Division of Pathological Anatomy, University of Florence, Florence, Italy.
Histol Histopathol. 2012 Sep;27(9):1175-81. doi: 10.14670/HH-27.1175.
Tumours of perivascular epithelioid cells (PEComas) are a heterogeneous group of uncommon mesenchymal neoplasms which exhibit a peculiar immunohistochemical co-expression of muscle and melanocytic markers. PEComas occur at various visceral and soft tissue sites, generally with a benign clinical course. Nevertheless, there has been evidence of cases having an unfavourable outcome, thus prompting investigation of pathological criteria for malignancy. A sclerosing variant of PEComa, more frequently encountered in the retroperitoneum of middle-aged women, has been reported. Prognosis has generally been regarded as favourable and complete surgical excision appears to be adequate treatment. To the best of our knowledge, only two cases of sclerosing PEComa displayed high-grade malignant morphology and were associated with adverse outcome. An additional case of retroperitoneal sclerosing PEComa with a two-year follow-up and indolent behaviour is herein described. Light and electron microscopy were performed, along with immunohistochemical analysis. Further studies are needed to clarify the histogenesis and to predict the biological behaviour of this uncommon entity.
血管周上皮样细胞肿瘤（PEComas）是一组异质性的不常见间叶性肿瘤，具有独特的免疫组织化学肌肉和黑色素细胞标志物共同表达。PEComas 发生在各种内脏和软组织部位，通常具有良性的临床过程。然而，已经有证据表明有些病例预后不良，因此促使人们研究恶性肿瘤的病理标准。PEComa 的硬化变体更常发生在中年女性的腹膜后，已有报道。一般认为预后良好，完全手术切除似乎是足够的治疗方法。据我们所知，只有两例硬化性 PEComa 表现出高级别恶性形态，并与不良预后相关。本文描述了一例附加的腹膜后硬化性 PEComa 病例，随访两年，表现为惰性行为。进行了光镜和电镜检查，并进行了免疫组织化学分析。需要进一步的研究来阐明这种罕见实体的组织发生，并预测其生物学行为。
