丛状纤维组织细胞瘤3例临床病理观察

目的 探讨丛状纤维组织细胞瘤临床病理特点及鉴别诊断要点.方法 对3例丛状纤维组织细胞瘤进行临床资料及光镜和免疫组化标记观察.结果 组织学特点:纤维结缔组织把肿瘤细胞分隔成丛状或结节状.结节则由单核或多核组织细胞样细胞构成,结节外周围绕短梭形的纤维母/肌纤维母细胞样细胞.部分结节则主要由纤维母细胞样细胞组成,不见多核巨细胞.免疫组化染色显示:单核或多核组织细胞样细胞表达CD68、α-ACT和溶菌酶,梭形细胞表达Vim和SMA.结论 丛状纤维组织细胞瘤是一种低度恶性的软组织肿瘤,其诊断主要依靠组织病理学和免疫组化标记.     

腮腺滤泡性树突状细胞肉瘤1例及文献复习

目的探讨滤泡性树突状细胞肉瘤的病理诊断及鉴别诊断。方法对1例腮腺滤泡性树突状细胞肉瘤进行HE切片、免疫组化等观察,并复习文献进行讨论。结果镜检肿瘤细胞呈梭形、卵圆形、多边形,胞质淡染,边界不清,核染色质颗粒状并见明显小核仁,可见散在核分裂象,呈片状、束状、编织状、旋涡状排列。较多淋巴细胞浸润及围绕血管形成袖套状结构。肿瘤细胞特异性地表达CD21、CD23、CD35、S-100蛋白、CD68、vim entin、EMA。结论滤泡性树突状细胞肉瘤是极少见的低中度恶性肿瘤,可出现多种组织结构,有一定的特征性,确诊需要免疫组化帮助。应与异位脑膜瘤、异位胸腺瘤、恶性纤维组织细胞瘤、淋巴上皮样癌及指突性树突状细胞肿瘤等相鉴别。     

软组织肿瘤WHO分类中新类型(二)

1994年WHO分类中的纤维组织细胞肿瘤在最新分类中也作了一些变动。在过去10多年中，人们对纤维组织细胞分化的概念提出了质疑，现在认为形态学上组织细胞分化难以得到进一步的确定，故更名为所谓纤维组织细胞肿瘤。多形性恶性纤维组织细胞瘤(MFH)一直被认为是一种最常见类型的成人软组织肉瘤，现已将它看成是未分化多形性肉     

栅状肌纤维母细胞瘤3例报道及文献复习

目的：观察栅状肌纤维母细胞瘤的临床病理特征，探索该肿瘤的组织来源及石棉样纤维的性质和来源。方法：对３ 例栅状肌纤维母细胞瘤进行组织形态学和免疫组织化学研究，结合文献对本病的诊断标准、鉴别要点及组织来源进行探讨。结果：栅状肌纤维母细胞瘤的组织学特点为梭形肿瘤细胞呈交叉束状、栅栏状排列，间质内有较多出血、散在的肥大细胞，肿瘤中出现石棉样纤维。免疫组化见肿瘤细胞及石棉样纤维星芒状突起中ａｃｔｉｎ、ｖｉｍｅｎｔｉｎ 呈阳性表达，ｄｅｓｍｉｎ、Ｓ１００ 、ＦａｃｔｏｒⅧ、ｃｙｔｏｋｅｒａｔｉｎ 呈阴性表达。结论：栅状肌纤维母细胞瘤是一种具有独特临床病理特征的肌纤维母细胞瘤，该肿瘤来源于肌纤维母细胞或特殊平滑肌细胞亚型。石棉样纤维中心为血管周围胶原的变性，星芒状纤维为增殖的肌纤维母细胞突出的中间丝和微丝。     

