36例恶性纤维组织细胞瘤的病理及免疫组化研究

应用ABC法,对36例MFH和27例其他软组织肿瘤进行Lyso、A_1AT、A_1ACT、Des、Vim、Mgo和S_(-100)蛋白免疫组化染色。结果A_1AT和A_1ACT阳性率最高,是肿瘤性组织细胞的良好标志物,对MFH有一定诊断价值。Des和Vim的表达分别提示肿瘤细胞向肌纤维母细胞和纤维母细胞分化。作者同意MFH来源于原始间叶细胞,在不同情况下向组织细胞、肌纤维母细胞和纤维母细胞等不同方向分化,使MFH构成了复杂多样的细胞成分。     

恶性纤维组织细胞瘤的病理学(综述)

1964年O′Brien证实有恶性纤维黄色瘤(MFX;即恶性纤维组织细胞瘤,MFH)存在,其发生率占纤维组织细胞瘤的1%。1978年Weiss等报道200例MFH,认为MFH是成人最常见的软组织肉瘤,以往许多病例曾被误诊为横纹肌肉瘤、脂肪肉瘤、纤维肉瘤等,必须引起重视。一、组织起源 MFH的组织起源尚有争论,存在三种学说: 1.起源于纤维母细胞,某些纤维母细胞可“转化”成组织细胞,构成双向分化,但此学说尚缺乏确切的证据。 2.O′Brien等提出MFH起源于组织细胞,它除具有吞噬作用外,部分细胞尚可转化为“功能性”纤维母细胞。它能产生网织纤维及胶元纤维。     

134例恶性纤维组织细胞瘤——临床病理分析和超微结构观察

恶性纤维组织细胞瘤(malignant fibroushistiocytoma,以下简称MFH)是一种来源于组织中的组织细胞或向纤维母细胞和组织细胞分化的原始间叶细胞,临床上常表现为一无痛性逐渐增大的肿块,组织学上主要由纤维母细胞、组织细胞和巨细胞组成。并杂以     

乳腺恶性纤维组织细胞瘤免疫组化研究（附8例报告）

应用ＡＢＣ法，对８例乳腺恶性纤维组织细胞瘤进行ＡＡＴ、ＡＡＣＴ、Ｖｉｍ、Ｄｅｓ、Ｋｅｒ、ＣＥＡ和ＥＭＡ免疫组化染色。结果ＡＡＴ、ＡＡＣＴ和Ｖｉｍ阳性率１００％，２例Ｄｅｓ阳性。作者同意恶纤组来源于原始间叶细胞，在不同情况下可向组织细胞、纤维母细胞和肌纤维母细胞等不同方向分化，构成了恶纤组复杂多样的细胞成分。     

腹部纤维母细胞/肌纤维母细胞性肿瘤的PET/CT影像学特点及其应用价值

 目的 分析腹部纤维母细胞/肌纤维母细胞性肿瘤的PET/CT影像学表现,提高对该少见病的认识。方法 搜集经手术或穿刺病理证实的腹部肌纤维源性肿瘤28例,分析其PET/CT表现。统计学方法采用独立样本t检验、方差齐性检验。结果 （1）中间型组有侵袭性纤维瘤病13例、孤立性纤维性肿瘤10例、肌纤维母细胞肿瘤3例;恶性组有纤维肉瘤2例。16例位于腹腔内、12例位于腹膜后,平均直径为12.4 cm。（2）PET/CT发现病灶以局部脂肪浸润或脏器浸润为主,所有病例均无淋巴结转移,3例出现远处转移。（3）侵袭性纤维瘤病、孤立性纤维性肿瘤、肌纤维母细胞肿瘤、纤维肉瘤的SUVmax均值分别为4.9、3.6、5.6和17.9,前三组（中间型）之间差异无统计学意义,但明显低于纤维肉瘤（恶性）。结论 PET/CT有助于提示腹部纤维母细胞/肌纤维母细胞性肿瘤的术前TNM分期;SUVmax值有助于肿瘤恶性程度的判断。     

