Intravenous leiomyomatosis: Case series and review of the literature

Abstract

Background: We retrospectively reviewed the data of three patients with intravenous leiomyomatosis (IVL), one of whom had intracardiac leiomyomatosis, and analyzed their clinical symptoms, preoperative assessment findings, operative approaches, and recurrence.

Case presentation: The present study describes three cases of IVL extending into the inferior vena cava, even as far as the right atrium and ventricle, and discusses the imaging findings and differential diagnosis of this tumor entity. The three patients, two of whom were diagnosed during the first operation and one of whom had a giant pelvic mass, were surgically treated with complete tumor resection, hysterectomy, and bilateral salpingo-oophorectomy. The pathological examination findings were suggestive of IVL. The duration of time from the first myomectomy or hysterectomy to IVL occurrence ranged from 2 to 18 months. No signs of recurrence were observed during follow-up. Computed tomography and magnetic resonance imaging played a vital role in the diagnostic process and presurgical assessment.

Conclusion: In clinical practice, IVL should be considered before surgery for a broad ligament myoma or giant pelvic mass. Surgery should always aim for complete tumor excision and include hysterectomy and bilateral salpingo-oophorectomy. Vascular reconstruction computed tomography is a good choice for diagnosis and follow-up.

Keywords: Intravenous leiomyomatosis (IVL); bilateral salpingo-oophorectomy; broad ligament leiomyoma; giant pelvic mass; hysterectomy; intracardiac leiomyomatosis (ICL).

Figure 1.

Computed tomography findings of Case 1. An axial image of an abdominal and pelvic computed tomography scan at the level of the superior vena cava is shown. An intravascular tumor was seen in the vena cava (black arrow).

Figure 2.

Computed tomography and pathological findings of Case 2. (a) Axial image of a contrast-enhanced abdominal and pelvic computed tomography scan at the level of the left renal vein. An intravascular tumor was observed within the inferior vena cava (black arrow). (b) Hematoxylin–eosin staining of an intravenous leiomyoma thrombus.

Figure 3.

Computed tomography and gross findings of Case 3. (a) Vascular reconstruction computed tomography showed a leiomyoma located in the inferior vena cava (white arrow). (b) Surgically removed leiomyoma that had been located in the inferior vena cava.