Intravenous Uterine Leiomyomatosis with Right Heart Extension: Successful Two-Stage Surgical Removal

Castelli, Patrizio; Caronno, Roberto; Piffaretti, Gabriele; Tozzi, Matteo

doi:10.1007/s10016-006-9024-0

Cited by 54 publications

(45 citation statements)

References 8 publications

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“…1,2 Its extrauterine involvement occurs in approximately 30% of cases, and intracardiac extension accounts for about 10%. [3][4][5] This extension of IVL into the right side of the heart is called intracardiac leiomyomatosis (ICL).The diagnosis of ICL can be overlooked. Echocardiography, abdominal ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) are available for detection and diagnosis.…”

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Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Li¹,

Shen²,

Sun³

et al. 2014

Texas Heart Institute Journal

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I ntravenous leiomyoma (IVL) is a rare, histologically benign smooth-muscle-cell tumor that occurs only in women. This neoplasm occupies vascular spaces from the intrauterine venules to the systemic veins, including the iliac vein and inferior vena cava (IVC), and it does not invade the tissue. The mass can extend into the right heart chambers and pulmonary arteries. 1,2 Its extrauterine involvement occurs in approximately 30% of cases, and intracardiac extension accounts for about 10%. [3][4][5] This extension of IVL into the right side of the heart is called intracardiac leiomyomatosis (ICL).The diagnosis of ICL can be overlooked. Echocardiography, abdominal ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) are available for detection and diagnosis. Echocardiography is important in the initial diagnosis of ICL. To our knowledge, the literature about ICL chiefly comprises case reports, and the authors of the few case series have not in general discussed the echocardiographic characteristics and extending pathways of ICL. We retrospectively studied the cases of 7 patients with ICL who underwent successful tumor resection in our hospital. We outlined the echocardiographic characteristics of the tumors and analyzed their clinical features, confirmed the extending pathways by means of CT reports, and studied the surgical and pathologic results. We discuss the echocardiographic diagnosis of ICL and briefly review the pertinent medical literature. Patients and MethodsWe reviewed our hospital's clinical database and identified 7 women who had undergone surgical resection of ICL tumors from January 2003 through July 2012. The echocardiographic images included parasternal, apical, and subcostal views. In addition, M-mode, pulsed and continuous-wave Doppler, and color-flow Doppler images

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Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Li¹,

Shen²,

Sun³

et al. 2014

Texas Heart Institute Journal

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“…Different surgical techniques have been utilized for this purpose: removal of the intracardiac mass through the iliac vein or through the right atrium, performed with a one-or double-stage procedure, with extracorporeal circulation or during a period of circulatory arrest, has been proposed [1,9] . In our cases, we chose a one-stage strategy.…”

Section: Treatmentmentioning

confidence: 99%

“…When growing into the heart the IVL is called intracardiac leiomyomatosis (ICL) [1] . Although histological benign, IVL might behave clinically in a "malignant" way.…”

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Intravenous leiomyomatosis with right heart involvement—A report of 4 cases and literature review

Mei

Yang

et al. 2011

J. Huazhong Univ. Sci. Technol. [Med. Sci.]

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Intravenous leiomyomatosis (IVL) is a rare benign neoplasm which originates from the smooth muscle cells and is usually confined to the pelvic venous system. Rarely, intracaval and intracardiac extension has been described. Death can occur as a result of intracardiac involvement. We reported 4 cases of IVL with right heart involvement (intracardiac leiomyomatosis, ICL). Three of them suffered recurrent sudden syncope, and the other one was totally asymptomatic. All of them were successfully treated through one-stage operation under extracorporeal circulation.

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“…2 Intravenous leiomyoma is a rare histologically benign smooth muscle cell tumor occurring in women, which arises either from the wall of a vein (usually pelvic, but it can also originate from other veins) or from a uterine leiomyoma. It is a very slow and insidiously growing tumor that extends into vessel lumens without invading them.…”

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confidence: 99%

From Uterus to Pulmonary Embolus

et al. 2009

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A 36-year-old Afro-Caribbean woman presented with recurrent syncope and shortness of breath after a longhaul flight. On admission, ECG revealed sinus tachycardia at 130 bpm and mildly flattened T waves in V 2 through V 5 ( Figure 1a). D-Dimers were elevated (663 ng/mL), and blood gases showed hypoxia. Chest x-ray was unremarkable ( Figure 1b). Family history revealed that the patient's mother had suffered a previous deep venous thrombosis.The initial clinical presentation was consistent with pulmonary embolism, confirmed by computed tomographic scan that showed a large saddle-shaped embolus in the pulmonary trunk extending into the right main pulmonary artery ( Figure 2a), the right ventricle, the right atrium, and the inferior vena cava (IVC). There was also a smaller extension into the left main pulmonary artery as well as smaller, segmental, and subsegmental thrombi.Given the extensive radiological findings along with clinical deterioration, the patient received a thrombolytic regimen with streptokinase. A repeat scan 12 hours after initiation of thrombolytic therapy showed that the saddle-shaped embolus in the pulmonary trunk had resolved (Figure 2b), but thrombotic elements in the right heart cavities and the IVC remained. An echocardiogram confirmed the presence of a large mobile mass in the right atrium, prolapsing through the tricuspid valve into the right ventricle, which was believed to be at high risk of embolization. The patient subsequently received a second thrombolysis regimen with alteplase, 110 hours after the first one, without complications.Despite the 2 thrombolytic therapies, there was no resolution of the intracardiac elements. Cardiac magnetic resonance imaging (MRI) was then requested, which revealed a large lobulated soft tissue mass extending from the inferior cava to the right atrium and into the right ventricle (Figure 3a and 3b; Movies I, II, and III in the online-only Data Supplement). The structure was vascular in nature, without invasion of the right atrial wall or of the tricuspid valve, and with a thrombus at the tip of the mass (Figure 3c). Gadolinium-enhanced magnetic resonance angiography confirmed the partial obstruction of the suprarenal IVC. The infrarenal IVC and the iliac veins were unobstructed, without evidence of tumor extension into these vessels. The hepatic veins were not involved ( Figure 4a). There was, however, a filling defect in the left renal vein (Figure 4b) and distention of the left ovarian vein, raising suspicion of an extension of the mass into this vessel, via the

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Intravenous Uterine Leiomyomatosis with Right Heart Extension: Successful Two-Stage Surgical Removal

Cited by 54 publications

References 8 publications

Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Intravenous leiomyomatosis with right heart involvement—A report of 4 cases and literature review

From Uterus to Pulmonary Embolus

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