尿路膀胱软组织肿瘤（Ⅱ）：恶性肿瘤

大多数原发膀胱癌为移形细胞（尿路上皮）癌，鳞状细胞癌、原发腺癌或小细胞癌均较少碰到，其它膀胱癌均非常少见。在上一期杂志中，作者提出了“尿路膀胱的软组织肿瘤第1部分：肌纤维母细胞增生、良性肿瘤和具有不确定恶性潜能的肿瘤”，此两部分综述的第Ⅱ部分描述了不同类型的膀胱恶性间质病变，包括：平滑肌肉瘤、横纹肌肉瘤、血管肉瘤、恶性纤维组织细胞瘤（未分化肉瘤）、原始神经外胚叶肿瘤、恶性外周神经鞘瘤、血管外皮细胞瘤和腺泡状软组织肉瘤，并描述了这些病变的临床表现、形态学特点和免疫组化特征。     

TFE3、CD147免疫组化和ASP-SCR1-TFE3融合基因RT-PCR检测在腺泡状软组织肉瘤与其他肿瘤鉴别诊断中的价值及比较

腺泡状软组织肉瘤(ASPS)是一种罕见的肿瘤,大多发生在青年人,以四肢软组织好发。ASPS组织学特征是肿瘤细胞被纤维血管分隔成巢状或器官样结构。当ASPS发生于少见部位时诊断较为困难,需与一些常见的肿瘤,如颗粒细胞瘤、副神经节瘤、透明细胞肉瘤,转移性肾透明细胞癌     

具有上皮—间叶性分化恶性肿瘤的病理诊断

在病理诊断中具有双向分化的恶性肿瘤较为常见 ,一般指同一种肿瘤组织中 ,可见二个胚层或二种组织成分 ,或同属一个胚层或一种组织成分伴不同方向、不同程度分化。双向分化肿瘤可以分为上皮 间叶、上皮 上皮、间叶 间叶、神经 非神经等组织分化〔1〕。上皮 间叶组织分化的恶性肿瘤形态学上主要表现为肉瘤样癌或癌肉瘤 ,上皮性癌成分可以为鳞癌、腺癌、移行细胞癌、未分化癌 ,间叶性肉瘤成分常为纤维肉瘤、恶性纤维组织细胞瘤、横纹肌肉瘤 ,还可为骨肉瘤、软骨肉瘤等 ,两种成分可以完全分开 ,也可以是互相移行无分界的混杂结构。这类肿瘤…     

胶质肉瘤的临床病理研究

为探讨胶质肉瘤的临床病理特征、组织发生学及预后，观察了12例胶质肉瘤，其肿瘤均发生于大脑半球，颞叶多见。肿瘤半数界限清楚，质硬。随访10例，9例死亡，平均生存8个月，1例生存3年2个月。部分病例HE染色切片中难以确定梭形肿瘤细胞来源。通过电镜及胶质纤维酸性蛋白、Mac387、波形蛋白、第Ⅷ因子相关抗原免疫组织化学染色观察，证实肿瘤中均含胶质母细胞瘤和恶性纤维组织细胞瘤成分，仅1例尚含骨肉瘤成分。其组织起源为原始多潜能间叶细胞。     

软组织肌纤维母细胞肉瘤的组织学特征及鉴别诊断

软组织肌纤维母细胞肉瘤是一种独立的新型肿瘤，较罕见。光镜下主要由梭形肿瘤细胞组成。组织化学及免疫组化染色具有辅助诊断价值。电镜下，瘤细胞胞质中含有丰富的粗面内质网、大量肌丝束及多少不等的密体和密斑，这是诊断的重要依据。鉴别诊断主要包括平滑肌肉瘤和纤维肉瘤     

