Epithelioid sarcoma: The spectrum of ultrastructural differentiation in seven immunohistochemically defined cases

Fisher, Cyril

doi:10.1016/s0046-8177(88)80519-7

Cited by 85 publications

(39 citation statements)

References 52 publications

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“…29 and epithelial membrane antigen. 30 They also possess ultrastructural features such as specialized junctions, tonofilaments and microvilli indicative of epithelial differentiation both in vivo 31 and in tissue culture. 32 In the current study, we are able to report that immunohistochemical expression of dysadherin is significantly useful for the differential diagnosis between epithelioid sarcoma and malignant rhabdoid tumor, and for the prediction of prognosis in epithelioid sarcoma.…”

Section: Discussionmentioning

confidence: 99%

Prognostic significance of dysadherin expression in epithelioid sarcoma and its diagnostic utility in distinguishing epithelioid sarcoma from malignant rhabdoid tumor

et al. 2006

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Dysadherin is a cancer-associated cell membrane glycoprotein, which downregulates E-cadherin and promotes metastasis. We studied the clinicopathological features in 72 cases of epithelioid sarcoma and in six cases of malignant rhabdoid tumor, and also assessed the immunohistochemical expression of dysadherin, E-cadherin and MIB-1 in epithelioid sarcoma and malignant rhabdoid tumor cases. In addition, we compared dysadherin mRNA expression between epithelioid sarcoma and malignant rhabdoid tumor cell lines, using RT-PCR and realtime quantitative RT-PCR analysis. Immunohistochemical dysadherin expression was more frequently observed in proximal-type epithelioid sarcoma (71%) in comparison with distal-type epithelioid sarcoma (36%) (P ¼ 0.037). Furthermore, seven proximal-type epithelioid sarcoma cases mimicking malignant rhabdoid tumor (histologically classified as the large cell type, accompanied by frequent rhabdoid cells and located in deep soft tissue) were all positive for dysadherin (100%), whereas dysadherin expression was not detected at all in any of the true six malignant rhabdoid tumors (0%). Cell lines established from proximal-type epithelioid sarcoma revealed significantly higher levels of dysadherin mRNA expression, compared with the levels seen in malignant rhabdoid tumor cell lines by real-time quantitative RT-PCR (P ¼ 0.0433). Epithelioid sarcoma patients with dysadherin expression survived for a significantly shorter time than those without dysadherin expression (P ¼ 0.001). In multivariate analysis, dysadherin immunopositivity (P ¼ 0.0004) was one of the two independent adverse prognostic factors. We conclude that dysadherin expression in epithelioid sarcoma is a significant poor prognostic factor and that it is a powerful diagnostic marker for distinguishing epithelioid sarcoma, including the proximal-type epithelioid sarcoma, from malignant rhabdoid tumor. In epithelioid sarcoma, especially in proximal-type epithelioid sarcoma, increased cell disadhesion and motility by dysadherin plays an important role to acquire aggressive biological behavior. However, in malignant rhabdoid tumor, cell growth cycle that is regulated by hSNF5/INI1 gene seems to be critical to lethal biological behavior rather than dysadherin.

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Section: Discussionmentioning

confidence: 99%

Prognostic significance of dysadherin expression in epithelioid sarcoma and its diagnostic utility in distinguishing epithelioid sarcoma from malignant rhabdoid tumor

et al. 2006

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“…In addition, myofibroblastic differentiation has been observed in tumours showing predominantly epithelial differentiation, notably epithelioid sarcoma [5,29,33] and sarcomatoid carcinomas [1]. In both, there appears to be progression or transition from cells with epithelioid features to those of myofibroblasts, with immunophenotypical and ultrastructural changes accompanying the change in cell shape.…”

