肝神经鞘瘤的临床病理学特征分析

Clinicopathological features of hepatic schwannoma

目的分析肝神经鞘瘤的临床病理学特征并进行相关文献检索、复习。方法收集解放军总医院第一医学中心病理科2005-05—2020-01明确诊断为肝神经鞘瘤3例,以及检索1982-02—2020-02国内外文献报道的肝神经鞘瘤13例,观察总结其临床病理学特征。结果患者年龄24~72岁,其中女性11例,男性5例。10例位于肝左叶,5例位于肝右叶,1例位于肝尾状叶。肿瘤最大径2.3~20cm不等,呈结节状,切面呈灰白或灰黄色,质中,部分伴有囊性变,与周围肝组织分界尚清,包膜完整。镜下见肿瘤由细胞密集区(AntoniA区)和细胞稀疏区(Antoni B区)构成,AntoniA区主要由梭形细胞构成,肿瘤细胞较丰富,胞质嗜酸性,胞核呈柳叶形,呈栅栏状编织状或漩涡状排列,其内可见厚壁血管和陈旧性出血,局部可见泡沫细胞聚集;AntoniB区,肿瘤细胞稀疏,间质玻璃样变性伴黏液变性,其间散在泡沫细胞、淋巴细胞,并可见扩张的薄壁血管;其中1例为极其罕见的假腺样型神经鞘瘤,肿瘤内可见大小不等的囊状、腺样及腔隙样结构,腔内衬单层肿瘤细胞。免疫组化:肿瘤细胞波形蛋白和酸性钙结合蛋白(S-100)阳性,血管内皮细胞标记CD34、广谱细胞角蛋白(CK)、平滑肌肌动蛋白(SMA)、神经纤维细丝蛋白(NF)、结蛋白、酪氨酸激酶生长因子受体蛋白(CD117)及钙离子调节相关的氯离子通道蛋白(DOG-1)均阴性,增殖细胞核抗原(Ki-67)阳性指数极低。预后方面,15例经手术切除,术后随访4~120个月,除5例失访外,其余均存活,未出现本病的复发及转移。结论肝神经鞘瘤是一种极罕见的良性肿瘤,病理组织学形态及免疫组织化学染色可帮助诊断及鉴别诊断。一般手术完整切除后,预后良好。

Objective To analyze the clinicopathological features of hepatic schwannoma with review of the relevant literatures. Methods Three cases of hepatic schwannoma were definitely diagnosed in the Department of Pathology, the First Medical Center of PLA General Hospital from May 2005 to January 2020, and 13 cases of hepatic schwannoma were retrieved from the literature from February 1982 to February 2020, and their clinical, pathological characteristics and immunohistochemical staining results were observed and summarized. Results The age of the patients ranged from 24 to 72 years. There were 11 females and 5 males. Ten were located in the left lobe of the liver, five in the right lobe of the liver, and one in the caudate lobe of the liver. The maximum diameter of the tumors ranged from 2.3 to 20 cm, showing a nodular pattern, gray-white or gray-yellow in cut section, with some cystic changes in the texture, clear boundary with surrounding liver tissue, and intact capsule. Microscopically, the tumor was composed of cell-dense area(Antoni A area) and cell-sparse area(Antoni B area). Antoni A area was mainly composed of spindle cells, with abundant tumor cells and eosinophilic cytoplasm. The nuclei were wavy or willow-shaped, arranged in a fence, braided or whirlpool shape. Thick-walled blood vessels and old hemorrhage could be seen in it, and foam cell aggregation could be seen locally. In area B, the tumor cells were sparse, interstitial hyalinization with mucinous degeneration, scattered foam cells, chronic inflammatory cells, and dilated thin-walled blood vessels were seen. Among them, 1 case diagnosed as pseudo adenoid schwannoma was extremely rare. This tumor was cystic, with adenoid and lacunar arranged in different sizes and lined by epithelioid cells. However, the epithelioid cells were also tumor cells, and acidic calcium binding protein(S-100) were positive. Immunohistochemical staining showed that tumor cells were positive for vimentin and S-100, but vascular endothelial cell markers CD34, broad-spectrum cytokeratin(CK), smooth muscle actin(SMA), neurofilament protein(NF), desmin, CD117 and DOG-1 were negative;Ki-67 index was lower. In terms of prognosis, 15 patients underwent surgical resection and were followed up for 4-120 months. Except for 5 patients who were lost to follow-up, all survived without recurrence or metastasis of the disease. Conclusions Hepatic schwannoma is a rare benign tumor, and histopathological morphology and immunohistochemical staining can help in the diagnosis and differential diagnosis. Generally, the prognosis is good after complete resection.