Intravenous Leiomyomatosis Complicated by Arteriovenous Fistula: Case Series and Literature Review

Abstract

BackgroundUterine intravenous leiomyomatosis (IVL), a rare type of uterine leiomyoma, is defined by the intravascular proliferation of a histologically benign smooth muscle cell tumor. Pelvic arteriovenous fistula (AVF) is a rare vascular malformation that is most commonly congenital, post-traumatic, or iatrogenic. The link between leiomyomatosis and AVF has received little attention in the medical literature.ResultsWe provide a case series of seven patients, four of whom were from our center, who had IVL complicated by a pelvic AVF. The symptoms of right heart failure were noted as swelling in the abdomen and two legs as well as a significant amount of ascites. Coil embolization of AVFs may be beneficial in minimizing bleeding during IVL surgery. A review of all accessible literature published on IVLs from 2000 to 2020 was conducted, and data were retrieved from 78 papers totaling 262 cases. Complications and recurrence were associated with pelvic mass excision and intravascular remnant tumor, respectively.ConclusionIntravenous leiomyomatosis combined with AVF aggravates congestion symptoms of surrounding organs. It is worth noting the uncommon combination of AVF and IVL, stressing the importance of a thorough assessment and surgical approach in IVL treatment.

Intravenous leiomyomatosis of the uterus: Preoperative and intraoperative assessment

“…Leiomyomas involve the lumen of the vein and grow along the venous system, which can lead to serious complications such as arteriovenous fistula, congestive heart failure, pulmonary embolism, or sudden death. [1][2][3] Intravenous leiomyomatosis has an occult onset and nonspecific clinical manifestations. When the lesions are confined to the uterus, some patients do not have any clinical symptoms or specific serologic tumor markers, leading to difficulties in early diagnosis and a high rate of missed diagnoses.…”

“…It is classified as a histologically benign disease, despite its aggressive clinical behavior. Leiomyomas involve the lumen of the vein and grow along the venous system, which can lead to serious complications such as arteriovenous fistula, congestive heart failure, pulmonary embolism, or sudden death 1–3 …”