A case of intravenous leiomyomatosis of uterine origin, extending through the inferior vena cava to right atrium

Abstract

Intravenous leiomyomatosis (IVL) is a rare benign tumor that originates from uterus, and sometimes extends to the right heart. We report a case of IVL that extended to right atrium through the inferior vena cava (IVC) which was resected using partial cardiopulmonary bypass. Multi detector computed tomography and ultrasound played a vital role in arriving at the diagnosis. Complete resection of tumor in the heart and great vein, and separation of the tumor stump from the IVC are essential in the treatment of IVL.

Intravenous leiomyomatosis with intracardiac extension depicted on computed tomography and magnetic resonance imaging scans: A report of two cases and a review of the literature

“…The features of malignant thrombosis are similar to those of the primary tumor and the lesion is generally considerably smaller than the IVL. In addition, malignant thrombosis is not associated with a history of uterine myoma or surgery for uterine myoma (10). Complete surgical excision of the tumor is an appropriate treatment option for IVL, and the final diagnosis is dependent on histopathological analysis.…”

Intravenous leiomyomatosis

“…Lam et al have recently reviewed the literature and identified 200 reported cases of leiomyomatosis, 68 of which had intracardiac extension. 5 They identified signs of cardiac failure, venous obstruction or abdominal distension (in women with previous history of hysterectomy done for fibroid uterus) as being the most common mode of presentation in those with involvement of the right atrium as in our case. In the early stages of venous extension, the diagnosis is often unrecognized and further extension of intravenous leiomyomatosis is not prevented by resection of the primary tumour.…”

“…2 IVL may also embolise to the right cardiac chambers or pulmonary artery and cause obstruction .It may be diagnosed as a primary cardiac tumour or a venous thrombus. 5 Intra-venous leiomyomatosis, benign metastasizing leiomyoma and malignant leiomyosarcoma are three rare associated variants of uterine leiomyoma. Recognition of the potential of fibroids for venous invasion was accredited to Birch Hirschfeld who described four cases in 1896.…”

“…6 Surgery can be performed as both single and staged procedures and cardiopulmonary bypass is advocated for resection of tumours with intra-cardiac extension. 5 Although there may be some difficulties in distinguishing such lesions from low-grade sarcomas, they are different histologically from sarcomatosis uteri because the intravenous plug is mainly smooth muscle in origin. The tumor behaves like a benign tumor, and the mitotic index is low with most active lesions showing only one mitosis per 15 high-power fields.…”

Intravascular Leiomyomatosis and the CT Appearance: A Case Report and Review of Literature

“…The features of malignant thrombosis are similar to those of the primary tumor and the lesion is generally considerably smaller than the IVL. In addition, malignant thrombosis is not associated with a history of uterine myoma or surgery for uterine myoma [18]. Complete surgical excision of the tumor is an appropriate treatment option for IVL, and the final diagnosis is dependent on histopathological analysis.…”