CONTEXT: Cellular angiofibroma of the vulva is a rare tumor that was first described in 1997. It occurs in middle-aged women (average age: 47 years), has small size (< 3 cm) and well-circumscribed margins. CASE REPORT: We describe a case in a 51-year-old woman whose preoperative diagnosis was confounded with Bartholin's glandular cyst. The neoplasia was well delimited and made up of three characteristic components: fusiform cells forming small fascicles, numerous blood vessels and adipose tissue interspersed between the fusiform cells. The stroma cells were positive for vimentin and negative for CD34, protein S-100, actin and desmin. The differential diagnoses for this tumor include aggressive angiomyxoma, angiomyofibroblastoma, lipoma of fusiform cells, solitary fibrous tumor, perineurioma and leiomyoma.

Cellular angiofibroma of the vulva with DNA ploidy analysis. Int J Gynecol Pathol. 2001;20(2):200-3.

Cellular angiofibroma (CAF) is a recently described rare soft tissue neoplasm of the vulva (with only four reported cases) that typically occurs as a well-circumscribed solid rubbery vulvar mass in middle-aged women. The distinct histologic features of bland spindle cells admixed with numerous hyalinized medium to small blood vessels, and a vimentin-positive desmin-negative immunoprofile differentiates this neoplasm from other vulvar tumors such as angiomyofibroblastoma and aggressive angiomyxoma. In this report an additional case of CAF is presented with DNA ploidy analysis and CD99 immunohistochemistry.

Extragenital subcutaneous cellular angiofibroma. Case report.APMIS. 2007 Mar;115(3):254-8.

Cellular angiofibroma (CAF) is a rare distinctive mesenchymal neoplasm that occurs almost exclusively in the genital area. We report the case of a 38-year-old woman who presented with an asymptomatic subcutaneous mass, 3.5 cm in diameter, located in the left hypochondrium, which had progressively enlarged during the previous 6 months. The lesion was completely excised. No recurrence was observed 3 months after the excision. A review of the literature--and including the present report--revealed five cases (three men and two women) with location outside the urogenital tract. Mean age was 57 (range 38-78) years; mean 41 years for women and 68 years for men. Average tumor size was 9 (range 3.5-25) cm; mean 5 cm for women and 12 cm for men. Tumors were located in the superficial soft tissue of the trunk, except for one case in the retroperitoneum. Mean follow-up was 29 (range 3-102) months, and no patient developed recurrence or metastasis. Extragenital CAF, except for location, shows similar clinicopathological features to genital CAF. Simple tumorectomy appears to be adequate treatment. Morphologically, CAF is closely related to spindle cell lipoma.