低度恶性肌纤维母细胞性肉瘤

目的 探讨低度恶性肌纤维母细胞性肉瘤的临床病理学特征、免疫学表型和超做结构特点。方法 对1例发生于左上颌窦的低度恶性肌纤维母细胞性肉瘤进行临床资料复习、光镜观察、免疫组化标记和电镜检测。结果 患者因“左上颌窦囊肿”2次行切除活检，病理诊断分别为“纤维组织增生”和“鳞状细胞癌Ⅰ级”。4个月后因左上颌骨隐痛行左上颌骨部分切除术，术后病理检查未发现肿瘤组织。5个月后左上后磨牙区肿块复发，再行左上颌、颊、颈联合根治术，诊断为“侵袭性纤维瘤病”。9个月后左侧颧部出现包块，术后病理为“左上颌纤维肉瘤，部分伴平滑肌分化”复片显示，第1次活检标本中的“纤维组织增生”实际上是梭形细胞肿瘤组织，而第2次活检中的“鳞状细胞癌1级”实为鳞状上皮假上皮瘤样增生。第3次术后标本中肿瘤组织不明显，而第4次术后标本则由成束的梭形细胞组成，弥漫浸润至邻近的软组织内，类似侵袭性纤维瘤病，但部分区域内可见鱼骨样排列结构，类似低度恶性纤维肉瘤。第5次术后标本中瘤细胞显示轻～中度的异型性，可见核分裂象(2个／10HPF)，并弥漫浸润横纹肌组织，在部分区域内，瘤细胞穿插在肌束之间形成类似增生性肌炎中的棋盘样结构，另一些区域则在形态上类似经典的纤维肉瘤。免疫表型：瘤细胞表达Vim、α—SMA和Ki—67，不表达MSA、Des、h—caldesmon和S—100蛋白。电镜下瘤细胞胞质内可见到平行肌丝。结论 低度恶性肌纤维母细胞性肉瘤是一种少见的软组织肉瘤，组织学形态、免疫表型及超微结构均显示瘤细胞具肌纤维母细胞性分化。     

Characterization of human soft tissue sarcomas in nude mice. Evidence for histogenic properties of malignant fibrous histiocytomas.

Twenty-two human sarcomas were grafted subcutaneously into nude mice. Twelve tumors grew successfully. Nine of these 12 tumors had an aneuploid DNA content, whereas only 1 of 10 nonsuccessful tumors was aneuploid. The 12 sarcomas included two leiomyosarcomas, two malignant schwannomas, one synovial sarcoma, and seven malignant fibrous histiocytomas (MFHs). With light and electron microscopic and immunolabeling studies the original and xenografted tumors (the latter for at least two generations) were histopathologically compared. The xenografted leiomyosarcomas showed ultrastructurally a more pronounced leiomyodifferentiation, and one of the malignant schwannomas a more pronounced schwannian differentiation. The second malignant schwannoma and the synovial sarcoma, however, remained unchanged. Five storiform pleomorphic MFHs expressed features that were not observed in the original tumors. Tumor cells of three of these xenografted sarcomas showed leiomyogenic differentiation (filamentous densities, pinocytotic vescicles, and desmin immunoreactivity), whereas cells of the two others demonstrated schwannian differentiation (long cytoplasmic processes, basal lamina). A xenografted myxoid MFH and a pleomorphic MFH gave rise to pleomorphic sarcomas composed of undifferentiated cells. It appeared that under transplantation conditions tumor cells of storiform pleomorphic MFH can differentiate into various directions.     

Gene expression analysis of soft tissue sarcomas: characterization and reclassification of malignant fibrous histiocytoma.

