[A case report of intravenous leiomyomatosis of the uterus with extension into the right pulmonary artery].

Intravenous leiomyomatosis (IVL) is a rare neoplastic disease which is defined as the extension into venous channels of histologically benign smooth muscle tumors arising either from a uterine myoma or from the walls of a uterine vessel. We report a case of IVL originating from the uterus, growing up in the inferior vena cava, and extending into the right pulmonary artery. The patient was a 42-year-old woman, who was admitted to our hospital with clinical signs of right-side heart failure and syncope in December 1987. Her medical history included a total hysterectomy in August 1986. On examination, two dimensional echocardiography showed a freely floating tumor in the right atrium and right ventricle, which protruded via the inferior vena cava. Cavography confirmed the above findings. Because of the clinical disability caused by the tumor localized in the right heart, emergency operation was performed, extracorporeal circulation was instituted through the femoral vein, the superior vena cava, and the femoral artery. Opening the right atrium confirmed that a white sausage-like mass quite free from the wall extended into the right ventricle, and the head of the tumor was adherent to the right pulmonary artery. After detaching the head of the tumor from the right pulmonary artery, the remaining tumor in the inferior vena cava was also successfully pulled out. The operative specimen was measured 55 cm in length, but the basal end of the tumor was supposed to be left in place. Two months later, the second-stage operation for the remaining tumor was carried out through a median laparotomy.(ABSTRACT TRUNCATED AT 250 WORDS)