Department of Histopathology Birmingham Women's Hospital, Somerset, UK.
Int J Gynecol Pathol. 2009 Nov;28(6):589-93. doi: 10.1097/PGP.0b013e3181a3a4de.
The perivascular epithelioid cell family of tumors (PEComas) includes angiomyolipoma, lymphangioleiomyomatosis, clear-cell "sugar" tumor of the lung and a variety of extrapulmonary epithelioid and spindle-cell tumors referred to by various names. PEComas are now widely recognized histologically, and have been reported to occur at most anatomical sites, including the female genital tract. However, a distinctive variant of PEComa, designated "sclerosing PEComa," has only recently been described in the literature. All 13 of the cases of sclerosing PEComa reported thus far have been in women (mean age of 49 y), with a predilection for the retroperitoneum. Only one of the reported cases showed transition to a high-grade malignant phenotype. We report the first case of sclerosing PEComa arising in the uterine adnexa, with high-grade malignant transformation.
血管周上皮样细胞肿瘤家族（PEComas）包括血管平滑肌脂肪瘤、淋巴管平滑肌瘤病、肺透明细胞“糖”瘤和各种不同名称的肺外上皮样和梭形细胞肿瘤。PEComas 现在在组织学上得到广泛认可，并已报道发生在大多数解剖部位，包括女性生殖道。然而，一种独特的 PEComa 变体，称为“硬化性 PEComa”，最近才在文献中描述。迄今为止报告的 13 例硬化性 PEComa 均发生在女性（平均年龄 49 岁），偏爱腹膜后。报告的病例中只有一例显示向高级别恶性表型转化。我们报告首例发生在子宫附件的硬化性 PEComa，伴有高级别恶性转化。
