and its rarity. Here, we report a case of a 56-year-old male patient misdiagnosed with malignant liver tumor, later identified as primary hepatic schwannoma. Furthermore, clinical and histopathological features of 19 cases of primary hepatic schwannoma are also documented. The age of the patients ranged from 38 to 72 years, with a mean age of 56.4 years, and the disease was more common in females. Patients typically presented without clinical symptoms and were not associated with neurofibromatosis type 1. Histopathological features of the tumor were similar to soft tissue schwannoma, characterized by a thick capsule consisting of Antoni A and Antoni B areas. Immunohistochemically, the tumor showed strong positivity and diffusely stained with S-100, while being negative for CD34, CD117, and SMA. Complete resection of the tumor was achieved in all patients. The prognosis was favorable, with no signs of recurrence. Follow-up examinations revealed disease-free survival ranging from 6 to 27 months. Differential diagnosis of primary hepatic schwannoma from malignant liver tumors and metastatic liver tumors can be made based on histopathological features and immunohistochemical staining with S-100.

摘要

原发性肝神经鞘瘤是一种极为罕见的肿瘤,预后良好。由于临床症状不具特异性且该病罕见,术前诊断往往具有挑战性。在此,我们报告一例56岁男性患者,最初被误诊为恶性肝肿瘤,后来确诊为原发性肝神经鞘瘤。此外,还记录了19例原发性肝神经鞘瘤的临床和组织病理学特征。患者年龄在38岁至72岁之间,平均年龄为56.4岁,该疾病在女性中更为常见。患者通常无临床症状,且与1型神经纤维瘤病无关。肿瘤的组织病理学特征与软组织神经鞘瘤相似,其特点是有一层厚包膜,由Antoni A区和Antoni B区组成。免疫组织化学检查显示,肿瘤对S-100呈强阳性且弥漫性染色,而对CD34、CD117和SMA呈阴性。所有患者均实现了肿瘤的完整切除。预后良好,无复发迹象。随访检查显示无病生存期为6至27个月。原发性肝神经鞘瘤与恶性肝肿瘤和转移性肝肿瘤的鉴别诊断可基于组织病理学特征以及S-100免疫组织化学染色来进行。

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