In soft tissue sarcomas, the diagnosis of malignant fibrous histiocytoma (MFH) has been a very controversial issue, and MFH is now considered to be reclassified into pleomorphic subtypes of other sarcomas. To characterize MFH genetically, we used an oligonucleotide microarray to analyze gene expression in 105 samples from 10 types of soft tissue tumors. Spindle cell and pleomorphic sarcomas, such as dedifferentiated liposarcoma, myxofibrosarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor (MPNST), fibrosarcoma and MFH, showed similar gene expression patterns compared to other tumors. Samples from those five sarcoma types could be classified into respective clusters based on gene expression by excluding MFH samples. We calculated distances between MFH samples and other five sarcoma types (dedifferentiated liposarcoma, myxofibrosarcoma, leiomyosarcoma, MPNST and fibrosarcoma) based on differentially expressed genes and evaluated similarities. Three of the 21 MFH samples showed marked similarities to one of the five sarcoma types, which were supported by histological findings. Although most of the remaining 18 MFH samples showed little or no histological resemblance to one of the five sarcoma types, 12 of them showed moderate similarities in terms of gene expression. These results explain the heterogeneity of MFH and show that the majority of MFHs could be reclassified into pleomorphic subtypes of other sarcomas. Taken together, gene expression profiling could be a useful tool to unveil the difference in the underlying molecular backgrounds, which leads to a rational taxonomy and diagnosis of a diverse group of soft tissue sarcomas.     

Primary malignant giant cell tumor of bone: "dedifferentiated" giant cell tumor

Well documented examples of primary malignant giant cell tumor of bone (giant cell tumor and concurrent sarcoma arising de novo) are exceedingly rare in the literature. We report a case arising in the left ischium of a 44-yr-old man. He had no previous history of radiation therapy or multiple resections. Histologically, the tumor was a typical giant cell tumor of bone juxtaposed to a malignant fibrous histiocytoma (MFH). The juxtaposition of a high grade sarcoma (MFH) and a locally aggressive nonmalignant neoplasm such as giant cell tumor is analogous to several other tumors of bone and soft tissue in which a low grade malignant or locally aggressive tumor can be associated with MFH or fibrosarcoma de novo, namely chondrosarcoma, chordoma, liposarcoma, and well differentiated intraosseous and parosteal osteosarcoma. The presence of a high grade malignant component in each of the aforementioned neoplasms generally portends a more ominous prognosis, although this is not invariably true. Recognition of the phenomenon of "dedifferentiation" (or tumor progression) in some bone tumors and sarcomas is important to ensure appropriate treatment. Distinction from secondary malignant giant cell tumors which are usually radiation induced is also important, since the latter have a much worse prognosis than those with dedifferentiation occurring de novo.     

Does malignant fibrous histiocytoma exist?

Malignant fibrous histiocytoma (MFH) has come to be regarded as the most common malignant neoplasm of the mesenchymal soft tissues. It designates a spectrum of tumors which share morphologic features that allow their inclusion in a distinct clinicopathologic setting, although being not uniform in their histogenesis and pathogenesis. Clinicopathologic variants include the following: the storiform-pleomorphic form of MFH, the myxoid type of MFH, the giant cell type of MFH and the inflammatory type. The latter group, the angiomatoid variant, has been reclassified within the fibrohistiocytic tumors of low malignant potential. Tissue culture, ultrastructural and immunohistochemical studies have both endorsed or refuted the validity of the concept. As a whole, these morphologic studies which attempted to characterize MFH were not able to delineate specific markers or to describe the phenotype of this sarcoma of supposed fibrohistiocytic lineage. There is growing evidence that MFH is a second component in another sarcoma and represents a morphologic modulation resulting from tumor progression. Recent cytogenetic and molecular genetic investigations are consistent with that hypothesis: a comparative analysis between the most frequent genomic imbalances observed in series of MFH and leiomyosarcomas (LMS) demonstrated that both tumors had similar recurrent imbalances. Immunohistochemical and molecular biologic investigations have shown similar targets of chromosome deletions in both tumors. A new classification of soft tissue sarcoma based on molecular parameters is nevertheless premature. The morphologic characterization of MFH and its sub-types provides the clinician with unique information in the management of these tumors, by identifying a spectrum of tumors with well-recognized clinical profiles.     

HLA-DR (Ia-like) reactivity in tumors of bone and soft tissue: an immunohistochemical comparison of monoclonal antibodies LN3 and LK8D3 in routinely processed specimens.

