Background
Intravenous leiomyomatosis is a rare, benign, smooth muscle tumor originating in the uterus that may extend through the inferior vena cava into the heart. Intracardiac leiomyomatosis (ICL), present in 10% of patients with intravenous leiomyomatosis, may cause right heart failure, tricuspid valve obstruction, and pulmonary embolism. The imaging characteristics of ICL continue to be reported. The purposes of this study were to characterize the echocardiographic features of ICL and to correlate the clinical findings.
Methods
Between 1999 and 2012, 12 female patients with suspected ICL underwent cardiac surgery and histologic confirmation of the tumor. The clinical data, echocardiographic findings, and histologic results were retrospectively reviewed.
Results
The ages of the patients with ICL ranged from 40 to 59 years. Ten patients (83%) had undergone myomectomy or hysterectomy, one patient had a uterine fibroid, and one patient had endometriosis. Seven patients (58%) reported dyspnea and/or palpitations, and one patient had syncope; four patients were asymptomatic. Echocardiographic findings included six patients with homogenous right atrial masses, four patients with myxoma-like right atrial masses, and two patients with serpentine, convoluted right atrial masses. In nine patients, the right atrial masses were noted to cross the tricuspid valve. All masses extended from the inferior vena cava. No masses appeared to adhere to the right atrium, right ventricular or pulmonary arterial walls, or tricuspid valve. Tricuspid regurgitation was noted in all patients. No pulmonary emboli were present.
Conclusions
The echocardiographic features of the ICL tumors varied. Tricuspid regurgitation and tumors emerging from the inferior vena cava were seen in all patients. Cardiac symptoms, including dyspnea, palpitations, and syncope, occurred in 67% of patients; the remaining 33% were asymptomatic.
Intravenous leiomyomatosis is the extension of a histologic benign smooth muscle tumor originating from within the uterus into the venous system. The tumor primarily extends through the uterine veins and may extend through the inferior vena cava (IVC) to the right heart chambers and into the main pulmonary artery. Intravenous involvement may coexist with a benign uterine leiomyoma or occur after hysterectomy for leiomyoma. Intracardiac leiomyomatosis (ICL) is a rare and potentially dangerous form of intravenous leiomyomatosis in women. ICL is present in up to 10% of cases of intravenous leiomyoma. ICL was first reported in 1907 during an autopsy. To date, <300 cases have been reported and <100 cases reported with cardiac involvement, mostly as case reports. Patients may remain asymptomatic, even with extensive intravenous tumor extension, until intracardiac growth leads to cardiac complications, pulmonary embolism, or even sudden cardiac death. Because of its rarity, the tumor may be misdiagnosed or diagnosed in the late stages; the correct diagnosis relies on a high index of suspicion. Echocardiography is a noninvasive technology capable of diagnosing and assessing the characteristics of such a cardiac mass. The imaging characteristics of ICL are diverse and often not considered or diagnosed before surgery. The purpose of this study was to summarize in detail the echocardiography features of ICL and to prioritize the clinical findings that may lead to its early detection.
Methods
The time period of retrospective review was from January 1999 to January 2012. During this time, 557,485 patients underwent transthoracic echocardiography performed by a cardiologist at Beijing Anzhen Hospital (Beijing, China). ICL was identified by histologic examination in 12 female patients who underwent cardiac surgery during this time. The 12 patients were retrospectively reviewed. Data were obtained from medical and pathologic records, including age, symptoms on presentation, medical history, echocardiographic findings, and histologic results. The study protocol was approved by the review board of Beijing Anzhen Hospital. Transthoracic echocardiographic examinations were performed on each of the patients when indicated by their cardiac symptoms or their histologic diagnoses using either GE Healthcare (Milwaukee, WI) or Philips Medical Systems (Andover, MA) echocardiographic equipment. Echocardiographic measurements of the right atrial and right ventricular dimensions and estimates of right ventricular systolic pressures were made according to the guidelines and recommendations of the American Society of Echocardiography.
Results
From 1999 to 2012, 557,485 patients underwent transthoracic echocardiographic studies. Intravenous leiomyomatosis with intracardiac extension was identified after cardiac surgery in 12 female patients, an incidence of 0.0022%. The clinical data and echocardiographic findings of the 12 patients are listed in Table 1 . All patients had histologic confirmation of their disease. The age range of the patients was 40 to 59 years (mean age, 48 ± 6 years). Clinical cardiac symptoms included seven patients with dyspnea and/or palpitations and one patient with syncope; four patients were asymptomatic. Gynecologic and surgical histories included 10 patients (83%) who had previously undergone myomectomy or hysterectomy. One patient had been diagnosed with a uterine fibroid and one patient with endometriosis. The mean duration between the gynecologic or surgical diagnosis and the diagnosis of ICL was 6.5 years.
| Patients | Age (y) | History | Presenting symptom | Dilated cardiac chambers | Echocardiographic features |
|---|---|---|---|---|---|
| 1 | 40 | Hysterectomy | Dyspnea | RA | Solid mass |
| 2 | 41 | Hysterectomy | Dyspnea | RA | Large solid mass in the RA |
| 3 | 42 | Hysterectomy | Dyspnea | RA, RV | Elongated serpentine convoluted mass ( Figure 4 ; Video 3 ; available at www.onlinejase.com ) |
| 4 | 43 | Hysterectomy | Palpitation | RA | Solid mass |
| 5 | 45 | Myomectomy | Palpitation | RA, RV | Myxoma-like mass |
| 6 | 46 | Uterine fibroid | None | RA, RV | Elongated serpentine convoluted mass |
| 7 | 48 | Hysterectomy | None | RA, RV | Solid mass ( Figures 2, 5, 6, and 7 ; Videos 1 and 4 ; available at www.onlinejase.com ) |
| 8 | 51 | Adenomyosis | Dyspnea | RA | Myxoma-like mass |
| 9 | 52 | Hysterectomy | Syncope | RA, RV | Myxoma-like mass ( Figure 3 ; Video 2 ; available at www.onlinejase.com ) |
| 10 | 53 | Hysterectomy | None | RA | Solid mass |
| 11 | 56 | Hysterectomy | None | RA, RV | Myxoma-like mass |
| 12 | 59 | Myomectomy | Palpitation | RA | Large solid mass in the RA ( Figure 1 ) |
The echocardiographic findings of each patient are listed in Table 1 . In all 12 patients, masses were identified in and emerging from the IVC into the right atrium ( Figures 1–3 ; Videos 1 and 2 ; available at www.onlinejase.com ). The masses crossed the tricuspid valve into the right ventricle in six patients ( Figures 4 and 5 ; Videos 3 and 4 ; available at www.onlinejase.com ). The masses were characterized as solid (homogenous) (six patients; Figure 1 ), lobular or myxoma like (four patients; Figure 5 ; Video 4 ; available at www.onlinejase.com ), or elongated and serpentine (two patients; Figure 4 ; Video 3 ; available at www.onlinejase.com ). None of the masses appeared to be adhered or attached to the right atrial or right ventricular walls or to the tricuspid valve. Tricuspid regurgitation was reported in all patients. Nine patients had mild tricuspid regurgitation, and three patients had severe tricuspid regurgitation. A leiomyomatous tumor specimen is shown in Figure 6 (patient 7) after surgical removal from the left iliac vein, IVC, and right atrium; histologic confirmation is shown in Figure 7 . The ease with which tumors were removed surgically intact appeared to confirm that they were not adhered or attached to the IVC or the walls of the cardiac chambers. No evidence of pulmonary embolism was documented in any of the cases.
