Zamecnik Michal, Michal Michal
Sikl's Department of Pathology, Charles University, Medical Faculty Hospital, Pilsen, Czech Republic.
Pathol Int. 2007 Jan;57(1):26-31. doi: 10.1111/j.1440-1827.2007.02052.x.
Pseudoangiomatous spindle cell lipoma (PASCL) is a rare variant of spindle cell lipoma that is composed of spindle fibroblast-like cells and mature adipose cells, and that is further characterized by intratumoral branching spaces regarded originally to be non-vascular (hence the adjective 'pseudoangiomatous'). Reported herein are three cases of this tumor, with literature review of all six cases described previously. PASCL appears to have clinical features (age, sex, location, prognosis) similar to those of conventional spindle cell lipoma (SCL). Immunohistochemically, the spindle cells in all of the present cases were positive for CD34, as in conventional SCL. All lesions had at least mild positivity for sex steroid hormone receptors. The cells lining intratumoral dilated and branching spaces typical of this variant were strongly positive for lymphatic endothelial marker D2-40 in all cases, and for vascular markers CD31 and F-VIII in one case. Ultrastructural study confirmed the endothelial nature of these cells. The results indicate that pseudoangiomatous SCL is truly angiomatous and should be termed as such. It could represent a sex steroid hormone-dependent lesion.
假血管瘤样梭形细胞脂肪瘤（PASCL）是梭形细胞脂肪瘤的一种罕见变体，由梭形成纤维细胞样细胞和成熟脂肪细胞组成，其进一步特征是肿瘤内存在分支间隙，最初认为这些间隙无血管（因此有形容词“假血管瘤样”）。本文报告了3例该肿瘤病例，并对先前描述的所有6例病例进行了文献综述。PASCL似乎具有与传统梭形细胞脂肪瘤（SCL）相似的临床特征（年龄、性别、部位、预后）。免疫组化显示，与传统SCL一样，所有病例中的梭形细胞CD34均呈阳性。所有病变的性类固醇激素受体至少呈轻度阳性。在所有病例中，该变体典型的肿瘤内扩张和分支间隙内衬的细胞对淋巴管内皮标记物D2-40呈强阳性，在1例中对血管标记物CD31和F-VIII呈阳性。超微结构研究证实了这些细胞的内皮性质。结果表明，假血管瘤样SCL实际上是血管瘤样的，应如此命名。它可能代表一种性类固醇激素依赖性病变。
