Bilateral Salpingo-Oophorectomy for Intracardiac Leiomyomatosis: A Case Report

Patient: Female, 41-year-old Final Diagnosis: Intracardiac leiomyomatosis Symptoms: Abdominal discomfort • menometrorrhagia Medication: — Clinical Procedure: — Specialty: Obstetrics and Gynecology OBJECTIVE: Rare disease BACKGROUND: Intracardiac leiomyomatosis (ICLM) is an extremely rare tumor which is benign but presents with aggressive behavior. To date, there is still no standard of care for ICLM therapy, and treatment for complicated ICLM has obtained even less attention. Radical surgery was usually recommended to remove the patients’ tumors completely. Since initial complete surgical resection cannot be performed in all cases, bilateral salpingo-oophorectomy (BSO), via its effects of estrogen deprivation, may be a feasible primary step in the treatment of premenopausal women with unresectable ICLM. CASE REPORT: We describe a case of a residual mass in the inferior vena cava and right atrium that shrank dramatically after BSO. The patient was a 41-year-old woman with initially unresectable ICLM. Total hysterectomy with BSO and excision of the retroperitoneal mass was performed, but the intracaval tumor above L5 was not removed. Pathology revealed a benign leiomyoma which was strongly positive for both estrogen receptor and progesterone receptor. Two weeks after the BSO, the patient’s serum estradiol level had decreased to a postmenopausal level. At the same time, the proximal end of the intracaval tumor shrank dramatically from the level of the right atrium to the level of L3 only 2 weeks after the surgery. Therefore, this may provide a therapeutic window for a second reduction surgery. CONCLUSIONS: BSO, via its estrogen deprivation effect, may provide a simple but effective initial treatment choice for premenopausal women who suffer from primary unresectable ICLM.