Guillou L, Gebhard S, Coindre J M
University Institute of Pathology, Lausanne, Switzerland.
Hum Pathol. 2000 Sep;31(9):1108-15. doi: 10.1053/hupa.2000.9777.
The dinicopathologic, immunohistochemical and ultrastructural features of 13 lipomatous hemangiopericytomas are presented. There were 6 male and 7 female patients whose ages at diagnosis ranged from 27 to 75 years (median 48) all presenting with a mass of variable duration. The tumor sizes ranged from 1.7 cm to 19 cm (median 5.5 cm). The locations included the orbit (1), neck (1), mediastinum (1), epicardium (1), retroperitoneum (3), right iliac fossa (1), and upper (1) and lower (4) extremity. Histologically, the lesions were composed of a varying admixture of spindle-shaped to round cells, variably collagenous stroma, adipose tissue, and branched, often thick-walled, hemangiopericytoma-like vessels. For 11 tumors, the mitotic activity ranged from 1 to 3 mitoses per 10 high-power fields (HPF). One tumor which contained hypercellular areas showed 13 mitoses per 10 HPF, and another hypercellular lesion showed up to 43 mitoses per 10 HPF, abnormal mitoses, and necrosis. Immunohistochemically, tumor cells were invariably positive for vimentin and CD99, and mostly for CD34 but negative for desmin, keratin, CD31, CD117 (c-kit), and inhibin. About half of the tumors showed reactivity for bcl-2. Occasionally, focal reactivity was also observed for smooth muscle actin, muscle-specific actin, S100 protein, and epithelial membrane antigen. Ultrastructural examination of seven cases showed features in keeping with fibroblastic, myofibroblastic, or pericytic differentiation. Treatment consisted of simple tumorectomy in 10 cases and wide excision in 3. Follow-up information on 10 patients (range: 6 to 77 months; median: 18 months) showed no recurrence. Lipomatous hemangiopericytoma which share the clinical, pathologic, immunohistochemical, and ultrastructural features of solitary fibrous tumor (SFT) is likely to represent, in most cases, a fat-containing variant of SFT.
本文介绍了13例脂肪性血管外皮细胞瘤的临床病理、免疫组化及超微结构特征。患者6例男性,7例女性,诊断时年龄为27至75岁(中位年龄48岁),均表现为病程不等的肿块。肿瘤大小为1.7 cm至19 cm(中位大小5.5 cm)。肿瘤位于眼眶(1例)、颈部(1例)、纵隔(1例)、心外膜(1例)、腹膜后(3例)、右髂窝(1例)以及上肢(1例)和下肢(4例)。组织学上,病变由梭形至圆形细胞、不同程度的胶原性间质、脂肪组织以及分支状、常为厚壁的血管外皮瘤样血管混合组成。11例肿瘤的有丝分裂活性为每10个高倍视野(HPF)1至3个有丝分裂象。1例含有细胞增多区域的肿瘤每10个HPF有13个有丝分裂象,另1例细胞增多性病变每10个HPF有多达43个有丝分裂象,可见异常有丝分裂象及坏死。免疫组化方面,肿瘤细胞波形蛋白和CD99均呈阳性,多数CD34阳性,但结蛋白、角蛋白、CD31、CD117(c-kit)和抑制素均为阴性。约半数肿瘤bcl-2呈阳性反应。偶尔,平滑肌肌动蛋白、肌特异性肌动蛋白、S100蛋白和上皮膜抗原也可见局灶性阳性反应。7例的超微结构检查显示具有成纤维细胞、肌成纤维细胞或周细胞分化的特征。10例行单纯肿瘤切除术,3例行广泛切除术。10例患者的随访信息(范围:6至77个月;中位时间:18个月)显示无复发。脂肪性血管外皮细胞瘤具有孤立性纤维性肿瘤(SFT)的临床、病理、免疫组化及超微结构特征,在大多数情况下可能代表含脂肪的SFT变异型。