Aggressive angiomyxoma: Irradiation for recurrent disease

Background: Aggressive angiomyxoma (AAM) is a tumor of the soft tissues pre dominantely occurring in the genital and pelvic area with a strong propensi ty to local recurrences. The entity was first described in 1983. The tumor is regarded as low-grade sarcoma by some authors; its cause and pathogenesi s are presently unknown. Patient and Method: This is a case report on a 27-year-old man who underwen t 4 surgical procedures of the left lower extremity because of a recurrent soft tissue neoplasm, initially (August 1993) diagnosed as a myxolipoma. Th e patient suffered from recurrences in February 1995, September 1996 and Fe bruary 1998. The diagnosis was revised at the time of the latest recurrence . A palliative resection with macroscopic residuals left was performed in F ebruary 1998, followed by a radiation therapy with 56 Cy total dose and a c oncomitant administration of the radiosensitizer razoxane per os. The singl e radiation doses were 200 cGy 5 times a week. Results: The small residuals of the tumor obviously regressed although an o bjective response could not be shown because the lesion was not clearly mea surable. A follow-up 2 years after the radiation treatment revealed no recu rrence. The time of the local control achieved as yet is already longer tha n any former time to regrowth between the surgical procedures. This is, to our knowlegde, the first description of a therapeutic irradiation of a recu rrent aggressive angiomyxoma. Conclusion: 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.