• Omar F Altal, Shireen Rawashdeh, Sarah Al Sharie, Yazan O Al Zu'bi, Ahmed H Al Sharie, Majd N Daoud, and Khaled M Alkhawaldeh.
• Department of Obstetrics & Gynecology, Faculty of Medicine, Jordan University of Science and Technology, King Abdullah University Hospital, Irbid, Jordan.
• Medicine (Baltimore). 2022 Sep 9; 101 (36): e30125e30125.

RationaleCellular angiofibroma (CA) is a rare tumor of the soft tissue classified as a benign fibroblastic/myofibroblastic tumor. Considering this, the literature regarding CA mainly, but not exclusively, comprises single case reports and case series. Here, we report a case of giant CA of the vulva with comprehensive literature review.Patient ConcernsWe present a case of a massive vulvar CA arising in 53-year-old woman with no notable medical or surgical history. The mass has grown considerably over time, causing pain and difficult urination, defecation, and movement. The patient had normal regular menstrual cycle with no previous contraception use. Vaginal examination exposed a right-sided large tender vulvar mass with normal-looking vagina.DiagnosesPelvic magnetic resonance imaging with contrast revealed a large right vulvar heterogeneously enhancing soft tissue mass measuring 13.1 × 10.9 × 10.7 cm expending the left vulva, with internal and peripheral voids resembling feeding vessels. The mass was surgically removed, and subsequent histopathology showed skin-covered dermal-based lesion composed of fibroblast-like bland and spindle cell proliferation with thin-walled blood vessels of various sizes. Immunohistostaining of CD34 and smooth muscle antigen were both positive, while desmin was found to be negative. A diagnosis of vulvar angiofibroma was made based on the clinical scenario, imaging, and histopathology.InterventionsMass vulvectomy was performed starting with a circumferential incision at the base of the mass and structural dissection to separate the mass from the vulvar wall. The incision was successfully closed, and subcuticular stitches were applied to the skin.OutcomesThe patient's complaints were significantly relieved with no postoperative complications and the patient is being followed regularly in an outpatient setting.LessonsDue to its extremely benign nature of CA, and the implausible ability of its recurrence, it was decided to surgically excise it. Despite its rarity, it can be readily identified at its earlier stages preventing the vexing and exasperating symptoms accompanied with increased size as mentioned.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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