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颅内细胞性 schwann 瘤:20 例临床病理研究。

Intracranial cellular schwannomas: a clinicopathological study of 20 cases.

机构信息

Department of Anatomic Pathology, A. C. Camargo Cancer Center, Sao Paulo, Brazil.

Department of Pathology, Johns Hopkins University, Baltimore, MD, USA.

出版信息

Histopathology. 2020 Jan;76(2):275-282. doi: 10.1111/his.13967. Epub 2019 Nov 13.

Abstract

AIMS

Cellular schwannoma is a specific subtype of schwannoma, prone to misinterpretation as a malignant neoplasm. Involvement of the intracranial compartment by these tumours is extremely rare. We aim to characterise this clinicopathological subgroup.

METHODS AND RESULTS

We identified a total of 20 cellular schwannomas with predominant intracranial involvement. The mean age of the patients at the time of surgery was 37 years (range = 16-81), with a slight female predominance (1.5:1 ratio). The most common sites were the eighth (n = 8) and fifth (n = 6) cranial nerves. Three tumours involved the anterior cranial fossa/olfactory groove, and a single case involved the glossopharyngeal nerve. All tumours met established criteria for cellular schwannoma, and were composed of interlacing fascicles of spindle cells lacking Verocay bodies with minimal Antoni B pattern and variable chronic inflammation and foamy histiocytes. Rare findings included haemosiderin deposition (n = 6), necrosis (n = 4), brisk mitotic activity (>10 mitoses per 10 high-power fields) (n = 2), focal epithelioid morphology (n = 2), myxoid areas (n = 2), neuroblastoma-like pattern (n = 1) and granular cells (n = 1). Immunohistochemical stains demonstrated expression of Schwann cell markers (S100 protein, SOX10, collagen IV) and preserved H3 K27 trimethylation in all cases tested. Fourteen patients had postoperative follow-up, ranging from 2 months to 21 years (mean = 66 months). In patients with follow-up, local recurrence/persistence developed in six cases; five tumours were initially incompletely resected. No metastatic disease or deaths were reported.

CONCLUSIONS

Intracranial cellular schwannomas share morphological and immunophenotypical features with cellular schwannomas at others sites may demonstrate locally aggressive growth but appear to lack metastatic potential.

摘要

目的

细胞性神经鞘瘤是神经鞘瘤的一种特殊亚型,容易被误诊为恶性肿瘤。这些肿瘤累及颅内极为罕见。我们旨在描述这一临床病理亚组。

方法和结果

我们共鉴定出 20 例以颅内广泛受累为主的细胞性 schwannoma。手术时患者的平均年龄为 37 岁(范围= 16-81 岁),女性略多(男女比例为 1.5:1)。最常见的部位是第八颅神经(n=8)和第五颅神经(n=6)。3 例肿瘤累及前颅窝/嗅沟,1 例累及舌咽神经。所有肿瘤均符合细胞性 schwannoma 的既定标准,由纺锤形细胞交织束组成,缺乏 Verocay 小体,具有最小的 Antoni B 型和可变的慢性炎症和泡沫状组织细胞。罕见的发现包括含铁血黄素沉积(n=6)、坏死(n=4)、活跃的有丝分裂活动(>10 个有丝分裂/10 个高倍视野)(n=2)、局灶上皮样形态(n=2)、黏液样区域(n=2)、神经母细胞瘤样模式(n=1)和颗粒细胞(n=1)。免疫组化染色显示所有检测病例均表达 Schwann 细胞标志物(S100 蛋白、SOX10、胶原 IV),且保留 H3 K27 三甲基化。14 例患者有术后随访,随访时间从 2 个月到 21 年(平均随访时间为 66 个月)。在有随访的患者中,6 例局部复发/残留;5 例肿瘤最初未完全切除。无转移疾病或死亡报告。

结论

颅内细胞性 schwannoma 具有与其他部位细胞性 schwannoma 相同的形态学和免疫表型特征,可能表现出局部侵袭性生长,但似乎缺乏转移潜能。

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