Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken


Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_5349-1


A benign, well-circumscribed tumor of the pelvicoperineal region composed of a prominent vascular and stromal component, the latter showing myofibroblastic differentiation

Clinical Features

  • Incidence

    Angiomyofibroblastoma is uncommon.

  • Age

    These tumors typically occur in adults over a wide age range. In women, approximately 10% occur following menopause.

  • Sex

    Angiomyofibroblastoma more commonly occurs in women.

  • Site

    These tumors arise in the subcutaneous tissue of the pelvicoperineum, with the vulva being the most common location. Clinically, they are often thought to represent a Bartholin gland cyst. Approximately 10–15% occur in the vagina. In men, they occur in paratesticular soft tissue or the scrotum.

  • Treatment

    Local excision is adequate treatment.

  • Outcome

    Angiomyofibroblastoma is benign. Recurrence is rare even after marginal excision.


These tumors usually measure <5 cm and are well demarcated but lack a capsule. They typically have a soft to rubbery...

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

References and Further Reading

  1. Chen, B. J., Marino-Enriquez, A., Fletcher, C. D., & Hornick, J. L. (2012). Loss of retinoblastoma protein expression in spindle cell/pleomorphic lipomas and cytogenetically related tumors: An immunohistochemical study with diagnostic implications. The American Journal of Surgical Pathology, 36, 1119–1128.CrossRefGoogle Scholar
  2. Fletcher, C. D., Tsang, W. Y., Fisher, C., et al. (1992). Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma. The American Journal of Surgical Pathology, 16(4), 373–382.CrossRefGoogle Scholar
  3. Granter, S. R., Nucci, M. R., & Fletcher, C. D. (1997). Aggressive angiomyxoma: Reappraisal of its relationship to angiomyofibroblastoma in a series of 16 cases. Histopathology, 30(1), 3–10.CrossRefGoogle Scholar
  4. Laskin, W. B., Fetsch, J. F., & Tavassoli, F. A. (1997). Angiomyofibroblastoma of the female genital tract: Analysis of 17 cases including a lipomatous variant. Human Pathology, 28(9), 1046–1055.CrossRefGoogle Scholar
  5. Luis, P. P., Quiñonez, E., Nogales, F. F., et al. (2015). Lipomatous variant of angiomyofibroblastoma involving the vulva: Report of 3 cases of an extremely rare neoplasm with discussion of the differential diagnosis. International Journal of Gynecological Pathology, 34(2), 204–207.CrossRefGoogle Scholar
  6. Magro, G., Righi, A., Caltabiano, R., et al. (2014). Vulvovaginal angiomyofibroblastomas: Morphologic, immunohistochemical, and fluorescence in situ hybridization analysis for deletion of 13q14 region. Human Pathology, 45(8), 1647–1655.CrossRefGoogle Scholar
  7. Nielsen, G. P., Rosenberg, A. E., Young, R. H., et al. (1996). Angiomyofibroblastoma of the vulva and vagina. Modern Pathology, 9(3), 284–291.PubMedGoogle Scholar
  8. Ockner, D. M., Sayadi, H., Swanson, P. E., et al. (1997). Genital angiomyofibroblastoma. Comparison with aggressive angiomyxoma and other myxoid neoplasms of skin and soft tissue. American Journal of Clinical Pathology, 107(1), 36–44.CrossRefGoogle Scholar
  9. Sims, S. M., Stinson, K., McLean, F. W., Davis, J. D., & Wilkinson, E. J. (2012). Angiomyofibroblastoma of the vulva: A case report of a pedunculated variant and review of the literature. Journal of Lower Genital Tract Disease, 16, 149–154.CrossRefGoogle Scholar

Authors and Affiliations

  1. 1.Division of Women’s and Perinatal Pathology, Department of PathologyBrigham Women’s HospitalBostonUSA