肝纤维母细胞肉瘤1例

低度恶性肌纤维母细胞肉瘤非常罕见,约占软组织恶性肿瘤的1％,发生于肝的纤维母细胞肉瘤报道罕见,现将1例肝纤维母细胞肉瘤报道如下。     

骨原发恶性纤维组织细胞瘤的临床病理分析

目的:采用新的WHO软组织与骨肿瘤分类标准,对过去已诊断为原发于骨的恶性纤维组织细胞瘤/未分化多形性肉瘤(MFH/UPS)进行回顾性评估,并对重新诊断的MFH/UPS的病理特点、组织学来源及分化方向进行初步探讨,以提高本病的病理诊断水平.方法: 收集了16例过去被病理学诊断为原发于骨的MFH病例,用HE染色方法及选用神经特异性、肌特异性、脂肪、纤维、上皮等特异或相对特异性抗体进行免疫组织化学方法,由2位病理医生重新阅片,参照新标准并结合染色结果独立诊断.结果: 16例病例中,重新诊断为向恶性神经鞘瘤分化者6例,向横纹肌和平滑肌肉瘤分化5例,不能明确分化5例,其中炎症性恶性纤维组织细胞瘤1例.结论: 恶性纤维组织细胞瘤/多形性未分化肉瘤是一复杂且有争议的肿瘤家族,应当引起重视的是发生在骨内的MFH,除了排除其它肉瘤的可能,还应考虑向骨肉瘤分化的可能.     

乳腺恶性纤维组织细胞瘤1例报告并文献复习

目的：探讨乳腺原发性恶性纤维组织细胞瘤（MFH）的临床病理学、免疫组化特征和预后。方法：对手术切除一例乳腺MFH进行病理常规切片及免疫组化观察,并结合文献复习。结果：瘤组织由纤维细胞、纤维母细胞、黄色瘤样细胞组成。免疫组化CD68、Vimentin阳性表达,Ki67增殖指数38．38％。结论：MFH根据其分类及亚分类、分级、瘤体大小、部位、肿瘤深度,评估预后。     

乳腺恶性纤维组织细胞瘤1例报告并文献复习

目的:探讨乳腺原发性恶性纤维组织细胞瘤(MFH)的临床病理学、免疫组化特征和预后。方法:对手术切除一例乳腺MFH进行病理常规切片及免疫组化观察,并结合文献复习。结果:瘤组织由纤维细胞、纤维母细胞、黄色瘤样细胞组成。免疫组化CD68、Vimentin阳性表达,Ki67增殖指数38.38%。结论:MFH根据其分类及亚分类、分级、瘤体大小、部位、肿瘤深度,评估预后。     

乳腺恶性纤维组织细胞瘤免疫组化研究（附8例报告）

应用ＡＢＣ法，对８例乳腺恶生纤维组织细胞瘤进行ＡＡＴ、ＡＡＣＴ、Ｖｉｍ、Ｄｅｓ、Ｋｅｒ、ＣＥＡ和ＥＭＡ免疫组化染色。结果ＡＡＴ、ＡＡＣＴ和Ｖｉｍ阳性率１００％，２例Ｄｅｓ阳性。作者同意恶纤组来源于原始间叶细胞，在不同情况下可向组织细胞，、纤维母细胞和肌纤维母细胞等不同方向分化，构成了恶纤组复杂多样的细胞成分。     

A study to analyze the origin of tumor cells in malignant fibrous histiocytomas. A multiparametric characterization

