Inflammatory fibrosarcoma: update, reappraisal, and perspective on its place in the spectrum of inflammatory myofibroblastic tumors.
Inflammatory fibrosarcoma: update, reappraisal, and perspective on its place in the spectrum of inflammatory myofibroblastic tumors.
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炎性纤维肉瘤：对其在炎性肌纤维母细胞肿瘤谱系中的地位进行更新、重新评估和展望。
DOI：
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发表时间：
1998
影响因子：
2.3
通讯作者：
L. Kindblom
中科院分区：
文献类型：
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作者：
J. Meis;C. Kjellstrom;L. Kindblom
炎性纤维肉瘤（通常被称为炎性肌纤维母细胞肿瘤）越来越被认为是炎性肌纤维母细胞增生的一部分。它是一种潜在的局部侵袭性肌成纤维细胞肿瘤，主要发生在儿童和年轻人的肠系膜。目前还没有确定可靠的形态学参数来预测预后。我们评估了16例炎性纤维肉瘤的超微结构和免疫表型特征，并研究了Ki67 （MIB1）、PCNA、bcl-2和p53，以确定预后标志物。免疫组织化学均未检测到P53。没有发现任何标志物与局部复发、转移或肿瘤死亡相关。所有病例均呈低增殖活性（Ki67 < 10%）。再次证实了一种特征性的免疫表型，病变肌成纤维细胞染色为波形蛋白、α -平滑肌肌动蛋白、细胞角蛋白，很少染色为desmin。7例的超微结构研究证实了成纤维细胞-肌成纤维细胞谱的存在。由于炎性肌成纤维细胞肿瘤-炎性纤维肉瘤与全身症状相关，我们对12例患者进行了eb病毒（EBV）和巨细胞病毒（CMV）聚合酶链反应研究。在9个病例中，可评估的结果没有显示任何一种病毒的证据。本研究结果表明，炎性纤维肉瘤具有低增殖活性，这与低级别肉瘤的印象一致；肌成纤维细胞可参与真正的肿瘤形成；EBV和CMV在炎性纤维肉瘤的发病机制中不起作用。本文讨论了肌成纤维细胞的可变表型及其在反应性和肿瘤性过程中的作用。结合目前的研究和文献，对炎性纤维肉瘤在炎性肌成纤维细胞肿瘤谱系中的位置进行了展望。
Inflammatory fibrosarcoma (commonly referred to as inflammatory myofibroblastic tumor) has become increasingly recognized as part of a spectrum of inflammatory myofibroblastic proliferations. It is a potentially locally aggressive myofibroblastic tumor that occurs predominantly in the mesentery of children and young adults. No reliable morphological parameters have been identified that predict prognosis. We evaluated the ultrastructural and immunophenotypic features of 16 cases of inflammatory fibrosarcoma and studied Ki67 (MIB1), PCNA, bcl-2, and p53 in an effort to identify prognostic markers. p53 was not detected immunohistochemically in any case. None of the markers were found to correlate with local recurrences, metastases, or tumor deaths. Low proliferative activity (Ki67 < 10%) was seen in all cases. A characteristic immunophenotype was reconfirmed in which lesional myofibroblasts stained for vimentin, alpha-smooth muscle actin, cytokeratins, and rarely desmin. Ultrastructural studies of seven cases confirmed the presence of a fibroblastic-myofibroblastic spectrum. Because inflammatory myofibroblastic tumor-inflammatory fibrosarcoma is associated with systemic symptoms, polymerase chain reaction studies for Epstein-Barr virus (EBV) and cytomegalovirus (CMV) were performed in 12 cases. Evaluable results in nine cases did not show evidence of either virus. The results of this study indicate that inflammatory fibrosarcoma has a low proliferative activity, which is in keeping with the impression that this is a low-grade sarcoma; that myofibroblasts can participate in true neoplasia; and that EBV and CMV do not play a role in the pathogenesis of inflammatory fibrosarcoma. The variable phenotype of the myofibroblast and its role in reactive and neoplastic processes are discussed. A perspective on the position of inflammatory fibrosarcoma in the spectrum of inflammatory myofibroblastic tumors is also given in light of the current study and the literature.