脊髓淋巴缺陷与乳头内皮扩散:一种罕见的组织学变体还是独特的实体?
Jonathan C Slack1, Juan Putra1, Michael J Callahan2
1Departments of Pathology.
The American journal of surgical pathology
|June 19, 2023
概括
带有乳头内皮扩散的淋巴瘤 (LMs-PEPs) 是一种罕见的良性淋巴发育不良. 这项研究证实了它们独特的良性临床和组织学特征,使它们与侵袭性脏瘤有所区别.
科学领域:
- 病理学 病理学 病理学
- 血管异常 血管异常
- 在瘤学瘤学.
背景情况:
- 淋巴发育不良 (LMs) 是先天性淋巴系统异常,通常是多焦点的,并与综合征相关.
- 脏LMs很少见,通常发生在多器官淋巴细胞瘤中.
- 带有 papillary endothelial proliferation (LM-PEPs) 的骨LM可以模仿侵略性瘤,但其性质尚不清楚.
研究的目的:
- 要确定脏LM-PEP是否是一个独特的实体或LM的变体.
- 系统地评估脏LM-PEPs的临床,组织学,放射学,电子显微镜和分子特征.
主要方法:
- 骨LM-PEP病例的回顾性,单一机构审查.
- 评估包括临床过程,组织学,放射学,电子显微镜和下一代分子变化测序.
主要成果:
- 所有3例脏LM-PEP都表现出良性临床过程.
- 放射学显示了具有"螺旋轮"模式的亚囊性病变;组织学显示了淋巴细胞微囊内独特的PEP.
- 免疫组织化学和电子显微镜证实了淋巴内皮细胞的表型;在一名患者中发现了PIK3CA突变.
结论:
- 脏LM-PEP是一种具有特征特征的良性实体.
- 诊断因素可以将其与更具攻击性的脏淋巴血管瘤区分开来.
- 进一步的研究可能会澄清这种罕见疾病的分子基础和分类.
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