Sarcoma epithelioid

A H- - -H- H- - Carcinoma Carcinoma breast, salivary, sweat gland Mesothelioma Synovial sarcoma Epithelioid sarcoma Chordoma Mixed tumor... 

Some sarcomas besides epithelioid sarcoma or synovial sarcoma show coexpression of vimentin and keratin (18). Examining these tumors for the expression of other epithelial markers will help clarify their true nature. 

Kuhnen, C., Tolnay, E., Steinau, H. U., Voss, B. and Muller, K. M. (1998). Expression of c-met receptor and hepatocyte growth factor/scatter factor in synovial sarcoma and epithelioid sarcoma. Virchows Arch. 432, 337-342. 

A + Carcinoma Mesothelioma Synovial sarcoma Epithelioid sarcoma... 

Antibodies to these PEMs are directed to the apical membrane portions of secretory mammary cells surrounding milk fat globules (mol wt >250 kDa). They react with a variety of epithelial tumors, including mesotheliomas. Like keratin, these antibodies are also expressed on synovial sarcomas, epithelioid sarcomas, and chordomas. They are not as reactive with as wide a variety of tumors as antikeratins. For instance, they do not react with many endocrine tumors. However, they may recognize some renal-cell carcinomas and small-cell anaplastic carcinomas... 

AE1/AE3 Boehringer- Mannheim 1 150 MWER Recognition of epithelioid sarcoma, synovial sarcoma, and divergent... 

Anti-CD34 (M) Dako 1 800 MWER Recognition of endothelial tumors, dermatofibrosarcoma protuberans, solitary fibrous tumors, and selected peripheral nerve sheath tumors and epithelioid sarcomas... 

KER, keratin, as detected by a mixture of CAM5.2, MAK-6, and AEl /AE3 EMA, epithelial membrane antigen VIM, vimentin DES, desmin MSA, muscle-specific actin SMA, smooth muscle (alpha isoform) actin CALD, h-caldesmon S-IOOP, S-100 protein TY, tyrosinase Ml, MART-1 (melan-A) OGN, osteocalcin EPS, epithelioid sarcoma EPSS, epithelioid synovial sarcoma EAS, epithelioid angiosarcoma EMPNST, epithelioid malignant peripheral nerve sheath tumor CCS, clear cell sarcoma SEFS, sclerosing epithelioid fibrosarcoma ... 

Keratins are regularly found in synovial sarcoma, chordoma, parachordoma, epithelioid sarcoma, and adamantinoma. They are seen less commonly in leiomyosarcoma, malignant peripheral nerve sheath tumors, epithelioid angiosarcoma, and polyphenotypic tumors such as PNET. 

EMA regularly decorates synovial sarcoma, epithelioid sarcoma, perineuriomas, neurothekeomas, chordoma/ parachordoma, and plasmacytomas. 

A recent study has shown that loss of nuclear labeling for INIl (Snf5) is highly specific for epithelioid sarcoma in this differential diagnosis, since all evaluated cases of EHE showed retained nuclear staining for this ubiquitously expressed component of the SWI/SNE chromatin remodeling complex. " ... 

Billings and colleagues have described a peculiar tumor in this group that even more closely simulates the image of epithelioid sarcoma on conventional microscopy. 

It has accordingly been named epithelioid sarcomalike hemangioendothelioma (ESLH). Indeed, this entity shares immunoreactivity for keratin and vimentin with true epithelioid sarcoma (EPS), but ESEH paradoxically lacks CD34 (which is seen in EPS and most other hemangioendotheliomas) and additionally labels for CD31 and FLI-1. 

A consistent immunophenotypic attribute of epithelioid sarcoma is an intense perinuclear zone of vimentin and keratin reactivity (Fig. 4.20). It is attributable to perinuclear collections of intermediate filaments that are seen at an ultrastructural level. The scope of cytokeratin labeling may be heterogeneous in any given tumor (Fig. 

Epithelioid sarcomas share immunohistologic attributes with synovial sarcomas, in that both tumor types are reactive for cytokeratin and EMA. In addition, both types may occasionally show reactivity for carcinoem-bryonic antigen and S-100 protein. Most cases of EPS and epithelioid synovial sarcoma also strongly express E-cadherin. ... 

