胸腔孤立性纤维瘤伴阵发性低血糖症1例报道并文献复习

Solitary fibrous tumor of pleura with episodic hypoglycemia:a case report and review of literature

目的 研究胸腔孤立性纤维性肿瘤 (PSFT)伴阵发性低血糖的病理特点和鉴别诊断。方法 通过组织形态学和免疫组化对胸腔孤立性纤维性肿瘤进行病理分析并复习文献。结果 肿瘤细胞由纤维母细胞样梭形细胞构成 ,束状交错排列及片状分布 ,局部呈血管外皮瘤形态。间质内有大量胶原纤维 ,部分区域黏液变性。在细胞密集区 ,可见核分裂象 3个 / 10HPF。CD34、vimentin、somatotastin和insulin(+) ;CK、HBME 1,calretinin、EMA、AE1/AE3、Cam5 2和p5 3(- )表达。结论 PSFT确能合成和分泌胰岛素样物质及生长抑素 ,但低血糖症则由多种因素造成。vimentin和CD34阳性以及上皮性标记阴性有助于诊断。

Objective To study the clinicopathologic features of pleura solitary fibrous tumor (PSFT) with episodic hypoglycemia and its differential diagnosis. Methods A case of PSFT with severe episodic hypoglycemia was studied by morphological and immunohistochemical techniques. Results The tumor located in the thorax with 19?cm×12?cm×10?cm in size and showed gray-yellow appearance on cut surface. Microscopically, the neoplasm was composed of fibroblastic-like spindle cells in a collagenous background. The tumor cells were arranged in strands among collagen bundles and some parts of stroma showed myxoid changes. In more hypercellular regions, there were a few mitotic figures (<3/10HPF). Immunohistochemical staining revealed that tumor cells were positively reacted with CD34, somatostatin, insulin, and vimentin, but negatively with pan-cytokeratin, AE1/AE3, Cam5.2, HBME-1, calretinin, EMA, and p53. Conclusions PSFT is a rare specific tumor arising from mesenchymal which does produce and secret insulin-like substance and somatostatin. The cause of hypoglycemia in this case of PSTF may result from multiple factors. It favors the diagnosis of SFP if the spindle tumor cells react positively with vimentin and CD34 but negatively with epithlial markers on immunohistochemistry.