Recent studies of Class II histocompatibility antigen expression in bone and soft tissue sarcomas have suggested that malignant fibrous histiocytoma (MFH) may express HLA-DR, whereas histologically similar pleomorphic, epithelioid, and spindle cell malignant neoplasms generally do not. To test whether these observations are reproducible in the differential diagnosis of soft tissue sarcomas, anti-HLA-DR antibodies LK8D3 and LN3 were applied to formalin-fixed, paraffin-embedded sections of MFH, neurofibrosarcoma (NFS), leiomyosarcoma (LMS), synovial sarcoma (SS), fibrosarcoma (FS), angiosarcoma (AS), Kaposi's sarcoma (KS), chondrosarcoma (ChS), "dedifferentiated" chondrosarcoma (DChS), osteosarcoma (OS), epithelioid sarcoma (ES), and clear cell sarcoma (CCS; malignant melanoma of soft parts). The only consistent difference in Class II antigen expression was seen in the group of neoplasms composed of large polygonal cells. Among the latter lesions, four of six clear cell sarcomas were labeled by LK8D3 or LN3, but none of 12 epithelioid sarcomas were reactive. Otherwise, a diversity of tumors in other morphologic categories expressed Class II antigens, with no clear diagnostic patterns. These results may be of use in the diagnostic separation of large cell epithelioid tumors of soft tissue, but neither LN3 nor LK8D3 appears to be helpful in the identification of other sarcomas.     

Imprint cytological features of soft tissue sarcomas]

The imprint cytologic features in typical sarcomas, such as malignant fibrous histiocytoma (MFH), fibrosarcoma, malignant peripheral nerve sheath tumor (MPST), liposarcoma, synovial sarcoma, clear cell sarcoma and epithelioid sarcoma, were presented in comparison with each histologic feature. Cytological diagnosis of soft tissue sarcomas is usually difficult because of their rarity, various kinds, wide range of features in the tumor and similar features to those of other tumors. Based on the cellular morphology, sarcoma cells were divided into five cell types as follows: small cell type, spindle cell type, epithelioid cell type, epithelioid-spindle type, and pleomorphic type. The results obtained from "retrospective" immunostainings for decolorized Papanicolau's stained sections with a panel of markers, together with differentiation of the cell type, were useful for the cytological diagnosis of the tumors examined. Since the value of aspiration cytology is much higher than that of imprint cytology in the cytopathologic diagnosis, methods, such as immunostaining and differentiation of the cell type, are recommended in aspiration cytology to make a definitive cytological diagnosis in sarcoma cases.     

Das maligne fibröse Histiozytom

The entity and nosology of pleomorphic malignant fibrous histiocytoma (MFH) is still ambiguous. The actual WHO-Classification uses pleomorphic malignant fibrous histiocytoma (MFH) and pleomorphic sarcoma NOS (not otherwise specified) synonymously. On the other hand text and illustrations convey the impression, that these tumors also could be pleomorphic lipo-, leio- or rhabdomyosarcomas etc. It would have been more informative to emphasize, that with the above mentioned specific sarcoma types MFH-like appearance may occur. Furthermore it would have been more up to date to consider pleomorphic sarcomas NOS as pleomorphic fibrosarcomas and include them in the chapter of fibroblastic and myofibroblastic tumors. This concept already has been carried out for the former myxoid variant of MFH, nowadays preferentially called myxofibrosarcoma.There is controversial discussion about the clinical significance of exact typing of pleomorphic sarcomas. Problems may also occur due to the lack of standards, which degree of desmin expression signifies leiomyosarcoma or just indicates myofibroblasts in MFH. The requirement of exclusion of other tumor-types before diagnosing pleomorphic fibrosarcoma still remains obligatory. After verification of the diagnosis pleomorphic sarcoma NOS or pleomorphic fibrosarcoma, grading e.g. according to criteria of the FFCCS can be carried out. Most cases of pleomorphic fibrosarcoma will qualify as high grade malignant.     

