Skip to main content
SpringerLink
Log in

Myoepitheliale Tumoren der Haut und des Weichgewebes

Myoepithelial neoplasms of skin and soft tissues

  • Schwerpunkt: Myoepitheliale Tumoren
  • Published:
Der Pathologe Aims and scope Submit manuscript

Zusammenfassung

Myoepitheliale Tumoren der Haut und des Weichgewebes umfassen kutane Mischtumoren (chondroide Syringome), Mischtumoren der Subkutis und des tiefen Weichgewebes, Myoepitheliome und die seltenen malignen Myoepitheliome (myoepitheliale Karzinome). Wie auch in anderen anatomischen Lokalisationen sind Myoepitheliome der Haut und des Weichgewebes durch eine außerordentliche klinisch-pathologische Heterogenität charakterisiert. Die Tumoren entstehen sowohl im Kindes- als auch im Erwachsenenalter und bestehen aus epithelioiden, histiozytoiden, spindeligen, plasmozytoiden und/oder klarzelligen Tumorzellen in wechselnder Zusammensetzung, die in einer myxoiden oder hyalinisierten Matrix angeordnet sind. Immunhistochemisch nachweisbar werden von den Tumoren epitheliale Marker (Panzytokeratin und/oder epitheliales Membranantigen), häufig S-100 Protein sowie teilweise muskuläres Aktin, gliales saures Faserprotein, Calponin und p63 exprimiert, während Desmin in der Regel nicht zu detektieren ist. Der Nachweis mittel- bis hochgradiger zytologischer Atypien ist in Myoepitheliomen mit einer verschlechterten Prognose assoziiert, und maligne Myoepitheliome können in einer signifikanten Anzahl von Fällen metastasieren und zum Tode der betroffenen Patienten führen.

Abstract

Myoepithelial neoplasms of skin and soft tissues comprise cutaneous mixed tumor (chondroid syringoma), mixed tumor of subcutaneous and deep soft tissues, myoepithelioma and rare malignant myoepithelioma (myoepithelial carcinoma). Myoepithelial tumors of skin and soft tissues are characterized by an extreme clinicopathological heterogeneity as in other anatomic locations. The neoplasms arise in childhood as well as in adults and are composed of epithelioid, histiocytoid, spindled, plasmocytoid and/or clear tumour cells in varying combinations, and are set in a myxoid or hyalinised intercellular matrix. Immunohistochemically, neoplastic cells stain positively for epithelial markers (pancytokeratin and/or epithelial membrane antigen), and often for S 100 protein. More rarely muscle actin, glial fibrillary acid protein, calponin, and p63 are expressed, whereas desmin is usually negative. The presence of at least moderate cytological atypia is associated with a significant risk for aggressive behavior and prospensity for metastasis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4

Literatur

  1. Bisceglia M, Cardone M, Fantasia L et al. (2001) Mixed tumors, myoepitheliomas, and oncocytomas of soft tissues are likely members of the same family: a clinicopathologic and ultrastructural study. Ultrastruct Pathol 25:399–418

    PubMed  Google Scholar 

  2. Eckert F, Betke M, Schmöckel C et al. (1992) Myoepithelial differentiation in benign sweat gland tumors demonstrated by monoclonal antibody to alpha-smooth muscle actin. J Cutan Pathol 19:294–301

    PubMed  Google Scholar 

  3. Fernandez-Figueras MT, Puig L, Trias I et al. (1998) Benign myoepithelioma of the skin. Am J Dermatopathol 20:208–212

    PubMed  Google Scholar 

  4. Franke WW, Schmid E, Freudenstein C et al. (1980) Intermediate-sized filaments of the prekeratin type in myoepithelial cells. J Cell Biol 84:633–654

    PubMed  Google Scholar 

  5. Fulford LG, Kamata Y, Okudera K et al. (2001) Epithelial-myoepithelial carcinomas of the bronchus. Am J Surg Pathol 25:1508–1514

    PubMed  Google Scholar 

  6. Hamperl H (1970) The myothelia (myoepithelial cells): normal state; regressive changes; hyperplasia; tumors. Curr Top Pathol 53:161–220

    PubMed  Google Scholar 

  7. Harrist TJ, Aretz TH, Mihm MC, Evans GW (1981) Cutaneous malignant mixed tumor. Arch Dermatol 117:719–724

    PubMed  Google Scholar 

  8. Hornick JL, Fletcher CDM (2003) Myoepithelial tumors of soft tissue. A clinicopathologic and immunohistochemical study of 101 cases with evaluation of prognostic parameters. Am J Surg Pathol 27:1183–1196

    PubMed  Google Scholar 

  9. Hornick JL, Fletcher CDM (2004) Cutaneous myoepithelioma: a clinicopathologic and immunohistochemical study of 14 cases. Hum Pathol 35:14–24

