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胃肠道神经鞘瘤:9例病例系列及文献综述

Gastrointestinal schwannomas: A case series of 9 patients and literature review.

作者信息

Uludağ Server Sezgin, Erginöz Ergin, Güreş Nazım, Özdemir Zeynep, Kepil Nuray, Batur Şebnem

机构信息

Department of General Surgery, İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul, Türkiye.

Department of General Surgery, Balıkesir Atatürk City Hospital, Balıkesir, Türkiye.

出版信息

Turk J Surg. 2025 Sep 3;41(3):327-332. doi: 10.47717/turkjsurg.2025.2025-5-28.

DOI:10.47717/turkjsurg.2025.2025-5-28
PMID:40899484
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12406649/
Abstract

Gastrointestinal schwannomas are benign, slow-growing, rare tumors comprising 2-6% of all mesenchymal tumors of the gastrointestinal tract and 0.2% of all gastric neoplasms. In the gastrointestinal system, schwannomas are mostly observed in the stomach, followed by the colon and rectum. In this case series, we present the clinicopathological results of 9 cases, along with a literature review. A retrospective analysis was conducted on nine patients diagnosed with gastrointestinal schwannoma in a single institution. Tumors were located in the small intestine and stomach, with an average tumor size of 4.6 cm (range: 1.8-8.5 cm). Diagnoses were incidental in most cases, with only four patients presenting symptoms such as epigastric pain and changes in bowel habits. Histopathological characteristics of tumors were studied. Surgical resection with negative margins was performed in 8 cases. Histopathological analysis confirmed schwannomas characterized by solid, homogeneous, spindle-cell structures without cystic changes or necrosis. Immunohistochemically, all tumors were S-100 positive, with variable expression of other markers. Desmin was negative in seven samples. One gastric schwannoma showed focal smooth muscle actin positivity, while others were negative. The Ki-67 index ranged from 0% to 6%, and c-Kit was negative in all cases. DOG-1 expression was examined in four cases, showing focal positivity in small bowel schwannoma and negativity in three gastric schwannomas. Gastrointestinal schwannomas are predominantly benign tumors, more common in women, and typically occur in the sixth decade of life. While imaging and endoscopic techniques help in diagnosis, definitive diagnosis relies on histopathological analysis. Surgical resection remains the gold standard for treatment.

摘要

胃肠道神经鞘瘤是良性、生长缓慢的罕见肿瘤,占胃肠道所有间叶组织肿瘤的2% - 6%,占所有胃肿瘤的0.2%。在胃肠道系统中,神经鞘瘤大多见于胃,其次是结肠和直肠。在本病例系列中,我们呈现9例的临床病理结果,并进行文献复习。对在单一机构诊断为胃肠道神经鞘瘤的9例患者进行回顾性分析。肿瘤位于小肠和胃,平均肿瘤大小为4.6厘米(范围:1.8 - 8.5厘米)。大多数病例诊断为偶然发现,只有4例患者出现上腹部疼痛和排便习惯改变等症状。对肿瘤的组织病理学特征进行了研究。8例进行了切缘阴性的手术切除。组织病理学分析证实神经鞘瘤具有实性、均匀、梭形细胞结构,无囊性变或坏死。免疫组化方面,所有肿瘤S - 100均为阳性,其他标志物表达各异。7个样本中结蛋白为阴性。1例胃神经鞘瘤显示局灶性平滑肌肌动蛋白阳性,其他为阴性。Ki - 67指数范围为0%至6%,所有病例中c - Kit均为阴性。对4例检测了DOG - 1表达,显示小肠神经鞘瘤有局灶性阳性,3例胃神经鞘瘤为阴性。胃肠道神经鞘瘤主要是良性肿瘤,女性更常见,通常发生在60岁左右。虽然影像学和内镜技术有助于诊断,但确诊依赖于组织病理学分析。手术切除仍然是治疗的金标准。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e0b9/12406649/57d4132753da/TurkJSurg-41-3-327-figure-4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e0b9/12406649/575b5f027981/TurkJSurg-41-3-327-figure-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e0b9/12406649/677f856d855d/TurkJSurg-41-3-327-figure-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e0b9/12406649/f9ffcd627467/TurkJSurg-41-3-327-figure-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e0b9/12406649/57d4132753da/TurkJSurg-41-3-327-figure-4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e0b9/12406649/575b5f027981/TurkJSurg-41-3-327-figure-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e0b9/12406649/677f856d855d/TurkJSurg-41-3-327-figure-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e0b9/12406649/f9ffcd627467/TurkJSurg-41-3-327-figure-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e0b9/12406649/57d4132753da/TurkJSurg-41-3-327-figure-4.jpg

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本文引用的文献

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Differential diagnosis of submucosal gastric tumors: gastric schwannomas misdiagnosed as GISTs.胃黏膜下肿瘤的鉴别诊断:误诊为胃肠道间质瘤的胃神经鞘瘤。
J Surg Case Rep. 2024 Dec 18;2024(12):rjae793. doi: 10.1093/jscr/rjae793. eCollection 2024 Dec.
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A rare case of gastric schwannoma mimicking GIST: diagnostic and therapeutic approach.
一例罕见的酷似胃肠道间质瘤的胃神经鞘瘤:诊断与治疗方法
J Surg Case Rep. 2024 Aug 23;2024(8):rjae525. doi: 10.1093/jscr/rjae525. eCollection 2024 Aug.
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Malignant melanotic schwannoma of the cervical spinal cord: a case report.颈脊髓恶性黑色素性雪旺细胞瘤:1 例报告。
BMC Neurol. 2024 May 30;24(1):181. doi: 10.1186/s12883-024-03686-0.
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Incidental Finding of an Asymptomatic Jejunal Schwannoma: A Rare Case Report and Review of Literature.无症状空肠神经鞘瘤的偶然发现:一例罕见病例报告及文献复习。
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