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A wide variety of sarcomas occur in the uterus but two subtypes - leiomyosarcoma and endometrial stromal sarcoma - account for a majority of those more routinely encountered. Using the 2003 World Health Organization classification, this review focuses on six uterine sarcomas: endometrial stromal sarcoma, undifferentiated endometrial sarcoma, leiomyosarcoma, rhabomyosarcoma, angiosarcoma and liposarcoma. The epidemiological, clinical, pathological and molecular features are presented along with therapeutic approaches. Familiarity with molecular aspects of these tumors and application of novel technologies in their assessment should be encouraged as they may provide alternate therapies resulting in improved survival for the patient. Clinical information necessary for accurate diagnosis of these lesions is emphasised. A multidisciplinary approach to management of patients with uterine sarcomas is essential for optimal management. 相似文献
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The authors report on 79 cases of primary brain sarcomas, correlating clinical and pathoanatomical data. In the material analyzed the group of gliosarcomas prevailed, while the smallest group were rhabdomyosarcomas. In some groups the topic predilection did not show any specific characteristics, except that there were more gliosarcomas occurring in the parietal lobe. The symptoms lasted 1-3 months before operation. 相似文献
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Fisher C 《Ultrastructural pathology》2004,28(5-6):291-305
A subset of low-grade fibrosarcomas is composed of CD34-positive spindle cells. These include dermatofibrosarcoma, its morphologic variants, and its associated fibrosarcoma, solitary fibrous tumor, hemangiopericytoma and their malignant counterparts, and some cases of myxoinflammatory fibroblastic sarcoma. Dermatofibrosarcoma and related lesions are characterized by a t(17;22)(q22;q13) rearrangement resulting in fusion of the genes COL1A (17q21-22) and PDGFB1 (22q13). Solitary fibrous tumor displays varying cellularity and fibrosis and a peripheral hemangiopericytomatous pattern; most tumors formerly called hemangiopericytoma are now subsumed into the category of solitary fibrous tumor, although a few strictly defined examples are recognized; however, these are probably not composed of pericytes. Myofibroblastic malignancies are best identified by electron microscopy, with which varying degrees of differentiation, including the presence of fibronexus junctions, can be identified. Low-grade sarcomas showing myofibroblastic differentiation include myofibrosarcomas and inflammatory myofibroblastic tumors. Myofibrosarcomas are spindle cell neoplasms that occur in children or adults in the head and neck, trunk, and extremities as infiltrative neoplasms and that display a fascicular or fasciitis-like pattern with focal nuclear atypia and variable expression of myoid antigens. These sarcomas are prone to recurrence and a small number metastasize. Inflammatory myofibroblastic tumor (synonymous with inflammatory fibrosarcoma) is a neoplasm arising predominantly in childhood, and frequently in intraabdominal locations. It has spindle cells in fascicular, fasciitis-like and sclerosing patterns, with heavy chronic inflammation including abundant plasma cells. Many IMT have clonal chromosomal abnormalities involving 2p22-24, and fusion of the ALK gene with tropomyosin 3 (TPM3-ALK) or tropomyosin 4 (TPM4-ALK) is found in a subset. 相似文献
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《Diagnostic Histopathology》2017,23(10):431-441
Connective tissue tumours, particularly sarcomas, are rare and present as a variety of histological subtypes with diverse management protocols and have varied prognoses. Many of these tumours have specific cancer driver genetic alterations that can be leveraged for diagnostic purposes. For practical purposes, these tumours can be categorised as harbouring genetic alterations including chromosomal rearrangements, gene amplifications, single nucleotide substitutions, and complex genetic abnormalities. Herein we discuss different tumour subtypes with their associated genetic abnormalities that may be used in clinical practice, when interpreted in the context of the relevant clinical, histological and radiological information.The benefits of large scale sequencing studies of sarcoma are leading to new insights into sarcoma development and are providing a biological rationale for personalised medicine. Genomic profiling and other “omic” studies will likely play a fundamental part in the development of new diagnostic and predictive biomarkers in the near future. 相似文献
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Th. Löning J. Liebsch G. Delling 《Virchows Archiv : an international journal of pathology》1985,407(3):323-336
Summary Primary malignant bone tumors, osteosarcomas (9 cases), and Ewing's sarcomas (10 cases) were examined for their reactivities with monoclonal and polyclonal antibodies against filamentous proteins and cell membrane determinants of the lymphoid and macrophage marker series. The reactivity of antibodies was studied on snap-frozen tissue probes by using a triple layer immunoperoxidase method.
