Froio Elisabetta, Piana Simonetta, Cavazza Alberto, Valli Riccardo, Abrate Martino, Gardini Giorgio
Department of Pathology, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy.
Int J Surg Pathol. 2008 Oct;16(4):443-6. doi: 10.1177/1066896908316067. Epub 2008 May 21.
The authors describe a case of multifocal perivascular epithelioid cell tumor (PEComa) arising in the pelvis of a 39-year-old woman affected by tuberous sclerosis. The tumor presented in the form of multiple fascicular, focally cystic nodules involving the uterine corpus, both ovaries, and the omentum. Microscopically, the nodules were composed of foci of adenomyosis and endometriosis (with focal atypical complex hyperplasia) associated with a stromal spindle cell population immunoreactive for HMB-45, smooth muscle actin, and estrogen and progesterone receptors. We interpret these foci as the result of a widespread proliferation of perivascular epithelioid cells (PEC). Because of the diffuse quality of the process, the designation of PEComatosis seems warranted.
作者描述了一例发生在一名39岁患有结节性硬化症女性盆腔的多灶性血管周上皮样细胞瘤（PEComa）。肿瘤表现为多个束状、局灶性囊性结节，累及子宫体、双侧卵巢和大网膜。显微镜下，结节由子宫腺肌病和子宫内膜异位症病灶（伴有局灶性非典型复杂性增生）组成，伴有对HMB-45、平滑肌肌动蛋白以及雌激素和孕激素受体呈免疫反应的基质梭形细胞群。我们将这些病灶解释为血管周上皮样细胞（PEC）广泛增殖的结果。由于该过程的弥漫性，PEComatosis这一命名似乎是合理的。
