Kanda M, Sonoyama A, Hirano H, Kizaki T, Ohara N
Department of Obstetrics and Gynecology, Sanda Municipal Hospital, Sanda, Japan.
Eur J Gynaecol Oncol. 2013;34(4):358-61.
The transition of low-grade endometrial stromal sarcoma (ESS) to high-grade ESS remains a rare clinical event.
A patient presented with abdominal pain and abnormal genital bleeding. She underwent a supracervical hysterectomy with bilateral salpingo-oophorectomy, omentectomy, and resection of peritoneal disseminated lesions. Pathological examination revealed low-grade ESS in the uterus and omentum. Immunohistochemical examination showed immunoreactivity for CD10 and Ki-67 (MIB1) in the uterus and omentum. However, estrogen receptor, progesterone receptor, alpha-SMA, desmin, h-caldesmon, and CAM5-2 were negative. P53 immunoreactivity was noted only in the omental lesion. Despite performing six courses of adjuvant chemotherapy, she recurred in the abdomen. She underwent ileostomy and resection of peritoneal disseminated lesions. Pathology showed high-grade ESS in the recurrent lesion of the ileum, which was characterized by severe cytologic atypia, high mitotic index, multifocal necrosis, increased Ki-67 index, and immunoreactivity for p53.
Although rare, the transition of low-grade ESS to high-grade ESS may occur and suggests the worsening of the prognosis. Pathological examination and immunohistochemistry are useful for the diagnosis of the transition of low-grade ESS to high-grade ESS.
低级别子宫内膜间质肉瘤（ESS）向高级别ESS的转变仍然是一种罕见的临床事件。
一名患者出现腹痛和生殖器异常出血。她接受了次全子宫切除术加双侧输卵管卵巢切除术、大网膜切除术以及腹膜播散性病变切除术。病理检查显示子宫和大网膜中有低级别ESS。免疫组化检查显示子宫和大网膜中CD10和Ki-67（MIB1）呈免疫反应性。然而，雌激素受体、孕激素受体、α-SMA、结蛋白、h-钙调蛋白和CAM5-2均为阴性。仅在大网膜病变中观察到p53免疫反应性。尽管进行了六个疗程的辅助化疗，她仍出现腹部复发。她接受了回肠造口术和腹膜播散性病变切除术。病理显示回肠复发病变中有高级别ESS，其特征为严重的细胞学异型性、高有丝分裂指数、多灶性坏死、Ki-67指数增加以及p53免疫反应性。
尽管罕见，但低级别ESS向高级别ESS的转变可能会发生，提示预后恶化。病理检查和免疫组化对诊断低级别ESS向高级别ESS的转变有用。
