We report a case of successful one-stage resection of intravenous leiomyomatosis (IVL) with extension into the main pulmonary artery. The patient was a 50-year-old woman, who was admitted to our hospital with clinical signs of syncope. Computed tomography (CT) and 3 D helical CT images showed a tumor arising in the left side of the uterus with extension into the pulmonary outflow tract. One-stage radical operation with cardiopulmonary bypass was performed. Because IVL is related to many fields concerning various organs, it is important that general surgeons, gynecologists and cardiovascular surgeons cooperate with each other.

Uterine tumors are the most common type of gynecologic neoplasm. Uterine leiomyosarcoma (LMS) is rare, accounting for 2% to 5% of tumors of the uterine body. Uterine LMS develops more often in the muscle tissue layer of the uterine body than in the uterine cervix. The development of gynecologic tumors is often correlated with female hormone secretion; however, the development of uterine LMS is not substantially correlated with hormonal conditions, and the risk factors are not yet known. Radiographic evaluation combined with PET/CT can be useless in the diagnosis and surveillance of uterine LMS. Importantly, a diagnostic biomarker, which distinguishes malignant LMS and benign tumor leiomyoma (LMA) is yet to be established. Accordingly, it is necessary to analyze risk factors associated with uterine LMS in order to establish a method of treatment. LMP2-deficient mice spontaneously develop uterine LMS, with a disease prevalence of ∼40% by 14 months of age. It is therefore of interest whether human uterine LMS shows a loss of LMP2 expression. We found LMP2 expression is absent in human LMS, but present in human LMA. Therefore, defective LMP2 expression may be one of the risk factors for LMS. LMP2 is potentially a diagnostic biomarker for uterine LMS, and gene therapy with LMP2-encording DNA may be a new therapeutic approach.
Animals ; Biomarkers, Tumor ; biosynthesis ; genetics ; Cysteine Endopeptidases ; biosynthesis ; genetics ; Down-Regulation ; Female ; Gene Deletion ; Humans ; Interferon Regulatory Factor-1 ; biosynthesis ; genetics ; Leiomyoma ; metabolism ; Leiomyosarcoma ; diagnosis ; genetics ; metabolism ; Mice ; Mice, Knockout ; Proteasome Endopeptidase Complex ; metabolism ; Uterine Neoplasms ; diagnosis ; genetics ; metabolism