Cellular angiofibroma of the vulva: a poorly known entity, a case report and literature review

Abstract

BackgroundCellular angiofibroma represents a newly described, site specific tumor. Histologically, CAF is a benign mesenchymal neoplasm characterized by two principal components: bland spindle cells and prominent small to medium-sized vessels with mural hyalinization. The indolent nature of the lesion is underscored by the uniformity of its constituent stromal cells, and their lack of nuclear atypia. Characterization by immunohistochemistry is helpful distinguishing Cellular angiofibroma from other mesenchymal lesions.Case presentationWe report the case of a 37-year-old woman, presenting with a painless nodule involving the vulva. This lesion had gradually increased in size; a simple excision was performed, and follow up was unremarkable. Gross examination showed a well circumscribed, firm tumor measuring 3× 3 × 2,5 cm. Histologically, the tumor was composed of uniform, short spindle-shaped cells, proliferating in an edematous to fibrous stroma and numerous small to medium-sized thick-walled vessels. A panel of immunohistochemical stains was performed, and confirmed the diagnosis of Cellular angiofibroma.ConclusionIn this report we aim to describe the clinical, pathological and immunohistochemical features of this rare entity through a literature review, and to discuss other vulvar mesenchymal lesions.

Cellular Angiofibroma: A Rare Vulvar Tumor Case Report

“…Mesenchymal tumors in the vulvovaginal region are relatively rare, and they can be either non-specific tumors and have a more generalized distribution, such as those arising from smooth muscle (e.g., leiomyoma), vessels (e.g., hemangioma), or neural cells (e.g., granular cell tumors, schwannoma, neurofibroma), or they can have a predisposition to occur in the vulvovaginal region, such as aggressive angiomyxoma, angiomyofibroblastoma, or CA. 4,6 Given such tumoral diversity at this site, differential diagnosis of vulvar masses can be challenging. Most of these lesions present as nonspecific lesions, with similar characteristics, which might lead to a misdiagnosis that may convey a distinct prognosis.…”

“…Histologically, these lesions are characterized by three components: spindle cells that form small fascicles surrounded by collagen bundles, numerous and prominent blood vessels, sometimes with hyalinized wall, and adipose tissue between fusiform cells. [1][2][3][4]6 It shows immunostaining for vimentin, estrogen and progesterone receptors, smooth muscle actin and, in 60% of cases, also for CD34. There is no staining for S100 protein, desmin, keratins or epithelial membrane antigen (EMA).…”

“…There is no staining for S100 protein, desmin, keratins or epithelial membrane antigen (EMA). [1][2][3][4]6,7 The significance of estrogen and progesterone receptors expression is still uncertain. Since these receptors are normally expressed in the lower female genital tract mesenchymal cells, their presence in CA might be only a reflection of its cells origin.…”

“…Since these receptors are normally expressed in the lower female genital tract mesenchymal cells, their presence in CA might be only a reflection of its cells origin. 4,6,7 But, alternatively, some authors suggest that the expression of estrogen and progesterone receptors may play a role in the pathogenesis of the tumor. 4,6,7 Although CA arises more frequently in the vulvovaginal region, there are some cases described in extrapelvic sites.…”

Intratesticular cellular angiofibroma — a rare benign tumour: case report and literature review

“…Cellular angiofibroma is a pathologically and clinically benign tumour, and the treatment of choice is surgical excision [9]. All of such described cases have a good clinical outcome; infiltration or metastatic changes have not been observed [8,11]. Only a single study reporting local recurrence six months after CAF excision has been published [12].…”