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胃肠道神经鞘瘤的自然病史。

Natural history of gastrointestinal schwannomas.

作者信息

Singh Amandeep, Aggarwal Manik, Chadalavada Pravallika, Siddiqui Mohamed Tausif, Garg Rajat, Lai Keith, Chahal Prabhleen

机构信息

Department of Gastroenterology, Hepatology and Nutrition, Digestive Diseases and Surgery Institute; Cleveland Clinic, Cleveland, Ohio, United States.

Department of Medicine; Cleveland Clinic, Cleveland, Ohio, United States.

出版信息

Endosc Int Open. 2022 Jun 10;10(6):E801-E808. doi: 10.1055/a-1784-0806. eCollection 2022 Jun.

Abstract

Data are lacking on the natural history of gastrointestinal tract schwannomas. We aimed to study the natural history of all gastrointestinal schwannomas including location, diagnosis, management, and long-term outcomes. Patients with a pathological diagnosis of gastrointestinal schwannoma between January 2000 and March 2020 were identified. Data on baseline demographics, presentations, associated malignancies, malignant transformation, treatment, and recurrence were collected. Our cohort consisted of 44 patients with a mean age of 58.6 years, with 63.6 % women and 84.1 % White. The stomach (38.6 %) was the most common location followed by the colorectum (31.8 %). Only 22.7 % of patients were symptomatic and 22.0 % had a personal history of other malignancies. Tissue diagnosis was obtained via endoscopy in 47.7 % and from surgical pathology in 52.3 %. On histology, 65.9 % of the tumors were solid, 11.4 % had mixed features, and 2.3 % had necrosis. SP100 was tested in all but one patient and was positive in all. Mean Ki-67 in 12 patients with tumors measuring ≥ 2 cm was 3.0 % indicating a low proliferation rate. Of the patients, 77.3 % had surgery and 18.2 % underwent endoscopic resection. At a mean follow-up of 5.0 ± 4.31 years, there was no malignant transformation, recurrence or mortality associated with gastrointestinal schwannomas. Gastrointestinal schwannomas are diagnosed in the fifth to sixth decade with predominance in women and Whites. They are benign, mostly asymptomatic, and diagnosed incidentally. Asymptomatic gastrointestinal schwannomas including lesions ≥ 2 cm in size do not appear to need further monitoring or intervention. Patients with them should be counseled to remain up to date with routine screening guidelines pertaining to the colon, breast, and lung cancer due to the high incidence of concomitant malignancy.

摘要

目前缺乏关于胃肠道神经鞘瘤自然病史的数据。我们旨在研究所有胃肠道神经鞘瘤的自然病史,包括其位置、诊断、治疗及长期预后。我们确定了2000年1月至2020年3月间经病理诊断为胃肠道神经鞘瘤的患者。收集了患者的基线人口统计学数据、临床表现、相关恶性肿瘤、恶性转化、治疗及复发情况。我们的队列包括44例患者,平均年龄58.6岁,其中63.6%为女性患者,84.1%为白人。胃(38.6%)是最常见的发病部位,其次是结直肠(31.8%)。只有22.7%的患者有症状,22.0%有其他恶性肿瘤个人史。47.7%的患者通过内镜检查获得组织诊断,52.3%通过手术病理获得诊断。组织学检查显示,65.9%的肿瘤为实性,11.4%具有混合特征,2.3%有坏死。除1例患者外,所有患者均检测了SP100,结果均为阳性。12例肿瘤直径≥2 cm的患者,平均Ki-67为3.0%,表明增殖率较低。77.3%的患者接受了手术,18.2%接受了内镜切除。平均随访5.0±4.31年,未发现与胃肠道神经鞘瘤相关的恶性转化、复发或死亡。胃肠道神经鞘瘤多在五、六十岁被诊断,女性和白人占多数。它们是良性的,大多无症状,多为偶然发现。无症状的胃肠道神经鞘瘤,包括直径≥2 cm的病变,似乎无需进一步监测或干预。由于同时发生恶性肿瘤的发生率较高,应建议患有胃肠道神经鞘瘤的患者遵循有关结肠癌、乳腺癌和肺癌的常规筛查指南,定期进行筛查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/94dc/9187404/8cd3548d6589/10-1055-a-1784-0806-i2549ei1.jpg

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