INFLAMMATORY FIBROUS HISTIOCYTOMA - AGGRESSIVE AND LETHAL LESION
INFLAMMATORY FIBROUS HISTIOCYTOMA - AGGRESSIVE AND LETHAL LESION
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DOI：
10.1002/1097-0142(197603)37:3
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发表时间：
1976-01-01
期刊：
影响因子：
6.2
通讯作者：
KEMPSON, RL
中科院分区：
文献类型：
--
作者：
KYRIAKOS, M;KEMPSON, RL
在软组织纤维组织细胞瘤的研究中，发现了7例可能代表纤维组织细胞瘤的一个新的和特异性的诊断亚组。所有患者均为成人（平均52.6岁），其中只有1例年龄小于40岁。有4名女性和3名男性;均为白人。肿瘤体积较大，平均8.5cm，好发于腹膜后、前胸壁、前腹壁、股部及口腔，虽然看起来有包囊，但在显微镜下是浸润性的。所有病例的共同组织学特征是弥漫性的，有时是强烈的嗜中性粒细胞浸润，与组织坏死无关，与温和的和间变性的组织细胞相结合。这种急性渗出反应的存在是这些病变的独特特征，不仅存在于原发肿瘤中，而且也存在于复发和转移灶中。其他细胞类型包括泡沫细胞、淋巴细胞、浆细胞、嗜酸性粒细胞、Reed-Sternberg样细胞和神经节样细胞。除1例外，其他所有病例均在某个时间出现了纤维组织细胞瘤的标志性特征--Storiform纤维结构。这些病变的危险方面是它们最初的组织学表现。泡沫细胞灶与炎性成分相关常导致良性炎症反应或黄色肉芽肿的诊断。随访证实了这些病变的侵袭性和肿瘤性。临床病程通常延长，多次复发并最终转移。所有患者均死于肿瘤，平均生存期为53个月; 4例存活5年以上。术语炎性纤维组织细胞瘤建议指定这些病变。
During an investigation of soft tissue fibrohistiocytic tumors, 7 cases were found which may represent a new and specific diagnostic subgroup of the fibrous histiocytomas. All patients were adults (mean 52.6 yr), only 1 of whom was younger than age 40. There were 4 females and 3 males; all were Caucasian. The tumors, which occurred in the retroperitoneum, anterior chest wall, anterior abdominal wall, femoral area and oral cavity, were large, averaging 8.5 cm., and although appearing encapsulated, were microscopically infiltrative. The common histologic feature of all cases was a diffuse and at times intense neutrophilic infiltrate unassociated with tissue necrosis, in combination with bland and anaplastic appearing histiocytes. The presence of this acute exudative reaction is a unique feature of these lesions, and was present not only in the original tumors, but in recurrences and metastatic foci as well. Other cell types including foam cells, lymphocytes, plasma cells, eosinophils, Reed-Sternberg-like and ganglion-like cells were commonly present. A storiform fibrous pattern, a hallmark of the fibrous histiocytomas was seen at some time in all but 1 case. The treacherous aspect of these lesions was their bland initial histologic appearance. The foci of foam cells associated with the inflammatory elements often led to a diagnosis of a benign inflammatory reaction or xanthogranuloma. Follow-up confirmed the aggressive and neoplastic nature of these lesions. The clinical course was usually protracted with multiple recurrences and eventual metastases. All patients died of their tumor, the average survival being 53 mo.; 4 patients survived over 5 yr. The term inflammatory fibrous histiocytoma is proposed to designate these lesions.