Definition
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.
Macroscopy
These tumors usually measure <5 cm and are well demarcated but lack a capsule. They typically have a soft to rubbery...
References and Further Reading
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.
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.
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.
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.
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.
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.
Nielsen, G. P., Rosenberg, A. E., Young, R. H., et al. (1996). Angiomyofibroblastoma of the vulva and vagina. Modern Pathology, 9(3), 284–291.
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.
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.
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Nucci, M.R. (2020). Angiomyofibroblastoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5349-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5349-1
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