本文报道了以子宫内膜息肉为临床表现的血管周围上皮样细胞肿瘤(PEComa)的病理特征和恶性生物学行为。一年前,该病例在当地一家医院刮治。术后诊断为“子宫内膜息肉”。这一次,由于“不规则出血”,我们在医院又做了一次刮宫手术。手术后,检查3个直径为0.3厘米的息肉,0.5厘米和0.6厘米,分别。肿瘤由具有肺泡和嵌套模式的上皮样细胞组成,并显示HMB45,Melan-A和TFE3的弥漫性强表达。患者随后接受了子宫切除术,并将“息肉”送去进行病理检查。结果显示肿瘤细胞浸润深层肌肉,靠近外膜,暗示了一种恶性生物学行为.TFE3相关的PEComa不同于一般的PEComa。该肿瘤和黑素瘤Xp11肾癌具有相似的临床病理特征,组织学,免疫和分子表型,属于同一类型的肿瘤。文献中建议将这种肿瘤命名为“具有黑素细胞分化的Xp11肿瘤”或“黑素性Xp11肿瘤”。我们的案例扩展了我们对PEComa特征的理解,并增加了TFE3易位相关PEComa的数据,提醒我们在PEComa表现为小息肉时避免误诊。
This article
reports the pathologic features and malignant biological behavior of a perivascular epithelioid cell neoplasm (PEComa) with the clinical manifestation being endometrial polyps. The
case was cured with curettage in a local hospital one year ago. The postoperative diagnosis was \"endometrial polyps\". This time, due to \"irregular bleeding\", we carried out another curettage in our hospital. After the operation, 3 pieces of polyps were inspected with diameters of 0.3 cm, 0.5 cm and 0.6 cm, respectively. The tumor consisted of epithelioid cells with alveolar and nesting pattern and showed a diffuse strong expression of HMB45, Melan-A and TFE3. The patient then underwent a hysterectomy and the \"polyps\" were sent for pathological examination. The result showed that tumor cells infiltrated the deep muscle layer, close to the outer membrane, suggesting a malignant biologic behavior. TFE3-related PEComa is different from general PEComa. This neoplasm and Melanotic Xp11 renal carcinoma have similar clinicopathologic features, histology, immunity and molecular phenotypes, belonging to the same type of tumor. It has been suggested in the literature naming this neoplasm as \'Xp11 neoplasm with melanocytic differentiation\' or \'melanotic Xp11 neoplasm\'. Our
case has expanded our understanding of PEComa characteristics and increased data for TFE3 translocation-related PEComa, reminding us to avoid misdiagnosis when PEComa manifests as small polyps.