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  • Print publication year: 2010
  • Online publication date: March 2011



Epithelial differentiation is often easily detected histologically and is almost always highlighted with immunohistochemical markers (i.e., keratins, epithelial membrane antigen [EMA], and others). Mesothelial proliferations and tumors are discussed in Chapter 28, and Merkel cell carcinoma and metastatic carcinomas in Chapter 29.

Some other nonepithelial cells and tumors also can express keratins and other epithelial markers. Examples of normal varyingly keratin-positive mesenchymal cells include endothelial and smooth muscle cells and some myofibroblasts. Many nonepithelial tumors, such as melanomas and many sarcomas, occasionally are keratin positive. These are discussed in the appropriate chapters.

Synovial sarcoma and epithelioid sarcoma are epithelial tumors of soft tissue of unknown histogenesis. These tumors are not thought to be closely related.

Mixed tumors and related skin adnexal tumors, and chordoma, a tumor mimicking the features of notochord cells, are also reviewed here.


A synovial sarcoma is a specific soft tissue sarcoma with dual epithelial and mesenchymal differentiation. The tumor is not related to synovial lining cells, and its association with synovia, if any, is only incidental. The name is based on historical reasons, because the epithelial component of the biphasic tumors was once thought to replicate synovial slits. The typical t(X;18) translocation with SS18-SSX gene fusion is a useful diagnostic marker.

Demographics of Synovial Sarcoma

Synovial sarcomas are relatively common sarcomas, constituting from 7% to 10% of all soft tissue sarcomas. This tumor typically presents in young adults, with a median age of approximately 30 years and a mild male predominance.

