Department of Respiratory Medicine, Shandong Provincial Qianfoshan Hospital, Shandong University, Jinan, China.
Department of Pathology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China.
Thorac Cancer. 2017 Sep;8(5):530-534. doi: 10.1111/1759-7714.12457. Epub 2017 May 25.
Perivascular epithelioid cell tumor of the lung, also known as clear cell "sugar" tumor, is a rare benign tumor arising from perivascular epithelioid cells. Herein, we present a case of spindle cell subtype of pulmonary perivascular epithelioid cell tumor with prominent calcification and malignant potential in a 49-year-old woman. Histologically, the striking feature of this lesion was attributed to the presence of spindle cells arranged in a diffuse pattern, which is a pitfall for diagnosis. However, some of the lesion contained polygonal tumor cells with clear abundant cytoplasm surrounded by thin-walled vascular spaces. The size of the tumor and its Ki-67 index suggested malignant potential, and calcification was another rare characteristic. Immunostaining indicated that the tumor cells were positive not only for HMB-45 and Melan A, but also for CD34 and CD1a. This tumor should be distinguished from tumors with rich spindle cells such as sarcoma, clear cell carcinoma, or metastatic tumors. The patient in this case was alive with no tumor recurrence or metastasis six months after lobectomy.
肺血管周上皮细胞瘤，也称为透明细胞“糖”瘤，是一种罕见的良性肿瘤，起源于血管周上皮细胞。本文报道了一例 49 岁女性肺血管周上皮细胞瘤梭形细胞亚型，伴显著钙化和恶性潜能。组织学上，该病变的显著特征是存在弥漫排列的梭形细胞，这是诊断的陷阱。然而，病变的一些区域包含多边形肿瘤细胞，其胞质丰富、透明，周围有薄壁血管腔。肿瘤的大小及其 Ki-67 指数提示恶性潜能，钙化是另一个罕见特征。免疫组化染色表明肿瘤细胞不仅表达 HMB-45 和 Melan A，还表达 CD34 和 CD1a。该肿瘤应与富含梭形细胞的肿瘤如肉瘤、透明细胞癌或转移性肿瘤相鉴别。本例患者行肺叶切除术后 6 个月，无肿瘤复发或转移，仍存活。
