Multimodality Evaluation of Intravenous Leiomyomatosis: A Rare, Benign but Potentially Life-Threatening Tumor

Abstract

Patient: Female, 40Final Diagnosis: Intravenous leiomyomatosisSymptoms: Chest pain • syncopeMedication: —Clinical Procedure: ThoracotomySpecialty: Radiology • CardiologyObjective:Rare diseaseBackground:Intravenous leiomyomatosis (IVL) is a rare tumor, which is usually of uterine origin, characterized by intravascular nodular masses of histologically benign smooth muscle that may extend variable distances, including into the inferior vena cava, right atrium and pulmonary arteries. Tumors may arise from uterine leiomyoma, walls of the uterine vessel, or myometrium. It usually occurs at between 20–70 years of age with a median age of 45 years. The most commonly affected women are pre-menopausal and multiparous. Intra-cardiac extension may represent a diagnostic challenge as it is usually misdiagnosed as a right atrial myxoma and may cause multiple symptoms, such as shortness of breath, tachycardia, chest pain, syncope, and even death.Case Report:We present the case of a 40-year-old female patient with past medical history of arterial hypertension, who was referred to a cardiovascular center due to an intra-cardiac mass found on 2D echocardiogram. The patient was given the rare diagnosis of intravenous leiomyomatosis of the uterus with extension into the gonadal veins, inferior vena cava, right atrium, right ventricle, and main pulmonary arteries. Imaging workup including trans-esophageal echocardiogram, cardiac catheterization, contrast-enhanced abdomen and pelvic CT scans, and cardiac MRI was performed for evaluation.Conclusions:Intravenous leiomyomatosis is a rare diagnosis that merits consideration in a young pre-menopausal female patient with cardiac symptoms associated with a right atrial mass. Radiologists play a vital role in the diagnosis and follow-up of patients with the diagnosis of intravenous leiomyomatosis. Differential diagnosis includes vascular thrombus as well as primary and metastatic tumors. Early detection is imperative for appropriate treatment and surgical planning.

Multidisciplinary approach to pelvic leiomyomatosis with intracaval and intracardiac extension: A case report and review of the literature

“…The clinical manifestations are closely related to the extent of involvement. Signs and symptoms include: i) pelvic pain, abnormal uterine bleeding due to myomatous uterus; ii) lower extremity edema from IVC occlusion; iii) dyspnea and chest pain caused by pulmonary embolus; iv) syncopal episodes due to tricuspid orifice obstruction from the tumor; v) ECG abnormalities related to enlargement of the cardiac chambers, systolic dysfunction, and cardiac valve involvement; and vi) sudden death from obstruction of the tricuspid valve orifice or right ventricular outflow tract ( Fornaris et al, 2015 ).…”

Extensive Intravascular Leiomyomatosis with Cardiac Involvement, Clinicopathological and Imaging Characteristics: A Case Report

“…If there is invasion to the cardiac cavities, it can cause fatigue, palpitations, chest pain, dyspnea, ascites, abdominal pain and hepatomegaly, syncope and even sudden death. [2,3] IVL has a serpentine appearance, proliferates throughout the venous system, but doesn´t invade it. Usually grows in the iliac veins or the inferior vena cava although there are very few reported cases with direct extension to the right atrium and causing symptoms of acute heart failure.…”

“…Usually grows in the iliac veins or the inferior vena cava although there are very few reported cases with direct extension to the right atrium and causing symptoms of acute heart failure. [3,4]…”

Intravenous Leiomyomatosis: An Uncommon Cause of Pulmonary Embolism

“…Intravenous leiomyomatosis (IVL) is a rare benign tumor characterized by continuous intraluminal growth, found predominantly in premenopausal women. The initial presentation is often insidious and depends on the tumor size and vascular involvement, as it may extend from pelvic veins, through iliac veins and inferior vena cava (IVC) into right chambers of the heart, in rare cases affecting pulmonary vasculature by continuous growth or by formation of non-thrombotic pulmonary emboli (NTPE) [ 1 , 2 ]. This may have significant clinical implications, as pulmonary embolism is one of the most common causes of cardiovascular death; however, it is predominantly caused by venous thrombi [ 1 – 3 ].…”