To study the derivation of tumor cells of malignant fibrous histiocytomas (MFH), their phenotypical marker profile was investigated and compared with those of malignant histiocytosis (MH) and of different types of soft tissue tumors (STT). The presence of the following markers was investigated: on paraffin sections, alpha-1-antichymotrypsin (ACT); on frozen sections antigens associated with lymphocytes, macrophages and fibroblasts, the enzymes acid phosphatase, nonspecific esterase, and beta-glucuronidase; and, on isolated and cultured cells, the receptors for EA-gamma and complement. Furthermore, the capacity to phagocytose sensitized erythrocytes and carbon particles was studied in vitro. MFH tumor cells and a part of other types of STT shared the expression of ACT and lysosomal enzymes with MH. They differed, however, from MH by the absence of monocyte/macrophage-associated antigens and by the expression of fibroblast-associated antigens, which property they had in common with other STT. MFH tumor cells were not able to form rosettes or to phagocytose Ig-sensitized erythrocytes, but they showed phagocytosis of carbon particles. The results strongly indicate that MFH tumor cells originate from (primitive) fixed mesenchymal cells and are not related to monocyte-derived histiocytes.     

Malignant fibrous histiocytoma. A tumor of facultative histiocytes showing mesenchymal differentiation in cultured cell lines.

The histogenesis of malignant fibrous histiocytoma (MFH) is controversial. To elucidate the cellular origin and characteristics of this neoplasm, the authors analyzed cell lines grown from 17 patients (15 soft tissue MFH and 2 bone MFH) by using light and electron microscopy, immunocytochemistry, enzyme cytochemistry, and functional tests for receptors for the Fc portion of immunoglobulin (Fc receptors) and immunophagocytosis. Each culture exhibited a storiform/pleomorphic pattern with mixed cellular populations consisting of spindle cells, polygonal cells, and bizarre giant cells; these morphologic features corresponded to the histologic characteristics of the primary tumors. The cells in each MFH line displayed histiocytic functional markers such as lysosomal enzymes, Fc receptors and immunophagocytosis. However, these cells differed from monocyte-derived macrophages (histiocytes) in immunoreactivity; the MFH cells expressed a mesenchymal antigen (FU3) distributed among perivascular cells and fibroblasts but demonstrated no positive reactions with Leu-M1 (CD15) and Leu-M3 (CD14), which recognize the cells of the monocyte-macrophage lineage. In conclusion, these findings suggest that MFH is not a tumor of true histiocytes but of facultative histiocytes showing mesenchymal differentiation in vitro. Chromosomal analysis performed in one MFH line demonstrated abnormal karyotypes; the modal chromosome number was 58, with 5 marker chromosomes.     

Malignant fibrous histiocytoma of the heart

An autopsied case of malignant fibrous histiocytoma (MFH) in the left atrium of the heart of a 29-year-old Japanese woman was reported light and electron microscopically, and immunohistochemically. Metastasis was found in the adrenal, jejunum, and cervical regions. This is the fourth case of MFH of the heart in the literature. The tumor consisted of undifferentiated mesenchymal cells and histiocytoid cells, including giant cells and xanthomatous cells. Dense patches were commonly detected in all tumor cells. On frozen sections, histiocytoid cells formed EA and EAC rosettes, while fibroblastic cells formed EA rosettes only. Difference between them was also recognized in activity or amount of marker enzymes of histiocytes. These analyses suggested that MFH is a mesenchymal cell tumor with binary differentiation into histiocytoid cells and fibroblastic cells.     

Soft tissue sarcoma with additional anaplastic components. A clinicopathologic and immunohistochemical study of 27 cases

This clinicopathologic study concerns 27 cases of "dedifferentiated" soft tissue sarcoma (DSTS), including 14 liposarcomas, six leiomyosarcomas, five chondrosarcomas, and two rhabdomyosarcomas. In addition, the authors conducted an immunohistochemical survey of 23 cases and an electron microscopic examination of three. The findings were compared with observations of 32 cases of de novo malignant fibrous histiocytoma (MFH). All tumors contained additional distinct anaplastic portions indistinguishable from MFH under conventional light microscopy, ultrastructurally, and in cases of immunoreactivity for alpha-1-antichymotrypsin and alpha-1-antitrypsin and on lectin histochemical findings for ricinus communis agglutinin and concanavalin agglutinin. The desmin reactivity present in anaplastic portions of 14 DSTS and in eight de novo MFH is taken to mean that myofibroblasts are present in these tumors. The anaplastic components of DSTS are presumed to represent the proliferation of another clone of undifferentiated mesenchymal cells that fail to differentiate along any specific lineage other than fibroblast-like cells, histiocyte-like cells, and myofibroblasts. Nineteen patients died of tumor and four are alive and well 1.6, 1.7, 2.1, and 5.2 years after the initial treatment, respectively.     

