Leiomyosarcoma of the inferior vena cava propagating into the right atrium.

Abstract

Primary tumours of the venous system are not common. Thomas and Fine (1960)

Leiomyosarcoma of the inferior vena cava: A clinicopathologic review and report of three cases

“…The tumor spread mainly systemically and most commonly to the liver and lungs, but no organ was exempt. Lymph node metastasis occurred less frequently as it occurred in five cases only including our case [13,21,41,42]. Metastatic disease was initially present in 8 of 89 resected cases (8.9%), in 13 of the 40 nonresected cases (32.5%), and in none of cases where the tumor was an incidental autopsy finding.…”

“…In addition to luminal occlusion and collateralization, venography and intravenous cardioangiography revealed distortion of cava wall, the presence of intraluminal caval or cardiac growth, or filling defects. These findings were reliable criteria for the diagnosis of the tumor and proved informative when operative venous reconstruction was entertained [11,34,41,42].…”

“…Its deep-seated location, the presence of the liver and ascites, and the absence of an extraluminal growth made its detection difficult. The diagnosis of LMS of the IVC was made or suspected at the time of presentation or prior to surgery in <5% of the patients [34,[38][39][40][41][42]. In 28 cases (21%), the patient had been previously operated or simultaneously with the discovery of the LMS, most commonly for uterine myoma [Tables I,II].…”

Unusual abdominal tumors with intracardiac extension. Two cases with successful surgical resection

“…The tumor mass in the right atrium and ventricle with intermittent partial obstruction of the tricuspid valve leads to symptoms of dyspnea, palpitation, dizziness, syncope, congestive heart failure with hepatomegaly, ascites, jugular stasis, and peripheral edema, arrhythmias and pulmonary embolism 4,5,11,16 . Further growth of the tumor can worsen the clinical course and increase the risk of sudden death due to total obstruction of the tricuspid valve [17][18][19][20][21][22][23] . Tumor and thrombi in the upper segment of IVC lead to Budd-Chiari syndrome and edema of lower extremities 4,5,11,16,24. The bi-dimensional echocardiogram 25 with emphasis on transesophageal orientation, computed tomography, and magnetic resonance can be used to confirm the diagnosis.…”

Leiomyomatous Tumors of the Hepatobiliary Tract

“…The original description of a primary leiomyosarcoma of the inferior vena cava was published in 1871 (Perl 1871). Since then, numerous cases have been reported, either being restricted to distinct segments of the caval vein or concomitantly involving its large associated veins (Hallock et al 1940;Deutsch et al 1968;Wray and Dawkins 1971;Parrilla et al 1992;Hollenbeck et al 2003;Dew et al 2005;Hardwigsen et al 2005;Sokolich et al 2008;Kr€ uger et al 2010;Laskin et al 2010;Sessa et al 2010;Huang et al 2011;Ulla et al 2011). Leiomyosarcoma of the inferior vena cava occurs in patients usually older than 40 years; in a study of 25 patients, the age range was 41-79 years, with a median age of 56 years (Hollenbeck et al 2003).…”