Aggressive angiomyxoma of the vulva: our experience of a rare case with review of the literature.

Aggressive angiomyxoma is a rare, benign but locally aggressive mesenchymal neoplasm, which occurs almost exclusively during the reproductive years of women. A 28-year-old woman developed an aggressive angiomyxoma within the left labium minus of the vulva. The tumor was excised, but the lesion was expanded to the surgical margins. Microscopically, sections showed many walled vessels of various sizes, a loose myxoid and collagenous stroma and stellate and spindle-shaped neoplasmatic cells. Immunohistochemically, the neoplasmatic cells showed strong positivity for vimentin and desmin and moderate positivity for CD34 and estrogen receptors. In conclusion, aggressive angiomyxoma of the vulva should be distinguished from the benign and malignant myxoid tumors or tumor-like conditions of vulva. The pathologic and immunohistochemic characteristics, the difficulties in determining the surgical margins and the treatment of this tumor are discussed. Also, the international literature is reviewed.