Osteosarcomas were positive for vimentin and, in part, for HLA-DR. Other types of intermediate-sized filaments were not detected in tumour cells. In a small number of cases (2/9) tumour cells were reactive with antibodies of the macrophage series (Leu M2).In Ewing's sarcomas, vimentin and HLA-DR was also demonstrated. It was particularly interesting that Leu M2 staining was found in the majority of cases (8/10). The staining pattern supports the assumption that this peculiar tumour is of mesenchymal (monocyte/macrophage) histogenesis.It was evident from the present study that, in primary osteogenic tumors, none of the examined tumour markers were as distinctive as they are for bone metastases. Nevertheless, the reactivity of Ewing's sarcoma cells with monoclonal antibodies of the Leu M2 type throws some highlights on the, as yet, obscure histogenesis of the neoplasm and may be of diagnostic value in conjunction with the known light and electron microscope features of the tumour.The study was supported by a grant of the Deutsche Forschungsgemeinschaft (Lo 285/2-2) 相似文献
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Nielsen TO 《Advances in anatomic pathology》2006,13(4):166-173
Microarrays began to be used to study gene expression profiles in the mid-1990s, but it was only after 2000 that serious attempts have been made to apply this technology to investigate sarcomas. Microarray technologies provide a comprehensive survey of active molecular pathways and potential molecular targets for diagnosis and treatment, but are challenging to use because of issues of specimen collection, cost, and complexities in experimental design and data analysis. As a discovery-based technique, microarray analyses are most valuable when framed around specific gaps in our knowledge of tumor etiology and progression, challenges in differential diagnosis, and pressing therapeutic needs. To date, microarray analyses of sarcomas support their division into molecularly defined and molecularly heterogeneous categories, and have provided useful diagnostic markers for entities such as gastrointestinal stromal tumors, synovial sarcoma, and dermatofibrosarcoma protuberans. Signatures predicting outcome and response to therapy have been published for Ewing sarcoma and osteosarcoma, and receptor tyrosine kinase expression patterns have suggested novel therapeutic approaches which may be applied to several types of sarcoma. Nevertheless, results need to be interpreted in the context of histopathology and validated by complementary technologies and/or other research groups. 相似文献
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Diagnosis of soft tissue sarcomas 总被引:1,自引:0,他引:1
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Myofibroblasts in soft tissue sarcomas 总被引:7,自引:0,他引:7
Réal Lagacé Walter Schürch Thomas A. Seemayer 《Virchows Archiv : an international journal of pathology》1980,389(1):1-11
Summary A series of 129 soft tissue sarcomas was examined ultrastructurally to determine in which neoplasms and to what extent myofibroblasts could be demonstrated. Twenty cases of fibromatosis and fasciitis served as controls.Myofibroblasts were identified in all 30 cases of malignant fibrous histiocytoma and all 4 cases of well-differentiated sclerosing liposarcoma. Though most numerous in areas of desmoplasia, in no instance did myofibroblasts constitute the dominant cellular constituent of either neoplasm. Myofibroblasts were identified with lesser frequency and in smaller numbers in fibrosarcoma, synovial sarcoma, malignant hemangiopericytoma and neuroblastoma. None were observed in a wide assortment of diverse sarcomas in which desmoplasia was not a feature. In comparison each lesion judged by light microscopy to represent either fibromatosis or fasciitis was composed principally of myofibroblasts.The demonstration of abundant myofibroblasts within a soft tissue lesion which has been subjected to wide sampling strongly suggests a benign proliferative process as opposed to a malignant neoplasm. It is hypothesized that myofibroblasts observed within collagenized regions of soft tissue sarcomas may constitute an expression of host response to neoplasia. 相似文献
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Sarcomas included in the broad group of small round-cell tumors (SRCT) and some non-SRCT lesions that typically are seen in pediatric-age patients can rarely occur in adults. However, there are differences in the anatomic sites that are involved and the prognosis in these two patient groups. The diagnosis of pediatric-type sarcomas in adults is often challenging because of the unusual contextual clinical setting and morphologic features. Immunohistochemical studies have greatly facilitated this process. Moreover, limited biomolecular studies that have been conducted have demonstrated comparable cytogenetic alterations in adults and children with pediatric-type tumors. They also have raised interesting questions concerning possible biological bases for differences in clinical behavior in the two cohorts. This review focuses on the morphological, immunohistochemical, and molecular characteristics of childhood-type sarcomas that affect adults, with emphasis on possible pitfalls in differential diagnosis. 相似文献
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Reciprocal translocation in small-cell sarcomas 总被引:2,自引:0,他引:2
J P de Chadarevian M Vekemans T A Seemayer 《The New England journal of medicine》1984,311(26):1702-1703
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MicroRNAs are a class of small regulatory RNAs that influence the stabilities and translational efficiencies of target mRNAs. They have been implicated in an increasing number of biological processes, including carcinogenesis. A huge body of literature exists documenting up- or down-regulation of specific microRNAs during carcinogenesis and identifying molecular pathways by which these microRNAs influence every aspect of cancer development, including proliferation, angiogenesis, and metastasis. These studies have provided many insights into basic cancer biology as well as allowing identification of novel biomarkers and potential drug targets. However, the vast bulk of this literature concerns solid epithelial tumours, while sarcomas remain relatively under-studied. The purpose of this article is to review the roles of microRNAs in sarcomas and to highlight microRNAs or related molecular pathways that demonstrate consistent roles within individual or across sarcoma subtypes, with a view to identifying the key regulatory molecules. Further insights into sarcoma biology may be particularly valuable since sarcomas represent a tumour group with a particularly poor prognosis and rather limited treatment options. 相似文献