Takeuchi Tamotsu, Iwasaki Shinji, Miyazaki Junichi, Nozaki Yasuko, Takahashi Masaya, Ono Masafumi, Saibara Toshiji, Furihata Mutsuo
Department of Pathology, Kochi Medical School, Nankoku, Kochi, Japan.
Int J Clin Exp Pathol. 2010 Jun 25;3(6):634-9.
Angiosarcoma involving the serous membrane may mimic mesothelioma; therefore, the term "pseudomesotheliomatous angiosarcoma" has been suggested for this entity. However, the pathogenesis of pseudomesotheliomatous angiosarcoma remains unclear. Here, we report an autopsy case of splenic angiosarcoma, which systemically metastasized to the serous membrane of both the peritoneum and pleura, closely resembling a mesothelioma. The spindle-shaped tumor cells exhibited marked invasion of the lymphatic vessels and invaded the serous membrane causing thickening of the fibrous tissues like desmoplastic mesothelioma. In the present case, immunohistochemical staining showed that the tumor expressed not only the endothelial cell markers, such as CD31, vascular endothelial growth factor receptor 3, and podoplanin (D2-40), but also matrix metalloproteinase-1 (also known as collagenase-1), which is known to increase the invasiveness of mesothelioma cells. MMP-1 expression was not observed in the other cases of angiosarcoma, examined. This tumor might systemically metastasize to the serous membrane via the lymphatic route and might generate the fibrous stroma aided by the matrix metalloproteinase-1.
累及浆膜的血管肉瘤可能酷似间皮瘤;因此,有人提出用“假间皮瘤样血管肉瘤”这一术语来描述该实体。然而,假间皮瘤样血管肉瘤的发病机制仍不清楚。在此,我们报告一例脾脏血管肉瘤的尸检病例,该肿瘤全身转移至腹膜和胸膜的浆膜,与间皮瘤极为相似。梭形肿瘤细胞显示出明显的淋巴管浸润,并侵入浆膜,导致纤维组织增厚,类似促纤维增生性间皮瘤。在本病例中,免疫组化染色显示肿瘤不仅表达内皮细胞标志物,如CD31、血管内皮生长因子受体3和足板蛋白(D2-40),还表达基质金属蛋白酶-1(也称为胶原酶-1),已知该酶可增加间皮瘤细胞的侵袭性。在所检查的其他血管肉瘤病例中未观察到MMP-1表达。该肿瘤可能通过淋巴途径全身转移至浆膜,并可能在基质金属蛋白酶-1的作用下产生纤维性间质。