Deep (Aggressive) Angiomyxoma
🔑•Young women, pelvis/perineum
•Large, deep-seated mass (often >10 cm)
•Locally infiltrative (despite “benign” cytology)
•High recurrence rate, rare metastasis
🔬 Morphology
•Hypocellular spindle–stellate cells and abundant myxoid stroma
•Haphazard vessels: Thin-walled capillaries and thick-walled vessels (key clue)
•Entrapment of fat, nerves, adnexa → very important
•No atypia / mitosis / necrosis
🧪 IHC profile
•Positive: Desmin, SMA, ER / PR ⭐ (very helpful clue), CD34 variable/focal
•Negative: S100 , DOG1 , CD117
🧬 Molecular: Often HMGA2 rearrangement (12q15)
🔍 Differential diagnosis
1️⃣ Angiomyofibroblastoma
•Superficial, small, well-circumscribed. Alternating hypercellular & hypocellular areas.
•Plump stromal cells clustering around vessels. No infiltration/entrapment
•Desmin , ER , but location circumscription key
2️⃣ Cellular angiofibroma
✔️ Cellular hyalinized vessels CD34 strong
❌ Lacks diffuse myxoid stroma & infiltration
3️⃣ Myxoid neurofibroma
✔️ S100 wavy nuclei
❌ No desmin/ER pattern, no vessel spectrum
💎 Diagnostic pearls:
•Deep pelvic/perineal mass
•Hypocellular myxoid lesion
•Thick thin vessels
•Entrapment of fat/nerves
•Desmin ER/PR
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