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病例报告:1型神经纤维瘤病合并甲状腺乳头状癌和胃肠道间质瘤。

Case Report: Concurrent neurofibromatosis type 1 with papillary thyroid carcinoma and gastrointestinal stromal tumor.

作者信息

Dweik Ruba, Faroun Jana, Yacoub Rita, Smerat Mohammad I, Abu Asbeh Yousef

机构信息

Medical Research Club, Faculty of Medicine, Al-Quds University, Jerusalem, Palestine.

Department of Radiology, Al-Ahli Hospital, West Bank, Hebron, Palestine.

出版信息

Front Oncol. 2025 Jul 10;15:1529765. doi: 10.3389/fonc.2025.1529765. eCollection 2025.

Abstract

Neurofibromatosis type 1 (NF1) is a genetic disorder characterized by benign tumors such as neurofibromas and café-au-lait spots, with affected individuals at increased risk for malignant tumors, including gastrointestinal stromal tumors (GIST) and, rarely, papillary thyroid carcinoma (PTC). This case report presents a 30-year-old Palestinian woman with NF1 who experienced severe abdominal pain and melena, leading to the diagnosis of a jejunal GIST, which was surgically removed. Postoperative imaging revealed cervical and thoracic lesions. A follow-up PET scan indicated hypermetabolic masses in the thyroid and chest. Subsequent surgery confirmed the diagnosis of PTC and neurofibromas, with whole exome sequencing identifying a likely pathogenic variant in the NF1 gene. This case demonstrates the value of comprehensive evaluation and genetic counseling for NF1 patients due to the risk of multiple tumors, which points to careful monitoring for early detection and management. To our knowledge, this instance is the first reported case of concurrent GIST and PTC in a patient with NF1.

摘要

1型神经纤维瘤病(NF1)是一种遗传性疾病,其特征为出现神经纤维瘤和咖啡斑等良性肿瘤,患者患恶性肿瘤的风险增加,包括胃肠道间质瘤(GIST),罕见的还有乳头状甲状腺癌(PTC)。本病例报告介绍了一名30岁患有NF1的巴勒斯坦女性,她经历了严重腹痛和黑便,最终诊断为空肠GIST,并接受了手术切除。术后影像学检查发现颈部和胸部有病变。后续的PET扫描显示甲状腺和胸部有高代谢肿块。随后的手术确诊为PTC和神经纤维瘤,全外显子测序确定了NF1基因中一个可能的致病变异。该病例表明,由于NF1患者存在多种肿瘤风险,对其进行综合评估和遗传咨询具有重要价值,这也提示要进行仔细监测以便早期发现和处理。据我们所知,这是首例报告的NF1患者同时并发GIST和PTC的病例。

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