Abstract: Intravenous leiomyomatosis (IVL) is a rare kind of uterine myoma. It is a benign smooth muscle tumor with invading growth pattern. The tumor extends into venous channels, but rarely invades tissues. It grows along the refluxing direction of the venous channels, uterine vein, ovarian vein, and beyond the uterus, extends into the inferior vena cava till the right atrium or pulmonary arteries, resulting in intracardiac leiomyomatosis (ICL). At present, the tumor can be detected by ultrasonic waves, computer tomography and magnetic resonance imaging. The main ICL therapy is surgery which is divided into onestage operation and twostage operation in which the key is the complete tumor excision. Most sufferers have a good prognosis, but there are possibilities of recurrence. Missed diagnosis and misdiagnosis are not uncommon, because the disease is rare with hided and diversified clinical manifestations. It is fatal without special characteristics. For a better understanding of ICL, the recent research and treatment of ICL are reviewed.
ObjectiveTo summarize the diagnosis and management of intravenous leiomyomatosis, and to compare effect of the one-stage surgery and two-stage surgery. MethodsClinicopathological data of 18 patients hospitalized in Peking Union Medical College Hospital who were diagnosed as intravenous leiomyomatosis with intracaval and intracardiac extension during Jan. 2002 to Sep. 2013 were collected, and some indexes of the one-stage surgery group and two-stage surgery group were compared, including blood loss, blood transfusion, operation time, period of stay in ICU, hospital stay, and hospitalization expense. ResultsAll the patients were diagnosed as intravenous leiomyomatosis pathologically after operation. Of the 18 patients, 6 (33.3%) patients underwent one-stage surgery and 12 (66.7%) patients underwent two-stage surgery. There were no significant difference on blood loss, blood transfusion, operation time, period of stay in ICU, hospital stay, and hospitalization expense (P > 0.05). There were some patients suffered from complication, including 1 case of pleural effusion, 1 case of recurrent laryngeal nerve injury, 1 case of pulmonary infection in one-stage surgery group; 1 cases of arrhythmia, 2 cases of intestinal obstruction, 2 cases of pleural effusion in two-stage surgery group. No significance had be found in incidence rate of complication between one-stage surgery group and two-stage surgery group (P=1.000). Tumors of 2 patients who underwent two-stage surgery had developed before the second surgery, increasing the difficulty and risk of the second surgery. Three cases of one-stage group were followed-up for 48-63 month (the median time was 62.0 months), 10 cases in two-stage group were followed-up for 1-43 month (the median time was 19.5 months). During the followed-up period, occurrence happened in 1 case of two-stage group, but without death in all cases. ConclusionsBoth one-stage surgery and two-stage surgery are effective and safe. Taking physical and psychological endurance of patients into consideration, one-stage surgery is highly recommended if the patient is in good status and can tolerate the strike brought by the surgery.