Intravenous Leiomyomatosis With Extension to the Heart: Echocardiographic Features

Abstract

A 40-year-old female was admitted to the hospital for an operation with a presumptive diagnosis of right atrial myxoma. She had had a hysterectomy for leiomyoma 8 months earlier and has since experienced progressive dyspnea on exertion, pedal edema, and two syncope episodes in the past 2 months. Cardiac murmur was detected and two-dimensional echocardiography was arranged. A mobile right atrial mass was discovered, which was thought to be a myxoma. The patient subsequently received open heart surgery. Histologic examination of the resected mass confirmed intravenous leiomyomatosis. Magnetic resonance imaging performed on the eighth postoperative day revealed an intravascular mass from the right common iliac vein to the upper abdominal inferior vena cava. The patient underwent a second operation one and half months after the first operation, with resection of the tumor mass in the inferior vena cava and resection of multiple uterine myoma. She recovered well. Repeat magnetic resonance imaging 6 months following the second operation showed that the inferior vena cava and right atrium were clear of tumor.

Huge Intravascular Tumor Extending to the Heart: Leiomyomatosis

“…Because of rarity and unusual growth potential of intravenous leiomyoma, several diagnostic tests such as TTE/TEE, computerized tomographic angiography, MRV, and conventional venography should be used to avoid delay in diagnosis and extension. Extension to the right heart chambers can cause valve obstruction, leading to cardiac insufficiency and death [4, 5]. …”

Intravenous leiomyomatosis: A rare cause of intracardiac mass

“…9,11---13 Transesophageal echocardiography provides better spatial resolution and information about where the tumor is attached to the atrial wall or interatrial septum and the relationship with the IVC and superior vena cava. 9,12 However, accurate preoperative diagnosis of IVL is still usually difficult. 7,14 In the present case, a precise diagnosis was not obtained before surgical resection and histologic examination because the initial presentation and findings pointed to a large thrombus.…”

“…11---13,15 The characteristics and attachment site of the tumor detected by echocardiography may offer important information for the differential diagnosis of a right atrial mass. 12 Surgery remains the gold standard treatment; complete removal of the tumor, with hysterectomy and resection of all extra-uterine masses, is mandatory for a favorable outcome and to avoid recurrence. 3,4,7---9,16 The first successful resection of intracardiac extension of such tumors was reported by Timmis et al in 1980.…”

“…4,16 If the tricuspid valve has been destroyed by the tumor, tricuspid valvuloplasty or valve replacement should be carried out. 12,13 Long-term prognosis of IVL is usually good if the tumor can be completely removed. However recurrence and metastases are not unusual and can be found up to 15 years after surgery.…”

“…3,10,13,15 Reintervention is recommended in cases of recurrence to achieve long-term disease-free survival. 4,12 Conclusions IVL should be suspected on detection of a right atrial mass originating from the IVC in a middle-aged woman, especially with a history of uterine fibroids or hysterectomy. Imaging studies are helpful in the diagnosis.…”