Malignant fibrous histiocytoma with special reference to its differential diagnosis

The histological diagnosis of malignant fibrous histiocytoma (MFH) seems to have become recently fashionable among pathologists, although its histogenesis and diagnostic criteria are not entirely settled as yet. For practical purposes the differential diagnosis with other easily mistakable mesenchymal tumors should be strictly made with great caution, because of variable histological features of this tumor. The authors attempted to elucidate the differential points from other tumors in a review of 189 cases of malignant soft tissue tumors. Some cases of carcinoma mimicking MFH were also reviewed. No single criterion for making the histological diagnosis of MFH was obtained. Its histological features and differential points from pleomorphic rhabdomyosarcoma and fibrosarcoma were tabulated. The recognition of a true tumor osteoid was emphasized as a single differential point between osteosarcoma and MFH often with fibrous areas mimicking osteoid. Renal cell carcinoma metastasizing to bone, which was misinterpreted as MFH on biopsy tissue, was also described and its differential point was stated.     

Coexpression of HGF and c-Met/HGF receptor in human bone and soft tissue tumors

To understand the interaction between hepatocyte growth factor (HGF) and its receptor c-Met on various bone and soft tissue tumors, their expressions were investigated by western blot analysts, immunohistochemistry and enzyme immunoassay. Western blot analysis revealed that c-Met protein was expressed in 21 (38.8%) of 54 tumors, which detailed to seven (25.9%) of 27 bone tumors and 14 (51.8%) of 27 soft tissue tumors. Most malignant fibrous histiocy-tomas (MFH) and all neurofibromas expressed c-Met protein. The highest expression of c-Met protein was seen in a case of biphasic synovial sarcoma, where its immunoreac-tivity was localized only on the epithelial component and not on the sarcomatous component. By enzyme immunoassay for HGF, all but one MFH showed HGF production and the mean level of HGF was the highest among the tumors investigated. Neurofibmmas and osteosarcomas had the next highest mean levels of HGF production, respectively. Coexpression of HGF and c-Met was obsewed in 19 (35.2%) of 54 tumors and was frequently observed in neurofibroma, followed by MFH and synovial sarcoma. Although the mode of interaction between HGF and c-Met varies among the various bone and soft tissue tumors including MFH, their signaling system may play an Important role in the development and progression of bone and soft tissue tumors.     

Primary fibrosarcoma and malignant fibrous histiocytoma of bone--a comparative ultrastructural study: evidence of a spectrum of fibroblastic differentiation

As primary bone fibrosarcoma (FS) and malignant fibrous histiocytoma (MFH) have similar clinical, radiographic, or survival manifestations, ultrastructural and immunohistochemical studies were undertaken to determine the differentiation pathways of constituent malignant cells. Twelve cases of primary intraosseous FS and MFH were selected for this ultrastructural comparative study and were analyzed for fibroblastic or modified fibroblastic differentiation. There were 4 FS cases and 8 MFH cases, of which 5 were storiform-pleomorphic, 2 were giant cell, and 1 was myxoid type. All FS consisted of spindle fibroblasts with a prominent rough endoplasmic reticulum and Golgi apparatus, variable amounts of vimentin intermediate filaments, and extracellular collagen fibrils. The MFH were composed of a mixture of spindle and pleomorphic fibroblasts (8/8), histiofibroblasts (4/8), and myofibroblasts (3/8). Variable numbers of undifferentiated cells were found in both tumors. In conclusion, fibroblastic differentiation and collagen production was noted in all cases. The often pleomorphic histiofibroblasts present in some MFH cases most likely represent "modified fibroblasts," similar to myofibroblasts. These findings support the hypothesis that the fibroblast and its variants are the predominant cell types found in these tumors, suggesting that the diagnostic entity MFH should be classified as a pleomorphic fibrosarcoma.