Virchows Archiv

, Volume 448, Issue 2, pp 195–199 | Cite as

Elastofibroma: clonal fibrous proliferation with predominant CD34-positive cells

Original Article


Elastofibroma is a rare fibrous lesion that most commonly occurs in periscapular soft tissues and is characterized by accumulated abnormal elastic fibers. Although the lesion is generally regarded as a reactive process, an unusual fibroblastic pseudotumor, or as a fibroelastic tumor-like lesion, its etiology remains largely unknown. Recent cytogenetic demonstrations of chromosomal instability and some recurrent or clonal chromosomal changes have raised the possibility that the lesion represents a neoplastic process. We analyzed 14 cases of elastofibroma to further explore, morphologically and genetically, the characteristics of its cellular composition. The interspersed spindle or stellate cells showed a fibroblast-like appearance and were almost consistently positive for vimentin and frequently positive for CD34 and lysozyme immunohistochemically. No spindle cells of myofibroblastic phenotype were recognized. To assess the clonality of the lesions in female patients, the X-linked polymorphic human androgen receptor gene assay was performed using formalin-fixed, paraffin-embedded tissues. A nonrandom inactivation of the androgen receptor gene was detected in two informative cases. Thus, these findings suggest that CD34-positive mesenchymal cells are an integral component of elastofibroma, which represents a clonal fibrous proliferation.


Soft tissue Immunohistochemistry Electron microscopy Molecular genetics 



This work was partly supported by a grant provided by the Ichiro Kanehara Foundation.


  1. 1.
    Abe R, Donnelly SC, Peng T, Bucala R, Metz CN (2001) Peripheral blood fibrocytes: differentiation pathway and migration to wound sites. J Immunol 166:7556–7562PubMedGoogle Scholar
  2. 2.
    Batstone P, Forsyth L, Goodlad J (2001) Clonal chromosome aberrations secondary to chromosome instability in an elastofibroma. Cancer Genet Cytogenet 128:46–47CrossRefPubMedGoogle Scholar
  3. 3.
    Javanovic L, Delahunt B, McIver B, Eberhardt HL, Grebe SKG (2003) Optimising restriction enzyme cleavage of DNA derived from archival histopathological samples: an improved HUMARA assay. Pathology 35:70–74PubMedCrossRefGoogle Scholar
  4. 4.
    Honma M, Koizumi F, Wakaki K, Ochiai H (1995) Co-expression of fibroblastic, histiocytic and smooth muscle cell phenotypes on cultured adherent cells derived from human palatine tonsils: a morphological and immunocytochemical study. Pathol Int 45:903–913PubMedCrossRefGoogle Scholar
  5. 5.
    Kahn HJ, Hanna WH (1995) “Aberrant elastic” in elastofibroma: an immunohistochemical and ultrastructural study. Ultrastruct Pathol 19:45–50PubMedCrossRefGoogle Scholar
  6. 6.
    Kayaselcuk F, Demirhan B, Kayaselcuk U, Özedem ÖR, Tuncer I (2002) Vimentin, smooth muscle actin, desmin, S-100 protein, p53, and estrogen receptor expression in elastofibroma and nodular fasciitis. Ann Diagn Pathol 6:94–99CrossRefPubMedGoogle Scholar
  7. 7.
    Kindblom L-G, Spicer SS (1982) Elastofibroma. A correlated light and electron microscopic study. Virchows Arch 396:127–140CrossRefGoogle Scholar
  8. 8.
    McComb EN, Feely MG, Neff JR, Johansson SL, Nelson M, Bridge JA (2001) Cytogenetic instability, predominantly involving chromosome 1, is characteristic of elastofibroma. Cancer Genet Cytogenet 126:68–72CrossRefPubMedGoogle Scholar
  9. 9.
    Mitterbauer G, Winkler K, Gisslinger H, Geissler K, Lechner K, Mannhalter C (1999) Clonality analysis using X-chromosome inactivation at the human androgen receptor gene (HUMARA). Evaluation of large cohorts of patients with chronic myeloproliferative diseases, secondary neutrophilia, and reactive thrombocytosis. Am J Clin Pathol 112:93–100PubMedGoogle Scholar
  10. 10.
    Nagamine N, Nohara Y, Ito E (1982) Elastofibroma in Okinawa: a clinicopathologic study of 170 cases. Cancer 50:1794–1805PubMedCrossRefGoogle Scholar
  11. 11.
    Nishio J, Iwasaki H, Ohjimi Y, Ishiguro M, Koga T, Isayama T, Naito M, Kikuchi M (2002) Gain of Xp detected by comparative genomic hybridization in elastofibroma. Int J Mol Med 10:277–280PubMedGoogle Scholar
  12. 12.
    Pinkus GS, Said JW (1977) Profile of intracytoplasmic lysozyme in normal tissues, myeloproliferative disorders, hairy cell leukemia, and other pathologic processes: an immunoperoxidase study of paraffin sections and smears. Am J Pathol 89:351–366PubMedGoogle Scholar
  13. 13.
    Ramos CV, Gillespie W, Narconis RJ (1978) Elastofibroma. A pseudotumor of myofibroblasts. Arch Pathol Lab Med 102:538–540PubMedGoogle Scholar
  14. 14.
    Schepel JAC, Wille J, Seldenrijk CA, van Ramshorst B (1998) Elastofibroma: a familial occurrence. Eur J Surg 164:557–558CrossRefPubMedGoogle Scholar
  15. 15.
    Schürch W, Seemayer TA, Gabbiani G (1998) The myofibroblast. A quarter century after its discovery. Am J Surg Pathol 22:141–147CrossRefPubMedGoogle Scholar
  16. 16.
    Shteyngart B, Chaiwiriyakul S, Wong J, Cantor JO (1998) Preferential binding of lysozyme to elastic fibers in pulmonary emphysema. Thorax 53:193–196PubMedCrossRefGoogle Scholar
  17. 17.
    Silverman JS, Tamsen A (1998) Interactive CD34-positive fibroblasts and factor XIIIa-positive histiocytes in cutaneous mesenchymal tumors. Am J Dermatopathol 20:317–319CrossRefPubMedGoogle Scholar
  18. 18.
    Suwabe H, Serizawa A, Kajiwara H, Ohkido M, Tsutsumi Y (1999) Degenerative processes of elastic fibers in sun-protected and sun-exposed skin: immunoelectron microscopic observation of elastin, fibrillin-1, amyloid P component, lysozyme and α1-antitrypsin. Pathol Int 49:391–402CrossRefPubMedGoogle Scholar
  19. 19.
    Vanni R, Marras S, Faa G, Uccheddu A, Dal Cin P, Sciot R, Samson I, Van den Berghe H (1999) Chromosome instability in elastofibroma. Cancer Genet Cytogenet 111:182–183CrossRefPubMedGoogle Scholar
  20. 20.
    Weiss SW, Goldblum JR (2001) Elastofibroma. In: Weiss SW, Goldblum JR (eds) Soft tissue tumors. Mosby, St. Louis, pp 286–289Google Scholar
  21. 21.
    Yamazaki K (2005) CD10- and CD34-positive periglandular stromal cells in pulmonary benign metastasizing leiomyoma with metaplastic adenomyomatous glands: an ultrastructural and immunohistochemical study. Virchows Arch 446:270–277CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  1. 1.Department of Pathology and Oncology, School of MedicineUniversity of Occupational and Environmental HealthKitakyushuJapan

Personalised recommendations