Lubo Idania, Fermín Ileana, Massarelli Olindo, Gobbi Roberta, Cossu Rocca Paolo
Anatomy and Pathology Institute "Dr. José Antonio O'Daly", Central University of Venezuela, Los Chaguaramos 1040, Caracas, Venezuela.
Maxillofacial Surgery Unit, University of Sassari, Italy.
Case Rep Pathol. 2016;2016:1936421. doi: 10.1155/2016/1936421. Epub 2016 Jan 26.
The Perivascular Epithelioid Cell tumours (PEComas) are rare mesenchymal neoplasms recognized as entity by the World Health Organization. The tumour cells have an uncertain origin and are characterized by distinctive histological and immunohistochemical features. We report a case of PEComa occurring as intraorbital lesion in a 47-year-old man. We found only two other cases described in the literature and we considered all three cases together in order of histology, immunohistochemistry, and clinical outcome. We found a strict histological overlapping and quite similar immunohistological results. All three cases showed a favourable clinical course probably related to small size of tumours (<5 cm), low mitotic rate (<2 mitoses in 50 HPF), and absence of necrosis.
血管周上皮样细胞瘤（PEComas）是一种罕见的间叶性肿瘤，被世界卫生组织认定为独立的肿瘤类型。肿瘤细胞起源不明，具有独特的组织学和免疫组化特征。我们报告一例47岁男性眼眶内发生的PEComa病例。我们在文献中仅发现另外两例相关病例，并从组织学、免疫组化和临床结果方面对这三例病例进行综合分析。我们发现它们在组织学上有严格的重叠，免疫组化结果也非常相似。所有三例病例的临床病程均良好，这可能与肿瘤体积小（<5厘米）、有丝分裂率低（50个高倍视野中<2个有丝分裂象）以及无坏死有关。
