Most of the lesion consists of bland plump spindled cells in a haphazard distribution with a modest amount of fibrosis.

The second component consists of numerous irregular shaped and sometimes dilated vessels. The vessels contain a variable amount of smooth muscle in their walls.

Occurs exclusively in males (mean age of 15 years). Redheads with fair-skin more commonly affected. If found in a female, then, she is probably a male with testicular feminization. Very rare (less than 1% of nasopharyngeal tumors) and located in the posterolateral wall of the roof of the nose. Presents with nasal obstruction and epistaxis.

Excision and preoperative embolization (creating an embolus to block a feeder vessel of the neoplasm). Among different surgical techniques, the lowest recurrence rate has been reported with either a) the transpalatal approach when the tumor is limited to the nasopharynx with extension to the nasal cavity or paranasal sinuses, or b) the Lefort I approach when skull base invasion is present (Hosseini). Hormonal therapy is also considered (stromal cells for this tumor are androgen-receptor positive). Radiotherapy is used for intracranial tumors in unresectable areas.

Benign, but it can be locally aggressive and cause significant morbidity and mortality due to intracranial extension. Higher tumor stage lesions are more likely to recur. Tumor size appears to be important as well, as recurrence rates with tumor size < 4 cm and >/= 4 cm have been reported as 21.7% and 54.9%, respectively (Sun). 9% mortality due to hemorrhage or intracranial extension.