    PubMed  Google Scholar 

  10. Kilpatrick SE, Hitchcock MG, Kraus MD et al. (1997) Mixed tumor and myoepitheliomas of soft tissue: a clinicopathologic study of 19 cases with a unifying concept. Am J Surg Pathol 21:13–22

    PubMed  Google Scholar 

  11. Kim YC, Vandersteen DP, Chung YJ et al. (2001) Signet ring cell basal cell carcinoma. A basal cell carcinoma with myoepithelial differentiation. Am J Dermatopathol 23:525

    PubMed  Google Scholar 

  12. Kutzner H, Mentzel T, Kaddu S et al. (2001) Cutaneous myoepithelioma: an underecognized cutaneous neoplasm composed of myoepithelial cells. Am J Surg Pathol 25:348–355

    PubMed  Google Scholar 

  13. McKee PH (1996) Malignant chondroid syringoma. In: McKee PH (ed) Pathology of the skin, 2nd edn. Mosby-Wolfe, London, 15.66

  14. Meis-Kindlom JM, Bergh P, Gunterberg B et al. (1999) Extraskeletal myxoid chondrosarcoma: a reappraisal of its morphologic spectrum and prognostic factors based on 117 cases. Am J Surg Pathol 23:636–650

    PubMed  Google Scholar 

  15. Mentzel T, Requena L, Kaddu S et al. (2003) Cutaneous myoepithelial neoplasms: clinicopathologic and immunohistochemical study of 20 cases suggesting a continuous spectrum ranging from benign mixed tumor of the skin to myoepithelioma and myoepithelial carcinoma. J Cutan Pathol 30:294–302

    PubMed  Google Scholar 

  16. Michal M, Miettinen M (1999) Myoepitheliomas of the skin and soft tissues. Virchows Arch 434:393–400

    PubMed  Google Scholar 

  17. Morinaga S, Nakajima T, Shimosato Y (1987) Normal and neoplastic myoepithelial cells in salivary glands. Hum Pathol 18:1218–1226

    PubMed  Google Scholar 

  18. Neto AG, Pineda-Daboin K, Luna MA (2004) Myoepithelioma of the soft tissue of the head and neck: a case report and review of the literature. Head Neck 26:470–473

    PubMed  Google Scholar 

  19. Prasad AR, Savera AT, Gown AM et al. (1999) The myoepithelial immunophenotype in 135 benign and malignant salivary gland tumors other than pleomorphic adenoma. Arch Pathol Lab Med 123:801–806

    PubMed  Google Scholar 

  20. Sasaguri T, Tanimoto A, Arima N et al. (1999) Myoepithelioma of soft tissue. Pathol Int 49:571–576

    PubMed  Google Scholar 

  21. Savera AT, Sloman A, Huvos AG, Klimstra DS (2000) Myoepithelial carcinoma of the salivary gland. A clinicopathologic study of 25 patients. Am J Surg Pathol 24:761–774

    PubMed  Google Scholar 

  22. Suster S, Ramon y Cajal S (1991) Myoepithelial differentiation in basal cell carcinoma. Am J Dermatopathol 13:350–357

    PubMed  Google Scholar 

  23. Takahashi H, Ishiko A, Kobayashi M et al. (2004) Malignant chandroid syringoma with bone invasion: a case report and review of the literature. Am J Dermatopathol 26:403–406

    PubMed  Google Scholar 

  24. Tavassoli FA (1991) Myoepithelial lesions of the breast: myoepitheliosis, adenomyoepithelioma, and myoepithelial carcinoma. Am J Surg Pathol 15:554–568

    PubMed  Google Scholar 

  25. Venkatraman L, Sinnathuray AR, Raut V et al. (2002) Soft tissue myoepithelioma: a case report. Pathology 34:451–454

    PubMed  Google Scholar 

Download references

Interessenkonflikt:

Der korrespondierende Autor versichert, dass keine Verbindungen mit einer Firma, deren Produkt in dem Artikel genannt ist, oder einer Firma, die ein Konkurrenzprodukt vertreibt, bestehen.

Author information

Authors and Affiliations

  1. Dermatopathologische Gemeinschaftspraxis, Friedrichshafen

    T. Mentzel

  2. Dermatopathologische Gemeinschaftspraxis , Siemensstraße 6/1, 88048, Friedrichshafen

    T. Mentzel

Authors
  1. T. Mentzel
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to T. Mentzel.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mentzel, T. Myoepitheliale Tumoren der Haut und des Weichgewebes. Pathologe 26, 322–330 (2005). https://doi.org/10.1007/s00292-005-0776-z

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00292-005-0776-z

Schlüsselwörter

Keywords

Search

Navigation