The diagnosis, morphological particularities, and surgical technique in a case of intravascular leiomyoma extended to the right heart chambers

Abstract

Intravenous leiomyoma is a benign smooth muscle cell tumor of uterine origin that may grow into the pelvic veins and the inferior vena cava. It usually affects premenopausal women and the majority (90%) are parous. Because cardiac involvement is present in up to 10% of cases, it may be misdiagnosed as a primary cardiac tumor or a venous thrombus-in-transit. We describe a case of intravascular leiomyomatosis with cardiac extension and the morphological particularities of the removed tumor.

Intravascular leiomyoma with heart extension

“…An intravascular leiomyoma is an insidious tumor, and extension into the right heart chambers can occur slowly, over many years (2-4,7-10). Our PubMed and Cochrane library literature search retrieved only 132 cases of leiomyoma with heart extension, many of which (∼40%) have been described over the last 15 years.…”

“…An intravascular (or intravenous) leiomyoma is a histologically benign tumor characterized by the proliferation of smooth muscle cells arising from the intrauterine venules and/or the myometrium (1-4); however, exceptional cases that arise from cutaneous vessels and pelvic or retroperitoneal veins have been found (5,6). This tumor affects only women, particularly middle-aged women (median age, 44 years) with a history of hysterectomy (4).…”

“…This tumor affects only women, particularly middle-aged women (median age, 44 years) with a history of hysterectomy (4). Usually, an intravascular leiomyoma enters through the lumen of the iliac veins and grows into the inferior vena cava, sometimes reaching the right heart chambers (1-4,7-10). In this situation, one- or two-stage surgery has been used to prevent fatal complications (e.g., total tricuspid obstruction) (3,4,9-13).…”

Hypothermic Cardiac Arrest to Remove Right Atrial Thrombi Due to Abdominal Malignancies

“…It is known also as benign metastatic fibro-leiomyoma because pulmonary nodules (proliferating leiomycites) are often present. Although this tumour is usually confined to the pelvis, it sometime extends to the right cardiac cavities through the iliac vein and the vena cava (Wakiama et al, 2000) by means of intra-vascular proliferation and growth of fibro-myocites which results in an extension of the fibro-leiomyoma rather than in a thrombus formation conferring a typical consistency and resistance to traction to the endo-vascular proliferation (Galajda et al, 2010). IVL with cardiac extension is an exceedingly rare disease first reported in 1907 in an autopsy series (Durk, 1907).…”

“…Since then a further 34 cases have been reported involving the right heart (Wakiyama et al, 2000). Patients often have a history of hysterectomy or leiomyoma resection and various lengths of intravascular proliferation up to 29 cm with a diagnosis performed up to 18 yrs after the primary surgery ( Galajda et al, 2010). Complete surgical removal of the uterus and IVL is the therapy of choice (Topcouglu et al, 2004) and it has been carried out for the first time in 1974 using CPB and HCCA (Mandelbaum et al, 1974).…”