Adipogenic, osteogenic and myofibrogenic differentiations of a rat malignant fibrous histiocytoma (MFH)-derived cell line, and a relationship of MFH cells with embryonal mesenchymal, perivascular and bone marrow stem cells

Malignant fibrous histiocytoma (MFH) is regarded as an undifferentiated pleomorphic sarcoma with unproven histogenesis. We investigated pathobiological characteristics of a rat MFH cell line (MT-9). Immunocytochemically, MT-9 cells and MT-9-induced tumours reacted to vimentin, A3 (rat MFH cell-specific antibody), macrophage markers and α-SMA (myofibroblastic marker), indicating that MT-9 showed both histiocytic and (myo)fibroblastic features. Adipogenic supplement-added MT-9 showed increased accumulation of lipid droplets. Addition of BMP-2 or osteogenic supplement to MT-9 enhanced osteoblastic markers (ALP activity, osteocalcin mRNA expression and calcification). TGF-β1-treated MT-9 revealed increased numbers of α-SMA-immunopositive cells, and enhanced protein levels of α-SMA and fibronectin, indicating myofibrogenesis. In rat tissues, A3 labelled with immature mesenchymal and perivascular cells in foetuses and neonates, and with marrow stem cells in adults. c-kit mRNA expression was seen in bone marrows and MT-9. Collectively, progenitors of MFH should be sought in lineage of marrow stem cells capable of differentiating into mesenchymal cells.     

单克隆抗体P63、SMA、PCNA在乳腺良恶性病变鉴别诊断中的意义

目的探讨单克隆抗体P63、SMA、PCNA在乳腺疑难病变鉴别诊断中的价值。方法收集各种乳腺病变组织标本76例,采用免疫组织化学染色方法,同时标记P63、SMA、PCNA作对照研究。结果11例乳腺良性病变和12例导管原位癌中存留的肌上皮细胞几乎全部P63和SMA（＋）,2例良性病变中的少数导管见P63不均匀的间断表达,P63与间质肌纤维母细胞和血管平滑肌细胞无交叉反应;在浸润性癌中仅2例见小灶状癌细胞P63（＋）。SMA除标记肌上皮细胞,还与间质肌纤维母细胞和血管平滑肌细胞有交叉反应。PCNA在浸润性导管癌阳性率92．7％,在良性病变中阳性率72．7％。结论P63可作为识别乳腺肌上皮细胞的一个新的特异性标志,同时检测P63、SMA有助于提高乳腺疾病的诊断准确率。PCNA表达与否在乳腺良恶性病变鉴别诊断中意义有限。     

An immunocytochemical study on the distribution of ferritin and other markers in 36 cases of malignant histiocytosis

The distribution of ferritin in 36 autopsy cases of malignant histiocytosis was investigated by immunocytochemical staining, together with the detection of alpha 1-antichymotrypsin, alpha 1-antitrypsin, lysozyme, S-100 protein, and ricinus communis agglutinin in the consecutive sections. The results showed that ferritin-positive tumor cells were present in every case. The quantity of cellular ferritin in well-differentiated histiocytes was higher than that in atypical histiocytes. Double labeling showed that ferritin and alpha 1-antichymotrypsin might be located either in one tumor cell or in separate cells. Our data suggest that ferritin may be a tumor associated antigen in malignant histiocytosis, playing a regulatory role for tumor cell differentiation.