Section: Other Sarcomas Of Myofibroblastsmentioning

confidence: 99%

Myofibrosarcoma

Fisher

2004

Virchows Arch

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Myofibrosarcomas are malignant tumours of myofibroblasts, which have been recognised for many years, but have become clearly defined only recently. They are low- or high-grade sarcomas that arise in soft tissue or bone in adults or children. Low-grade myofibrosarcomas are infiltrative tumours, usually in deep soft tissue, with a predilection for the head and neck region, which display a range of microscopic appearances from fasciitis-like to fibrosarcoma-like; all cases at least focally display nuclear pleomorphism. They express smooth-muscle actin and calponin, and some express desmin, but most lack h-caldesmon. Low-grade myofibrosarcomas can recur but rarely metastasise. Their differential diagnosis is from benign myofibroblastic proliferations, such as fasciitis and fibromatosis, as well as from fibrosarcoma and leiomyosarcoma. Pleomorphic myofibrosarcomas are high-grade pleomorphic sarcomas (malignant fibrous histiocytoma), which show ultrastructural evidence of myofibroblastic differentiation. They closely resemble malignant fibrous histiocytoma clinically and morphologically, but are more frequently actin positive. This article describes the low- and high-grade variants of myofibrosarcoma and other malignant tumours with myofibroblastic differentiation.

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“…[2][3][4] Recently, loss of expression of INI1, a core subunit of a chromatin remodeling complex involved in transcriptional regulation, has been documented in both distal and proximal-type epithelioid sarcomas, providing a possible mechanism of tumorigenesis. 5,6 Current therapeutic strategies use wide surgical excision, radical resection, and in exceptional cases, amputation, followed by chemotherapy and/or radiation therapy.…”

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confidence: 99%

Epithelioid sarcoma expresses epidermal growth factor receptor but gene amplification and kinase domain mutations are rare

2010

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Epithelioid sarcoma is a rare, malignant, soft tissue neoplasm that can be classified into proximal, distal and fibroma-like subtypes. Regardless of subtype, epithelioid sarcoma often shows morphologic and immunophenotypic evidence of epithelial differentiation. Current therapeutic strategies include surgical resection, amputation, radiation or chemotherapy, although the overall prognosis remains poor. The epidermal growth factor receptor (EGFR) is a novel therapeutic target in carcinomas. In some carcinomas, EGFR kinase domain mutations or gene amplification may correlate with response to specific inhibitors. EGFR expression has been reported in some sarcoma types, but expression, amplification and mutations have not been studied in epithelioid sarcoma. We evaluated 15 cases of epithelioid sarcoma from 14 patients for EGFR expression using immunohistochemistry, EGFR copy number aberration using fluorescence in situ hybridization and screened for mutations in the tyrosine kinase domain of the EGFR gene using direct sequencing. In all, 14 of the 15 epithelioid sarcomas (93%) showed expression of EGFR by immunohistochemistry. A majority of the cases (n ¼ 11, 73%) showed strong (2 þ to 3 þ ) and homogeneous (475% of cells) membrane staining. No amplification or polysomy of the EGFR gene or mutations of the tyrosine kinase domain of EGFR (exons 18-21) were detected. These results imply that although EGFR is expressed in most epithelioid sarcomas regardless of subtype, gene amplification and activating mutations in the tyrosine kinase domain appear to be rare or absent. Thus, the benefit of targeted therapy against EGFR in patients with epithelioid sarcoma remains to be determined.

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Epithelioid sarcoma: The spectrum of ultrastructural differentiation in seven immunohistochemically defined cases

Cited by 85 publications

References 52 publications

Prognostic significance of dysadherin expression in epithelioid sarcoma and its diagnostic utility in distinguishing epithelioid sarcoma from malignant rhabdoid tumor

Prognostic significance of dysadherin expression in epithelioid sarcoma and its diagnostic utility in distinguishing epithelioid sarcoma from malignant rhabdoid tumor

Myofibrosarcoma

Epithelioid sarcoma expresses epidermal growth factor receptor but gene amplification and kinase domain mutations are rare

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