Experience in the diagnosis and treatment of intravenous leiomyomatosis involving the inferior vena cava and/or right cardiac chambers.

ABSTRACT

OBJECTIVE: This study aimed to summarize our experience with the diagnosis and treatment of intravenous leiomyomatosis (IVL) involving the inferior vena cava (IVC) or right cardiac chambers. METHODS: This study retrospectively analyzed clinical data from 10 patients diagnosed with IVL involving the IVC or right cardiac chambers between May 2009 and October 2019 at one medical center. RESULTS: All patients were females aged 35 to 56 years (average, 46.8 years) with a history of uterine leiomyoma. Of these 10 patients, 8 manifested clinical symptoms and 2 were asymptomatic. Four were diagnosed with lesions involving the right cardiac chambers, four had lesions that extended into the suprahepatic IVC, and an additional two had lesions extending into the infrarenal IVC. All patients underwent surgery. Three of the four patients with extension into the right cardiac chambers underwent a two-stage operation, and an additional patient was managed with a one-stage operation. Patients who underwent a two-stage operation experienced less hemorrhaging and a shorter intensive care unit stay than the patient who underwent a one-stage operation. Six patients with intracaval extension alone underwent laparotomy, including four with a lesion extending into the suprahepatic IVC, under transesophageal echocardiography monitoring. Bilateral adnexectomy and ovariectomy were performed in seven patients, and unilateral adnexectomy and ovariectomy were performed in two patients; antiestrogen therapy was administered to two patients who retained a unilateral ovary and to one patient who retained bilateral ovaries. One patient suffered deep vein thrombosis in the left lower extremity after surgery that improved after treatment. All patients received conventional anticoagulant treatment postoperatively. All pathologic findings confirmed IVL, and the follow-up period ranged from 27 to 120 months (average, 57.5 months). Recurrence was not observed in the iliac vein or IVC, excluding one case of pelvic leiomyoma that recurred at one year postoperatively. CONCLUSIONS: IVL should be highly suspected when an IVC mass occurs in a patient with a history of uterine leiomyoma. Surgery is the gold standard treatment for IVL; a two-stage operation is more beneficial for patient recovery if the lesion exhibits intracardiac involvement, and transesophageal echocardiography is a helpful tool to monitor safety during surgical procedure for patients with a lesion invading the IVC above the level of the renal vein.