右室に進展した血管内平滑筋腫の1例

Uterine Intravenous Leiomyomatosis with Right Ventricular Extension：Report of a Case

はじめに　静脈内平滑筋腫症（intravenous leiomyomatosis：IVL）は子宮に好発するまれな平滑筋腫である．静脈腔内での平滑筋細胞の増殖を特徴とし，組織学的には良性腫瘍に分類されるが，腸骨静脈，卵巣静脈，腎静脈を経由して下大静脈（inferior vena cava：IVC）から右房，右室，肺動脈に進展することがある1～3）．われわれは子宮平滑筋腫症手術の11年後に局所再発腫瘍が右室へ進展し，三尖弁嵌頓のため急性右心不全を呈したIVL例に対し，二期的手術を行い救命したので報告する．

A 63-year-old female patient was emergently admitted to our hospital with complaints of dyspnea and leg edema. She had a history of abdominal total hysterectomy and bilateral salpingo-oophorectomy for uterine leiomyoma 11 years ago. Computed tomography （CT） and echocardiography revealed a local recurrent tumor on the right side of the inferior vena cava in the retroperitoneal cavity, which extended to the right ovarian vein, inferior vena cava, and right atrium/ventricle. The patient suffered shock due to the tumor lodging in the tricuspid valve, so we performed an emergency intracardiac tumor resection under cardiopulmonary bypass, and two months later, we removed the locally recurrent tumor and the intravascular tumor below the diaphragm. The tumor in the inferior vena cava was removed entirely under hypothermic circulatory arrest. In cases such as this one, which is associated with hemodynamic instability before surgery, it is desirable to aim for a complete resection with a two-stage surgery.