General
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• Benign vulvar and vaginal tumor, first described in 1992 (Am J Surg Pathol 1992;16:373)
• No reported recurrences after excision

Epidemiology
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• Females of reproductive age (10% of patients are postmenopausal)
• Very rarely in males (scrotum, paratesticular tissue)

Sites
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• Vagina (10-15% of cases)
• Vulva (majority of cases)

Clinical features
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• Presents as slowly enlarging and painless mass
• May be mistaken for Bartholin's cyst or genital prolapse

Prognostic factors
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• Benign, rarely recurs when marginally excised
• Important to rule out angiomyxomatous or sarcomatous foci in rare cases

Case reports
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• 80 year old woman with angiomyofibroblastoma of the vagina and postmenopausal breast cancer treated with tamoxifen (Int J Gynecol Cancer 2006;16:581)
• 80 year old woman with angiomyofibroblastoma of the vulva with sarcomatous transformation (Am J Surg Pathol 1997;21:1104)

Treatment
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• Local excision

Gross description
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• Well circumscribed, soft, 0.5 to 12.0 cm, without necrosis
• May have pseudocapsule

Micro description
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• Circumscribed tumor with alternating hypercellular and hypocellular areas, spindle cells and plump stromal cells with eosinophilic cytoplasm that aggregate around small blood vessels
• May have binucleated or multinucleated cells
• Mast cells common
• Minimal stromal mucin, rare/no red blood cell extravasation, no atypia
• Rare/no mitotic figures

Micro images
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Zones of hypo and hypercellularity

Round to oval, plump spindle cells

Cytology description
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• Tumor cells with epithelioid or plasmacytoid features, may be binucleated or multinucleated

Positive stains
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• Vimentin, desmin (may be negative in postmenopausal patients), ER, PR

Negative stains
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• Actin (may have focal positivity), keratin, S100

Electron microscopy description
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• Well developed rough endoplasmic reticulum, Golgi apparatus, intermediate filaments, pinocytotic vesicles

Differential diagnosis
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• Angiomyxoma: not circumscribed, less cellular, fewer vessels; stromal mucin, RBC extravasation, infrequent plump stromal cells, no hyalinized vessels
• Cellular angiofibroma: hyalinized vessels, infrequent epithelioid or plasmacytoid nuclear features, more cellular, more fibrous stroma
• Fibroepithelial stromal polyp of vagina: no uninvolved Grenz zone; cells extend to dermal-epidermal junction (Int J Gynecol Pathol 2005;24:26)

Additional references
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• Fletcher: WHO Classification of Tumours of Soft Tissue and Bone (2013), Mod Pathol 1996;9:284

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