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伴有EWSR1-CREB1基因融合的血管样纤维组织细胞瘤发生于肺和肋骨并伴有全身多处转移:1例报告及文献复习

Angiomatoid fibrous histiocytoma with EWSR1-CREB1 gene fusion occurs in lungs and ribs with systemic multiple metastases: a case report and review of the literature.

作者信息

Feng Dongmei, Li Ying, Li Zhengjin, Pan Yun, Gao Yixuan, Cha Jinyan, Zhang Chunmei

机构信息

Department of Pathology, The First Affiliated Hospital of Dali University, Dali, Yunnan, China.

Department of Pathology, People's Hospital of Xiangyun County, Xiangyun, Yunnan, China.

出版信息

Front Oncol. 2025 Jan 8;14:1420597. doi: 10.3389/fonc.2024.1420597. eCollection 2024.

DOI:10.3389/fonc.2024.1420597
PMID:39845329
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11750646/
Abstract

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor with intermediate malignant potential, and it rarely metastasizes. We encountered a unique AFH case where, the tumor was discovered initially in unusual locations-the left lung and the left 4th rib. Combined histological features with FISH and NGS analysis, the diagnosis of AFH was supported, however, it is difficult to determine which of these two is the primary lesion. Eight months after the initial surgery, multiple systemic metastases were detected, eventually leading to the patient's death 18 months later due to widespread metastasis. Our case signifies the first reported occurrence of systemic metastasis in either bone-originating or pulmonary-originating AFH, and it is the initial instance of mortality resulting from multifocal metastasis originating from an atypical site.

摘要

血管样纤维组织细胞瘤(AFH)是一种具有中等恶性潜能的罕见软组织肿瘤,很少发生转移。我们遇到了一例独特的AFH病例,肿瘤最初在不寻常的部位被发现——左肺和左第4肋骨。结合组织学特征以及FISH和NGS分析,支持AFH的诊断,然而,很难确定这两个部位哪个是原发灶。初次手术后8个月,检测到多处全身转移,最终患者在18个月后因广泛转移死亡。我们的病例是首次报道的骨源性或肺源性AFH发生全身转移的情况,也是首例因非典型部位多灶转移导致死亡的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b48d/11750646/e429813c1f51/fonc-14-1420597-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b48d/11750646/82354f50325c/fonc-14-1420597-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b48d/11750646/e429813c1f51/fonc-14-1420597-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b48d/11750646/82354f50325c/fonc-14-1420597-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b48d/11750646/e429813c1f51/fonc-14-1420597-g002.jpg

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