[Uterine epithelioid leiomyosarcoma with PGR::NR4A3 fusion gene: report of a case]

Zhonghua Bing Li Xue Za Zhi. 2026 May 8;55(5):476-479. doi: 10.3760/cma.j.cn112151-20251227-00855.
[Article in Chinese]

Abstract

本文报道1例子宫伴PGR::NR4A3基因融合的上皮样平滑肌肉瘤。患者女,29岁。肿瘤呈多结节状生长,肿瘤细胞分布不均,疏密不等,部分区域细胞密度高,以上皮样细胞形态为主,细胞中-重度异型,部分呈横纹肌细胞样形态,部分区域肿瘤细胞呈短梭形。胞质嗜酸性,胞核空泡状,核仁明显。间质呈水肿、黏液样改变。免疫表型:结蛋白少量阳性,h-caldesmon阳性,SMA阳性,ER中等阳性,PR强阳性,WT1部分阳性,NOR-1仅在黏液样基质区域中呈胞核强阳性表达,其余上皮样区域以及梭形细胞区域均未见表达,CKpan、CD10、抑制素、Calretinin均为阴性,Ki-67阳性指数40%。.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • DNA-Binding Proteins* / genetics
  • DNA-Binding Proteins* / metabolism
  • Female
  • Gene Fusion*
  • Humans
  • Leiomyosarcoma* / genetics
  • Leiomyosarcoma* / metabolism
  • Leiomyosarcoma* / pathology
  • Leiomyosarcoma* / surgery
  • Oncogene Proteins, Fusion* / genetics
  • Oncogene Proteins, Fusion* / metabolism
  • Receptors, Progesterone* / genetics
  • Receptors, Progesterone* / metabolism
  • Receptors, Steroid* / genetics
  • Receptors, Steroid* / metabolism
  • Receptors, Thyroid Hormone
  • Uterine Neoplasms* / genetics
  • Uterine Neoplasms* / metabolism
  • Uterine Neoplasms* / pathology
  • Uterine Neoplasms* / surgery

Substances

  • Receptors, Progesterone
  • DNA-Binding Proteins
  • Receptors, Steroid
  • NR4A3 protein, human
  • Oncogene Proteins, Fusion
  • Receptors, Thyroid Hormone