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Introduction: Alport syndrome with diffuse pelvic leiomyomatosis is a complex combination for which the course of treatment is not well described. This case describes the outcomes of a trial with gonadotropin-releasing hormone analogues as an alternative to extensive surgery. Case Report: A 47-year-old female patient, known with Alport syndrome associated with diffuse leiomyomatosis, was referred to our gynecological service because of a pelvic mass. The patient complained of obstipation and pain in the right hypochondrium. Magnetic resonance imaging showed a diffuse multi-lobulated leiomyomatosis from the vulvar to the perineal area. As the removal of the mass would imply very extensive surgery, conservative treatment options were searched for, in analogy with the medical treatment of uterine leiomyomas. A few trials used gonadotropin-releasing hormone analogues in the treatment of diffuse pelvic leiomyomatosis but follow-up data of these articles are lacking. For this patient, who was close to menopausal age, a trial therapy with gonadotropin-releasing hormone analogues was followed by an adnexectomy for total hormonal suppression. The patient’s follow-up showed stabilization of the mass and decrease of the pain complaints. Conclusion: This case demonstrates that hormonal suppression can be a successful alternative to complex surgery in a case combining Alport syndrome and diffuse pelvic leiomyomatosis. |