Synovial Sarcoma – General Reviews and Clinicopathologic Series
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Synovial Sarcoma – Histologic Variants and Prognostic Factors
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Synovial Sarcoma – Immunohistochemistry
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Synovial Sarcoma – Genetics
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Epithelioid Sarcoma
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Genetics of Epithelioid Sarcoma
Stenman, S, Kindblom, LG, Willems, J, Angervall, L: A cell culture, chromosomal and quantitative DNA analysis of a metastatic epithelioid sarcoma. Cancer 1990;65:2006–2013.
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Large Cell (Proximal-Type) Epithelioid Sarcoma
Hasegawa, T, Matsuno, Y, Shimoda, T, Umeda, T, Yoakoyama, R, Hiroshasi, S. Proximal-type epithelioid sarcoma: A clinicopathologic study of 20 cases. Mod Pathol 2001;14:655–663.
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Mixed Tumor/Myoepithelioma and Parachordoma
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Headington, JT. Mixed tumor of the skin: Eccrine and apocrine type. Arch Dermatol 1961;84:989–996.
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Dabska, M. Parachordoma – A new clinical entity. Cancer 1977;40:1586–1592.
Fisher, C, Miettinen, M. Parachordoma: Clinicopathologic and immunohistochemical study of four cases of an unusual soft tissue neoplasm. Ann Diagn Pathol 1997;1:3–10.
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Other Adnexal Tumors
Johnson, BL, Helwig, EB. Eccrine acrospiroma: A clinicopathologic study. Cancer 1969;23:641–657.
Winkelmann, RK, Wolff, K. Solid-cystic hidradenoma. Arch Dermatol 1968;97:651–661.
Wiley, EL, Milchgrub, S, Freeman, RG, Kim, ES. Sweat gland adenomas: Immunohistochemical study with emphasis on myoepithelial differentiation. J Cutan Pathol 1993;20:337–343.
Demirkesen, C, Hoede, N, Moll, R. Epithelial markers and differentiation in adnexal neoplasms of the skin: An immunohistochemical study including individual cytokeratins. J Cutan Pathol 1995;22:518–535.
Kersting, DW, Helwig, EB. Eccrine spiradenoma. AMA Arch Dermatol 1956;73:199–227.
Kao, GF, Laskin, WB, Weiss, SW. Eccrine spiradenoma occurring in infancy mimicking mesenchymal tumor. J Cutan Pathol 1990;17:214–219.
Mambo, NC. Eccrine spiradenoma: clinical and pathologic study of 49 tumors. J Cutan Pathol 1983;10:312–320.
Evans, HL, Daniel, WP, Smith, JL, Winkelmann, RK. Carcinoma arising in eccrine spiradenoma. Cancer 1979;43:1881–1884.
Wick, MR, Swanson, PE, Kaye, VN, Pittelkow, MR. Sweat gland carcinoma ex eccrine spiradenoma. Am J Dermatopathol 1987;9:90–98.
Argenyi, ZB, Nguyen, AV, Balogh, K, Sears, JK, Whitaker, DC. Malignant eccrine spiradenoma: A clinicopathologic study. Am J Dermatopathol 1992;14:381–390.
Granter, SR, Seeger, K, Calonje, E, Busam, K, McKee, PH. Malignant eccrine spiradenoma (spiradenocarcinoma): A clinicopathologic study of 12 cases. Am J Dermatopathol 2000;22:97–103.
McKee, PH, Fletcher, CDM, Stavrinos, P, Pambakian, H. Carcinosarcoma arising in eccrine spiradenoma: A clinicopathologic and immunohistochemical study of two cases. Am J Dermatopathol 1990;12:335–343.
Oord, JJ, Wolf-Peeters, C. Perivascular spaces in eccrine spiradenoma: A clue to its histological diagnosis. Am J Dermatopathol 1995;17:266–270.
Watanabe, S, Hirose, M, Sato, S, Takahashi, H. Immunohistochemical analysis of cytokeratin expression in eccrine spiradenoma: similarities to the transitional portions between secretory segments and coiled ducts of eccrine glands. Br J Dermatol 1994;131:799–807.
Al-Nafussi, A, Blessing, K, Rahilly, M. Non-epithelial cellular components in eccrine spiradenoma: A histological and immunohistochemical study of 20 cases. Histopathology 1991;18:155–160.
Crain, RC, Helwig, EB. Dermal cylindroma (dermal eccrine cylindroma). Am J Clin Pathol 1961;35:504–515.
Takahashi, M, Rapley, E, Biggs, PJ, Lakhani, SR, Cooke, D, Hansen, J. Linkage and LOH studies in 19 cylindromatosis families show no evidence of genetic heterogeneity and define the CYLD locus on chromosome 16q12-q13. Gum Genet 2000;106:58–65.
Lee, DA, Grossman, ME, Scheinerman, P, Celebi, JT. Genetics of skin appendage neoplasms and related syndromes. J Med Genet 2005;42:811–819.
Massoumi, R, Paus, R. Cylindromatosis and the CYLD gene: New lessons on the molecular principles of epithelial growth control. Bioessays 2007;29:1203–1214.
Kao, GF, Helwig, EB, Graham, JH. Aggressive digital papillary adenoma and adenocarcinoma: A clinicopathological study of 57 patients, with histochemical, immunopathological, and ultrastructural observations. J Cutan Pathol 1987;14:129–146.
Duke, WH, Sherrod, TT, Lupton, GP. Aggressive digital papillary adenocarcinoma (aggressive digital papillary adenoma and adenocarcinoma revisited). Am J Surg Pathol 2000;24:775–784.
Bakotic, B, Antonescu, CR. Aggressive digital papillary adenocarcinoma of the foot: The clinicopathologic features of two cases. J Foot Ankle Surg 2000;39:402–405.
Chambers, PW, Schwinn, CP: Chordoma: A clinicopathologic study of metastasis. Am J Clin Pathol 1979;72:765–776.
Su, WPD, Louback, JB, Gagne, EJ, Scheithauer, BW: Chordoma cutis: A report of nineteen patients with cutaneous involvement of chordoma. J Am Acad Dermatol 1993;29:63–66.
Suster, S, Moran, C: Chordomas of the mediastinum: Clinicopathologic, immunohistochemical, and ultrastructural study of six cases presenting as posterior mediastinal masses. Hum Pathol 1995;26:1354–1362.
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Ectopic Hamartomatous Thymoma
Rosai, J, Limas, C, Husband, EM: Ectopic hamartomatous thymoma: A distinctive benign lesion of lower neck. Am J Surg Pathol 1984;8:501–513.
Fetsch, JF, Laskin, WB, Michal, M, Remotti, F, Heffner, D, Ellis, G. Ectopic hamartomatous thymoma: A clinicopathologic and immunohistochemical analysis of 21 cases with data supporting reclassification as a branchial anlage mixed tumor. Am J Surg Pathol 2004;28:1360–1370.
Fetsch, JF, Weiss, SW: Ectopic hamartomatous thymoma: clinicopathologic, immunohistochemical, and histogenetic considerations in four new cases. Hum Pathol 1990;21:662–668.
Chan, JK, Rosai, J. Tumors of the neck showing thymic or related branchial pouch differentiation: A unifying concept. Hum Pathol 1991;22:349–367.
Michal, M, Zamecnik, M, Gogora, M, Mukensnabl, P, Neubauer, L. Pitfalls in the diagnosis of ectopic hamartomatous thymoma. Histopathology 1996;29:549–555.
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