An unexpected determinant was found in 91% of epithelioid sarcomas by Kato and colleagues namely, CA125. This marker is typically associated with Miil-lerian epithelial tumors, especially in the ovaries, and its presence in a mesenchymal neoplasm is inexplicable. All other polygonal-cell tumors of soft tissue in the series just cited were CA 125-negative. ... 

The potential presence of CD34 in diverse polygonal-cell soft tissue tumors, including epithelioid LMS, epithelioid MPNST, clear cell sarcoma, and epithelioid sarcoma, has diminished its potential utility as an indicator of endothelial differentiation." " We also have observed that epithelioid angiosarcomas may be reactive... 

FIGURE 4.20 Epithelioid sarcoma showing intense immunoreac-tivity for vimentin, with perinuclear accentuation. 

FIGURE 4.21 Keratin positivity is shown in this epithelioid sarcoma. 

Angiosarcoma CD31-r/FLI-1-r epithelioid sarcoma CD34-I- synovial sarcoma negative for CD31 /CD34/FLI-1. 

Extrarenal rhabdoid tumors (ERTs) often show epithelial differentiation, and some may be difficult—if not impossible—to separate diagnostically from such entities as proximal-type epithelioid sarcoma. " INIl, a protein encoded by a gene at chromosome 22qll.2, is involved in chromatin remodeling it is usually absent in both ERT and proximal epithelioid sarcoma, as well as conventional (distal) epithelioid sarcoma. The majority of tumors in each category also show... 

Manivel JC, Wick MR, Dehner LP, et al. Epithelioid sarcoma An immunohistochemical study. Am J Clin Pathol. 1987 87 319-yiG. 

Zevallos-Giampietri EA, Barrionuevo C. Proximal-type epithelioid sarcoma report of two cases in the perineum Differential diagnosis and review of soft tissue tumors with epithelioid and/ or rhabdoid feamres. Appl Immunohistochem Mol Morphol. 2005 13 221-230. 

Arber DA, Kandalaft PL, Mehta P, et al. Vimentin-negative epithelioid sarcoma The value of an immunohistochemical panel that includes CD34. Am J Surg Pathol. 1993 17 302-317. 

Wick MR, Manivel JG. Epithelioid sarcoma and epithelioid hemangioendothelioma An immunohistochemical and lectin-his-tochemical comparison. Virchows Arch A. 1987 410 309-316. 

Hornick JL, Dal Cin P, Eletcher GDM. Loss of INll expression is characteristic of both conventional and proximal-type epithelioid sarcoma. Am J Surg Pathol. 2009, in press. 

Billings SD, Folpe AL, Weiss SW. Epithelioid sarcoma-like hemangioendothelioma. Am J Surg Pathol. 2003 27 48-57. 

Von Hochstetter AR, Meyer VE, Grant JW, et al. Epithelioid sarcoma mimicking angiosarcoma The value of immunohisto-chemistry in the differential diagnosis. Virchows Arch A Pathol Anat Histopathol. 1991 418 271-278. 

Ghetty R, Slavin JL. Epithelioid sarcoma with extensive chon-droid differentiation. Histopathology. 1994 24 400-401. 

Laskin WB, Miettinen M. Epithelioid sarcoma new insights based on an extended immunohistochemical analysis. Arch Pathol Lab Med. 2003 127 1161-1168. 

Guillou L, Wadden C, Coindre JM, et al. Proximal type epithelioid sarcoma A distinctive aggressive neoplasm showing rhabdoid feature Clinicopathologic, immunohistochemi-cal, and ultrastructural study of a series. Am J Surg Pathol. 1997 21 130-146. 

Gerharz CD, Moll R, Meister P, et al. Gytoskeletal heterogeneity of an epithelioid sarcoma with expression of vimentin, cyto-keratins, and neurofilaments. Am J Surg Pathol. 1990 14 274-283. 

Smith ME, Brown JI, Fisher C. Epithelioid sarcoma Presence of vascular-endothelial cadherin and lack of epithelial cadherin. Histopathology. 1998 33 425-431. 

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