Department of Pathology, Ningbo Diagnostic Pathology Center, Huan Cheng Bei Lu, Ningbo, Zhejiang, China.
J Clin Lab Anal. 2022 Jul;36(7):e24502. doi: 10.1002/jcla.24502. Epub 2022 Jun 1.
Endometrial stromal tumours are uncommon tumours of the uterus. They mainly occur in perimenopausal women. Tumours with typical clinicopathological features do not usually pose diagnostic problems. However, rare clinicopathological features can occur, and clinicians without significant experience may have difficulty diagnosing these tumours and managing these patients.
Herein, we report a case of endometrial stromal sarcoma that occurred in a 25-year-old woman. The pathological features, immunophenotype, treatment and prognosis were discussed.
The tumour revealed morphological heterogeneity, and there were similar proliferative-type endometrial stromal cells, an extensive amount of mature adipose tissue, and prominent rhabdomyoblastic and smooth muscle cells. Histopathological and immunohistochemical studies confirmed low-grade endometrial stromal sarcoma with smooth muscle, adipocytic and rhabdomyoblastic differentiation (approximately 60% were differentiated tissues). The final treatment of the tumour was total abdominal hysterectomy with bilateral salpingo-oophorectomy. There was no evidence of recurrence for 109 months postoperatively.
We found that low-grade endometrial stromal tumours with extensive adipocytic and prominent rhabdomyoblastic differentiation are misdiagnosed because they are infrequent. They must be differentiated from rhabdomyosarcoma with accurate identification of adipocytes, and long-term follow-up is needed.
子宫内膜间质肿瘤是一种罕见的子宫肿瘤。它们主要发生在围绝经期妇女中。具有典型临床病理特征的肿瘤通常不会造成诊断问题。然而，可能会出现罕见的临床病理特征，缺乏丰富经验的临床医生可能难以诊断这些肿瘤并管理这些患者。
本文报道了一例发生在 25 岁女性的子宫内膜间质肉瘤病例。讨论了其病理特征、免疫表型、治疗和预后。
肿瘤表现出形态异质性，有类似增殖型子宫内膜间质细胞、大量成熟脂肪组织以及突出的横纹肌样细胞和平滑肌细胞。组织病理学和免疫组织化学研究证实为低度分化子宫内膜间质肉瘤，具有平滑肌、脂肪细胞和成肌分化（约 60%为分化组织）。肿瘤的最终治疗方法是全子宫切除术和双侧输卵管卵巢切除术。术后 109 个月无复发证据。
我们发现，具有广泛脂肪细胞分化和突出横纹肌样分化的低度子宫内膜间质肿瘤由于罕见而容易误诊。必须通过准确识别脂肪细胞与横纹肌肉瘤进行鉴别，需要长期随访。
