EComment. Misdiagnosis of intravenous leiomyomatosis with cardiac extension

Abstract

We read with great interest the article by Li et al. [1]. They performed a comprehensive literature search including 194 cases of intracardiac leiomyomatosis. We also would like to add some comments on misdiagnosis of this complicated pathology. Intravenous leiomyomatosis (IVL) is a rare neoplastic disease, which is characterized by intravenous growth of histologically benign, smooth muscle tumours arising from either a uterine myoma or from the wall of a uterine vessel [2, 3]. The tumour usually enters through the lumen of the iliac vein and extends upward to varying locations, including the inferior vena cava, adrenal and renal veins, right heart (intracardiac leiomyomatosis) and pulmonary artery. It is an insidious process and may lead to heart failure, pulmonary embolization, or even sudden cardiac death. Intracardiac extension may be easily misdiagnosed as either a primary cardiac tumour or a venous thrombus-in-transit. Misdiagnosis subsequently may lead to improper treatment [2, 4, 5]. Differential diagnosis of right-sided cardiac masses includes thrombi-in-transit, right atrial myxomas, vegetations on the tricuspid valve, primary caval leiomyosarcoma, intracardiac electrodes, and tumour thrombi from malignancies such as hepatocellular carcinoma, renal cell carcinoma, Wilms' tumour (in children), and others [3, 4]. IVL may be diagnosed by echocardiography, abdominal ultrasound, magnetic resonance imaging, enhanced chest and abdominal computed tomography, and venography. The diagnosis is confirmed intraoperatively or by postoperative histopathological examination [3, 5]. A thrombus-in-transit appears as an elongated mobile mass of venous cast giving a popcorn appearance within the right-sided cardiac chamber, whereas there are multiple points of attachment in the inferior vena cava and right-sided cardiac chambers in patients with IVL [8]. A correct diagnosis depends on a high index of suspicion. This pathology should be considered in young women with a right-sided cardiac mass and a pelvic mass or those who have previously undergone hysterectomy for uterus leiomyoma [2, 3, 5]. Finally, we think that IVL is an underdiagnosed pathology and a huge index of clinical doubt with the modern diagnostic imaging tools will lead to an increased number of reported cases in the future. Conflict of interest: none declared.