下大静脈/右心系へ進展した静脈内平滑筋腫症の二期的手術の1例

Two-stage Surgery for 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）は子宮に好発するまれな平滑筋腫であり，静脈血管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．しかしながら，まれに骨盤内から腸骨静脈，卵巣静脈，腎静脈を経由して連続性に下大静脈から心腔内へ進展し，三尖弁嵌頓や肺塞栓などの致死的合併症を起こす危険がある1～3）．われわれは，巨大な子宮原発巣から複数の静脈経路で下大静脈/右心系へ進展した静脈内平滑筋腫症に対し，二期的手術を行ったので報告する．

Intravenous leiomyomatosis is a rare neoplastic condition characterized by the benign intravascular proliferation of smooth muscle cells originating from either the uterine venous wall or a uterine leiomyoma. In the present report, we describe the case of a 36-year-old woman, who was referred to our institution due to abdominal pain. Computed tomography indicated the presence of a giant intravenous leiomyoma originating from the uterus and extending to the right ventricle with complex pathways. The patient was successfully treated by tumor resection under circulatory arrest in two-stage operations in conjunction with gonadotrophin-releasing hormone agonists therapy. She continued to recover and did not exhibit any recurrence at the 8-month follow-up.