Management of intravenous leiomyomatosis with intracaval and intracardiac extension

Abstract

Objective: To report the results of management of intravenous leiomyomatosis with intracaval and intracardiac extension at Peking Union Medial College Hospital.

Methods: We reviewed a cohort of 20 patients with intravenous leiomyomatosis extending to the inferior vena cava and heart, focusing on the clinical characteristics, the results of surgical management, and prognosis.

Results: The mean age of the patients was 42.4 ± 7.0 years. The clinical manifestations of intravenous leiomyomatosis are various and nonspecific, including pelvic mass, chest tightness and shortness of breath, swelling in the lower extremity, abdominal distension, palpitation, syncope, hypermenorrhea, and skelalgia. All the patients had history of uterine leiomyoma and 16 patients (80%) had undergone uterine leiomyoma operation. After careful preoperative evaluation, nine patients underwent one-stage operations (cardiac surgery, vascular and gynecologic surgery together) and 11 patients underwent two-stage operations (cardiac surgery first, then vascular and gynecologic surgery). There was no significant difference in the postoperative complication rate (33.3% compared with 27.3%; P>.99) between one-stage and two-stage operations. All operations were performed without severe surgical-related complications or death. Approximately 78% of patients had complete resection of tumor and 22.2% of the patients experienced incomplete resection. Eleven (55%) patients received hormone therapy postoperatively. During mean follow-up time of 20.5 months, recurrence occurred in five patients (27.8%) but all the patients survived.

Conclusions: Precise and full-scale preoperative evaluation, complete tumor resection, and multidisciplinary cooperation are crucial for successful treatment.