Uterine Intravenous Leiomyomatosis with Right Ventricular Extension

Abstract

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 45-year-old woman without a history of gynaecological surgeries, who was referred to our institution due to repeated syncopal attacks. Computed tomography indicated the presence of an intravenous leiomyoma originating from the uterus and extending to the inferior vena cava, right atrium, and right ventricle. The patient was successfully treated by cardiotomy, which was performed under hypothermic circulatory arrest, and laparotomy in a single-stage operation. She continued to recover and did not exhibit any recurrence at the 10-month follow-up.

Inferior Vena Cava Syndrome of uncommon aetiology

“…In spite of this, no thromboembolic events occurred. In the review of the literature, the venous approach in order to remove the mass is usually performed by venotomy of the IVC directly [6,9,10]. By this approach, in stage II patients, a median of 1000cc blood loose has been reported [6].…”

Multimodality Evaluation of Intravenous Leiomyomatosis: A Rare, Benign but Potentially Life-Threatening Tumor

“…IVL usually occurs in premenopausal women at age 20–70 years with a median age of 45 years, who have had multiple pregnancies [ 4 , 5 ]. Symptoms include shortness of breath, tachycardia, syncope, chest pain, pelvic pain, irregular menses, and abdominal mass, and may even result in sudden death [ 6 , 7 ]. However, patients may also be asymptomatic.…”

“…Treatment of IVL is almost always surgical [ 7 , 10 , 14 ], which includes excision of the extra-uterine tumor and myomectomy or total hysterectomy, as necessary. Sternotomy with cardio-pulmonary bypass, as well as laparotomy in a single or 2-stage operation, may be performed for management of a mass that extends to the cardiovascular system.…”

Tumor Obstruction of the Inferior Vena Cava and Its Tributaries: Diagnosis, Features of Surgical Interventions, the Results of Surgical Treatment. Part I

“…Нередкими источниками образования опухолевых тромбов в магистральных венах бассейна НПВ являются опухоли матки. Среди новообразований матки возможность внутривенной инвазии имеется, прежде всего, при лейомиоме матки и некоторых видах лейомиосарком матки [24][25][26][27][28][29][30][31]. Характерная клиническая картина -как правило, субклиническое течение заболевания, и обширное опухолевое поражение подвздошных вен, нижней полой вены и сердца, позволили многим исследователям [22,29,31] называть эти ситуации «внутривенный лейомиоматоз» и «внутривенный лейомиосаркоматоз».…”

“…«Подвздошное» распространение лейомиоматоза отмечается примерно у 70 %, «гонадное» -у 30 % больных [28]. Это заболевание поражает женщин преимущественно в среднем возрасте 40-45 лет, у значительной части из которых в анамнезе имело место удаление матки и(или) придатков [26,27,30,31]. H. Zeng и соавт.…”