Zelger B W, Zelger B G, Steiner H, Ofner D
Department of Dermatology, University of Innsbruck, Austria.
J Clin Pathol. 1996 Apr;49(4):313-8. doi: 10.1136/jcp.49.4.313.
To describe the clinicopathological features of 33 aneurysmal fibrous histiocytomas (AFH), including five cases with a haemangiopericytoma-like pattern.
Thirty three cases of AFH were studied by using routine histology and immunohistochemistry for factor XIIIa, the "cell activity marker" E9 (anti-metallothionein), NK1C3 (CD57), smooth muscle actin (SMA), factor VIII, ulex europaeus agglutinin, JC70A (CD31), and QBEND10 (CD34). The time dependent variation in histopathological features was evaluated by statistical methods (Pearson chi 2, likelihood ratio chi 2).
Of the AFHs, 29 of 33 occurred on the extremities of adults (age range 30 to 50 years), six of which were associated with rapid growth, probably caused by trauma, and pain. Twenty one lesions were thought to be vascular and/or melanocytic lesions, including two melanomas, because of a bluish-black and/or cystic appearance. Histologically, large areas of haemorrhage, up to 50% of the tumour bulk, lacking an endothelial lining were seen in otherwise typical fibrous histiocytomas. Five cases resembled nodular stages of Kaposi's sarcoma. Variable haemosiderin deposition in histiocytes (18/33) and giant cells (11/33) was suggestive of haemosiderotic histiocytoma. A haemangiopericytoma-like pattern was seen in five otherwise indistinguishable cases. On immunohistochemistry, variable reactivity was seen for factor XIIIa (18/30), with E9 (18/30), NK1C3 (19/30), and for SMA (14/30), but labelling for vascular markers was not detected. Early lesions without iron deposition were factor XIIIa positive; late lesions with iron deposition were factor XIIIa negative. Labelling for SMA correlated with prominent sclerosis.
AFHs, including a haemangiopericytoma-like variant, have a characteristic time dependent histological and immunophenotypic profile, clearly different from nodular type Kaposi's sarcoma.
描述33例动脉瘤样纤维组织细胞瘤（AFH）的临床病理特征，包括5例具有血管外皮细胞瘤样形态的病例。
采用常规组织学及免疫组织化学方法对33例AFH进行研究，检测因子ⅩⅢa、“细胞活性标志物”E9（抗金属硫蛋白）、NK1C3（CD57）、平滑肌肌动蛋白（SMA）、因子Ⅷ、荆豆凝集素、JC70A（CD31）和QBEND10（CD34）。采用统计学方法（Pearson卡方检验、似然比卡方检验）评估组织病理学特征的时间依赖性变化。
33例AFH中，29例发生于成人四肢（年龄范围30至50岁），其中6例与快速生长有关，可能由创伤和疼痛引起。21个病变因呈蓝黑色和/或囊性外观而被认为是血管性和/或黑素细胞性病变，包括2例黑色素瘤。组织学上，在其他方面典型的纤维组织细胞瘤中可见大片出血，占肿瘤体积的50%，且缺乏内皮衬里。5例类似于卡波西肉瘤的结节期。组织细胞（18/33）和巨细胞（11/33）中可见不同程度的含铁血黄素沉积，提示含铁血黄素性组织细胞瘤。5例在其他方面难以区分的病例呈现血管外皮细胞瘤样形态。免疫组织化学显示，因子ⅩⅢa（18/30）、E9（18/30）、NK1C3（19/30）和SMA（14/30）有不同程度的反应性，但未检测到血管标志物的标记。无铁沉积的早期病变因子ⅩⅢa阳性；有铁沉积的晚期病变因子ⅩⅢa阴性。SMA标记与显著硬化相关。
AFH，包括血管外皮细胞瘤样变体，具有特征性的时间依赖性组织学和免疫表型特征，明显不同于结节型卡波西肉瘤。
