摘要目的 探讨非特殊性血管周上皮样细胞肿瘤(PEComa-NOS)的临床病理学特征,评价恶性血管周上皮样细胞肿瘤(PEComa)的诊断标准.方法 回顾性复习31例PEComa-NOS的临床表现、影像学资料、光镜形态和免疫学表型,分析预后资料.2例为空芯针穿刺活检标本,2例为剖腹探查活检标本,其余27例为手术切除标本.结果 患者中女性24例,男性7例,平均年龄40岁.肿瘤发生于女性生殖系统12例,腹盆腔10例,胃肠道4例,大腿2例,纵隔、左腹股沟和膀胱各1例.所有病例均不伴有结节性硬化.镜下观察:23例(74%)呈透明细胞糖瘤样,4例(13%)呈透明细胞肌色素细胞瘤样,4例(13%)为上皮样-梭形细胞混合型.参照Folpe等提出的诊断标准,19例(61%)符合恶性PEComa;7例(23%)为恶性潜能未定PEComa;5例(16%)为良性PEComa.免疫组织化学染色显示,所有病例均表达HMB45(31/31,100%),部分病例尚表达平滑肌肌动蛋白(14/21,67%)和结蛋白(6/18).随访16例恶性PEComa,7例无瘤生存,6例带瘤生存,3例死亡;16例中复发6例,转移5例.随访4例恶性潜能未定PEComa,1例死亡,3例无瘤生存.随访3例良性PEComa,均无瘤生存.结论 (1)Folpe等提出的PEComa分类标准具有可重复性,但在诊断穿刺或活检标本时需注意结合临床和影像学;(2)PEComa的组织学形态在不同的病例间有一定的差异,熟悉其形态变化谱有助于诊断和鉴别诊断.

abstractsObjective To study the clinicopathologic characteristics of perivascular epithelioid cell tumor (PEComa), not otherwise specified (NOS) and to evaluate the diagnostic criteria for malignancy.Methods The clinical and pathologic features of 31 cases of PEComa-NOS were reviewed. The follow-up data available were analyzed. Results There were a total of 24 females and 7 males. The age of the patients ranged from 13 to 66 years (mean =40 years). The site of tumor occurrence included gynecologic organs (n = 12), intraabdominal/peritoneal soft tissue (n = 10), gastrointestinal tract (n = 4), thigh (n = 2), mediastinum (n = 1), left groin (n = 1) and urinary bladder (n = 1). None of the cases was associated with tuberous sclerosis complex. Histologic examination showed that 23 cases (74%) were clear cell sugar tumor-like, 4 cases (13%) were clear cell myomelanocytic tumor-like and 4 cases (13%) were of mixed epithelioid-spindled morphology. According to the classification system proposed by Folpe et al,19 cases (61%) were classified as malignant, 7 cases (23%) as PEComa of uncertain malignant potential and 5 cases (16%) as benign. The expression rates of HMB45, smooth muscle actin and desmin in tested cases were 100% (31/31), 67% (14/21) and 6/18, respectively. Follow-up data (1 to 56 months) were available in 23 cases (74%). Amongst the 16 cases of malignant PEComa, 7 patients were still alive with no evidence of disease, 6 patients were alive with unresectable or recurrent/metastatic disease and 3 patients died of the disease. The local recurrence and metastasis in those 16 cases were 6 cases and 5 cases,respectively. One of the 4 patients with PEComa of uncertain malignant potential died, while the remaining 3 patients and all of the patients with benign PEComa had an uneventful clinical course. Conclusions The classification system of PEComas proposed by Folpe et al. is reliable in routine practice. Correlatio1 with the clinical and radiologic findings however is prudent when dealing with core biopsy specimens or sampling from exploration laparotomy. Owing to the histologic heterogeneity of this entity, thorough understanding of the morphologic spectrum is essential in arriving at a correct diagnosis.