Aggressive angiomyxoma: irradiation for recurrent disease

Aggressive angiomyxoma (AAM) is a tumor of the soft tissues predominately occurring in the genital and pelvic area with a strong propensity to local recurrences. The entity was first described in 1983. The tumor is regarded as low-grade sarcoma by some authors; its cause and pathogenesis are presently unknown.

This is a case report on a 27-year-old man who underwent 4 surgical procedures of the left lower extremity because of a recurrent soft tissue neoplasm, initially (August 1993) diagnosed as a myxolipoma. The patient suffered from recurrences in February 1995, September 1996 and February 1998. The diagnosis was revised at the time of the latest recurrence. A palliative resection with macroscopic residuals left was performed in February 1998, followed by a radiation therapy with 56 Gy total dose and a concomitant administration of the radiosensitizer razoxane per os. The single radiation doses were 200 cGy 5 times a week.

The small residuals of the tumor obviously regressed although an objective response could not be shown because the lesion was not clearly measurable. A follow-up 2 years after the radiation treatment revealed no recurrence. The time of the local control achieved as yet is already longer than any former time to regrowth between the surgical procedures. This is, to our knowledge, the first description of a therapeutic irradiation of a recurrent aggressive angiomyxoma.

Radiation therapy combined with the sensitizer razoxane is able to control a recurrent AAM for an unknown time. It remains open whether a radiation treatment alone would have had a similar effect.