血管内大b细胞淋巴瘤伴胸膜孤立性纤维瘤1例并文献复习

Amintas Samuel , Laurent Elodie , Gros Audrey , Sesboue Come , Merlio Jean-Philippe , Parrens Marie
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引用次数: 0

摘要

血管内大b细胞淋巴瘤(IVLBCL)是一种非常罕见的结外大b细胞淋巴瘤,选择性生长在血管内,可扩散到任何器官或组织。与恶性肿瘤同时发生的病例很少。我们报告一例罕见的IVLBCL合并胸膜恶性孤立性纤维瘤(MSFT)。76岁男性,表现为慢性干咳、发热、轻度全身状态恶化和全血细胞减少症。影像学显示一大片胸膜肿块。组织学上,肿块由MSFT组成。然而,CD20+恶性圆形细胞散在瘤内血管腔内,提示IVLBCL的同步发生。淋巴克隆分子分析鉴定出MYD88和CD79B基因突变。在胸膜肿块切除后,global health患者的细胞减少矫正得到改善。然而,术后4个月出现总体恶化,伴有腹膜后大肿块,其病理和分子模式与最初的IVLBCL克隆相同。
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Intravascular large B-cell lymphoma involving pleural solitary fibrous tumor: A case report and literature review

Intravascular large B-cell lymphoma (IVLBCL) is a very rare type of extranodal large B-cell lymphoma that selectively grows within vessels and can spread to any organs or tissues. A very few cases of synchronicity with malignant tumor have yet been described. We report a rare case of IVLBCL accompanying a pleural malignant solitary fibrous tumor (MSFT). A 76-year-old man presented with a chronic dry cough, fever, minor general state deterioration and pancytopenia. Imaging revealed a large pleural mass. Histologically, the mass consisted of a MSFT. However, CD20+ malignant round cells were scattered within lumina of intratumoral blood vessels, evidencing a synchronous IVLBCL occurrence. Molecular analysis of the lymphoid clone identified MYD88 and CD79B gene mutations. After pleural mass excision, global health’s patient improved with cytopenia correction. However, a general state deterioration appeared 4 months after surgery, associated with a large retroperitoneal mass presenting the same pathological and molecular patterns identical to the initial IVLBCL clone.

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来源期刊
Human Pathology: Case Reports
Human Pathology: Case Reports Medicine-Pathology and Forensic Medicine
CiteScore
0.50
自引率
0.00%
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0
审稿时长
16 weeks
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