Massive pelvic recurrence of uterine leiomyomatosis with intracaval-intracardiac extension: video case report and literature review

Abstract

Background: Uterine leiomyomas represent the gynecological neoplasm with the highest prevalence worldwide. This apparently benign pathological entity may permeate into the venous system causing the so-called intravenous leiomyomatosis of the uterus (IVL). IVL may seldom extend to large caliber veins and reach the right cardiac chambers or pulmonary arteries and cause signs of right sided congestive heart failure and sudden death. Due to its low incidence, however, IVL with intracardiac extension is often misdiagnosed resulting in deferred treatment. No consensus has been obtained regarding the standard surgical approach to be used for this rare condition. We describe the case of a massive pelvic recurrence of uterine leiomyomatosis with intracardiac extension and provide a review of the literature, analyzing management and surgical outcomes.

Case presentation: We present the case of a 46-year-old premenopausal woman presenting with lower-extremity edema, recurrent syncopes and a history of subtotal hysterectomy for multiple uterine fibroids. She was diagnosed with pelvic recurrence of uterine leiomyomatosis and IVL with cardiac involvement. A two-stage surgical excision of the intracardiac-intracaval mass and pelvic leiomyomatosis was performed. The patient had an uneventful recovery and no evidence of recurrence was observed on follow-up.

Conclusions: By virtue of the rarity of the present pathology, awareness is widely scarce and diagnosis is often delayed. Early recognition is difficult due to initial aspecific and subtle clinical manifestations. Nevertheless, suspicion should be held high in premenopausal women with known history of uterine leiomyomata, presenting with cardiovascular symptoms and evidence of a free-floating mass within the right cardiac chambers. In-depth imaging is crucial for defining its anatomical origin and relations. Prompt surgical treatment with radical excision of pelvic and intravenous leiomyomatosis guarantees favorable outcomes and excellent prognosis with low rates of recurrence, whereas delayed diagnosis and treatment exposes to increased risk of congestive heart failure and sudden death.

Keywords: Cardiopulmonary bypass; Hysterectomy; Intracardiac leiomyomatosis; Intravenous leiomyomatosis; Uterine leiomyoma.

Fig. 1

Transthoracic two-dimensional echocardiography revealing a fluctuating solid mass (asterisk) within the right atrium (RA) (5 × 3.5 cm), ascending from the inferior vena cava Right ventricle (RV); left ventricle (RL); left atrium (LA)

Fig. 2

Cardiac CT scan showing a hypodense mass occupying the right atrium (a) and the inferior vena cava in toto (b)

Fig. 3

Chest – abdomen – pelvis CT scan (a-d) and MR (e). a. Axial projection showing a voluminous pelvic mass of 15 × 22 × 9 cm (asterisk), with significant contrast enhancement due to hypervascularization, causing right antero-lateral dislocation of the bladder (arrow). b. The mass also determines left hydro-ureteronephrosis (white arrow). Notice the presence of contrast-enhanced large vessels (black arrows) within the IVC (arrowhead). c-d. Coronal and sagittal CT projections showing the pelvic tumor directly extending into the left iliac vein (white arrow), IVC (black arrows) and occupying the right atrium (arrowhead). e. Coronal MR projection showing the caval and cardiac extension of the tumor

Fig. 4

Arteriogram showing (a) bilateral hypogastric artery dilation, with (b) marked left-sided hypogastric artery prominence and tumor hypervascularization

Fig. 5

Gross specimen. a Intracardiac-intracaval mass measuring 16 × 4 × 3 cm with transection line at infrarenal level (arrow) and lower transection at supra-iliac level. b Iliocaval confluence and left iliac vein mass of 8 cm. c. Pelvic leiomyomatous mass measuring 15 × 22 × 9 cm. d. Cervix (6 × 3.5 × 2 cm)

Fig. 6

Clinical case timeline