Giant Angiomyofibroblastoma With a Florid Lipomatous Component: A Report and Review of Diagnostic Considerations

Angiomyofibroblastoma is a benign, usually small neoplasm typically constituted by spindle-shaped and epithelioid cells in a vascularized, myxoid-fibrous stromal background. It is most often seen in the superficial genitalia of female patients of reproductive age. However, various clinical and histologic features have been reported, including tumors in male patients, malignant transformation, extragenital sites, huge sizes, and a prominent lipomatous pattern. We report the clinical and pathologic features of one such tumor: a 23.5 cm lipomatous angiomyofibroblastoma of the vulva in a 40-year-old female patient. We also discuss important diagnostic considerations when approaching such large tumors, particularly in the setting of a biopsy specimen.