- Rare tumor, usually adolescent males (10-25 years), rarely in older patients or women (may be misdiagnoses)
- 75% have androgen receptors, but not estrogen or progesterone receptors
- Arises from erectile-like fibrovascular stroma in posterolateral wall of roof of nose
- May grow into nasopharynx, orbit or cranial cavity
- May regress after puberty, especially after incomplete surgical excision or radiation therapy
- Benign but recurs in 40%, usually within 1 year, particularly if not completely removed
- Rare sarcomatous transformation after radiation therapy
Treatment
- Surgery (difficult to excise), preoperative embolization or anti-androgen therapy
- Chemotherapy or radiation therapy if advanced or aggressive
Gross description
Well circumscribed but unencapsulated polypoid fibrous mass, bleeds severely on manipulation and biopsy, may occlude nares
Spongy cut surface
Micro description
- Intricate mixture of stellate and staghorn blood vessels with variable vessel wall thickness ranging from single layer of endothelium to variable smooth muscle coat
- Irregular fibrous stroma (loose, edematous to dense, acellular)
- Stromal cells are stellate fibroblasts with small pyknotic to large vesicular nuclei
- Larger vessels at base of lesion, smaller vessels with plump endothelial cells at growing edge of tumor
- Multinucleated stromal cells are common
- Mitotic figures are rare
- Minimal inflammation
