Lipomatous hemangiopericytoma: A fat-containing variant of solitary fibrous tumor? Clinicopathologic, immunohistochemical, and ultrastructural analysis of a series in favor of a unifying concept

Lipomatous hemangiopericytoma: A fat-containing variant of solitary fibrous tumor? Clinicopathologic, immunohistochemical, and ultrastructural analysis of a series in favor of a unifying concept
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DOI:
10.1053/hupa.2000.9777
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发表时间:
2000-09-01
期刊:
影响因子:
3.3
通讯作者:
Coindre, JM
Coindre, JM
中科院分区:
医学3区
文献类型:
--
作者:
Guillou, L;Gebhard, S;Coindre, JM

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本文报告13例脂肪瘤性血管外皮细胞瘤的临床病理、免疫组化及超微结构特点。有6名男性和7名女性患者的诊断年龄范围为27至75岁(中位数48),所有表现为可变持续时间的肿块。肿瘤大小范围为1.7 cm至19 cm(中位数5.5 cm)。位置包括眼眶(1)、颈部(1)、纵隔(1)、心外膜(1)、腹膜后(3)、右髂窝(1)、上肢(1)和下肢(4)。组织学上,病变由梭形至圆形细胞、胶原基质、脂肪组织和分支的、通常为厚壁的血管外皮细胞瘤样血管的不同混合物组成。对于11个肿瘤,有丝分裂活性范围为每10个高倍视野(HPF)1 - 3个有丝分裂。免疫组化显示,瘤细胞波形蛋白、CD 99、CD 34均为阳性,而结蛋白、角蛋白、CD 31、CD 117(c-kit)、CD 117(c-kit)、CD 118-bin均为阴性。大约一半的肿瘤显示出对bcl-2的反应性。偶尔,也观察到平滑肌肌动蛋白,肌肉特异性肌动蛋白,S100蛋白和上皮膜抗原的局部反应。7例超微结构检查显示符合成纤维细胞,肌纤维母细胞,或周细胞分化的功能。单纯肿瘤切除术10例,广泛切除术3例。10名患者的随访信息(范围:6至77个月;中位数:18个月)显示无复发。脂肪瘤性血管外皮细胞瘤具有孤立性纤维瘤(SFT)的临床、病理、免疫组织化学和超微结构特征,在大多数情况下,可能代表SFT的含脂肪变体。(C)2000年,W.B.桑德斯公司
The clinicopathologic, 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. (C) 2000 by W.B. Saunders Company.