Endocrine manipulation to treat aggressive angiomyxoma

Kashinath Dixit; Sarah O; Peter Trainer; Georg Brabant; Stephen Shalet

Aggressive angiomyxoma (AA) is a rare mesenchymal tumor which is more common in females, occurring predominantly in the pelvi-perineal region. It tends to be locally aggressive with high recurrence after primary excision. The majority of these tumors are positive for oestrogen and progesterone receptors. Hence hormonal manipulation provides the possibility of effective treatment.

Since 2005, we have been treating two female patients with AA medically (see Table). Both had surgical debulking of large pelvic tumors which were positive for oestrogen and progesterone receptors. They were started on GnRH analogue, goserelin with the addition of tamoxifen to cover the initial period to help counteract the hyperstimulation phase. Subsequently, the aromatase inhibitor, anastrazole was introduced. The endocrine combination resulted in complete suppression of serum oestradiol with severe symptoms of oestrogen deficiency. In one patient the progesterone receptor antagonist, Mifepristone was tried but with no added benefit.

Age at Δ (years)	Surgical treatment	Medical treatment drug dose duration	Outcome
38	Three debulking surgeries	Goserelin 10.8 mg implant every 3/12 9 months and every 6/52 15 months Anastrazole 1 mg OD 23 months Mifepristone 50 mg OD 6 months (stopped)	Tumor size reduced moderately in the first 6 months and has stabilized thereafter
52^a	One debulking surgery	Goserelin 10.8 mg implant every 3/12 27 months Anastrazole 1 mg OD 21 months	Tumor is continuing to shrink
^aPatient in peri-menopausal state with detectable serum oestradiol.
Δ, diagnosis.

This is the first report of the use of this novel combination of GnRH analogue and aromatase inhibitor in the treatment of AA; it constitutes the longest period of successful hormone manipulation therapy for AA, ever described in the literature.

Endocrine Abstracts

P167