Intravenous Leiomyomatosis with Right Heart Involvement—A Report of 4 Cases and Literature Review

Intravenous leiomyomatosis（IVL） is a rare benign neoplasm which originates from the smooth muscle cells and is usually confined to the pelvic venous system.Rarely,intracaval and intracardiac extension has been described.Death can occur as a result of intracardiac involvement.We reported 4 cases of IVL with right heart involvement（intracardiac leiomyomatosis,ICL）.Three of them suffered recurrent sudden syncope,and the other one was totally asymptomatic.All of them were successfully treated through one-stage operation under extracorporeal circulation.

Diagnosis and surgical treatment of intraveneous leiomyomatosis extending into the heart:two cases report and review of the literature

Objective： To investigate the clinical characteristics, diagnosis and surgical treatment of intravenous leiomyomatosis （IVL）, and outline the differences between Chinese and overseas cases. Methods： Clinical data of two IVL cases, treated in our hospital, were analyzed retrospectively and the related literature was also reviewed. The data of preoperative diagnostic rate, surgical procedures, and postoperative recurrence between patients in China and other countries were compared. Results： The first stage operation was performed successfully in 2 patients. However, they refused subsequent therapies, including a second stage operation to excise the remnants Of the tumor, uterus, bilateral oviducts and ovaries, and anti-estrogen therapy. Both suffered from IVL recurrence, one at 6-month and the other at 9-month, and died at 16-month and 12-month respectively after the first stage surgery. Worldwide reports of 110 IVL cases were reviewed, which included 28 cases in China and 82 cases in other countries. In the majority of the Chinese patients, tumors only extended into the fight atrium rather than the right ventricle （RA 22 cases vs RV 4 cases）. However, among the overseas patients, the rate of extension into the right atrium was similar to that of extension into the right ventricle （RA 41 cases vs RV 38 cases）. The rate of hysterectomies was not significantly different between Chinese and overseas patients （ 67.86% vs 55.9%, P=0.278）. The rate of correct preoperative diagnosis in the Chinese patients was significantly lower than that in the overseas patients （32.14% vs 80%, P=0.000002.）, as well asthe rate of complete excision of the tumor （22.7@0 vs 75.5%, P=0.000001）. The proportion of patients who undergoing a single-stage or a two-stage operation was similar in Chinese and overseas patients. The recurrence rate was significantly higher in the Chinese patients than in the overseas patients （36.8% vs 9.1%, P=0.0055）, and the patients with tumor recurrence were partial tumor excision patients. Conclusion： The possibility of IVL should be considered if echocardiography in female patients demonstrates a tumor in the right heart and a mass in the inferior vena cava （IVC）. Further imaging should be performed to confirm the diagnosis. The correct diagnosis and accurate preoperative delineation of tumor extension are essential for an optimal surgical outcome. The key point in IVL treatment is the complete excision of tumors （single-stage or two-stage surgical procedure）.