Huge pedunculated angiomyofibroblastoma of the vulva

Abstract

We present the case of a huge pedunculated benign mesenchymal myxoid tumor that developed on the right labia majora of a 48-year-old-woman. The excised mass weighed 4534 g and was 23 cm in diameter; the cut surface was yellowish and elastic. Microscopic examination revealed spindle and plump oval tumor cells arranged with abundant capillary vessels in an edematous stroma. Immunohistochemical staining showed that the tumor cells were positive for vimentin, desmin, estrogen receptor, and progesterone receptor, but negative for alpha-smooth muscle actin, CD34, CD45, CD68, and S-100. Based on these features, the pathological diagnosis was angiomyofibroblastoma. A pedunculated angiomyofibroblastoma is extremely rare and, to the best of our knowledge, this is the biggest such tumor in terms of size and weight reported to date. It is especially important in such a huge mass greater than 10 cm that angiomyofibroblastoma is differentiated from aggressive angiomyxoma, which is a deeply invasive and recurrent neoplasm.

Angiomyofibroblastoma of Vulva. A Rare Tumor Entity: A Case Report

“…Not all tumor cells are negative for alpha smooth muscle actin and CD34. Staining for ER and PR was observed in the nucleus of all tumor cells 1,6,8,9,10 . Bigotti et 11 al described about strong CD44 expression by aggressive angiomyxoma where as angiomyofibroblastoma shows a weak and heterogenous positivity for CD44.…”

“…Angiomyofibroblastoma cases are desmin positive and alpha smooth muscle actin negative. However,some are negative for desmin or positive for alpha smooth muscle actin 6,8,9,10 . The tumor cells are uniformly negative for cytokeratin and S-100 protein and CD34.…”

Angiomyofibroblastoma of the vulva: A case report with literature review

“…AMF is an unusual kind of vulvar disease and commonly misdiagnosed as Bartholin cyst or lipoma. The size of the tumor is usually less than 5 cm, but it was measured up to 23 cm in a report [2]. When the size of the tumor is big enough, it can be confused with perineal hernia.…”

A Vaginal Angiomyofibroblastoma as a Rare Cause of a Prolapsing Vaginal Mass: A Case Report and Review of the Literature

“…It is most prevalent in women in the reproductive age group with a mean age of 45 and varies in size (from 0.5 to 23 cm) but is usually less than 5 cm [1–4]. …”