Recurrent intracardiac leiomyomatosis

An intravenous leiomyoma, a histologically benign smooth muscle tumour, arises from either a uterine myoma or the walls of a uterine vessel, with extension into veins. The present report describes echocardiographic features of an intravenous leiomyoma that spread into the right-sided cardiac chambers in a middle-aged woman who had undergone a hysterectomy two years earlier. Echocardiographic features included an elongated mobile mass extending from the inferior vena cava and multiple masses in the right atrium and right ventricle. Intracardiac leiomyomatosis should be considered in women who present with a cardiac mass in the right-sided chambers.     

Benign Metastasizing Leiomyoma in the Heart of a 45-Year-Old Woman

We report a rare case of benign metastasizing leiomyoma in the heart of a 45-year-old woman 2 years after a uterine leiomyoma had been discovered during hysterectomy. Computed tomograms at presentation showed a large mixed cystic mass in the pelvis and bilateral lung nodules suggestive of metastatic disease. A large cardiac mass, attached to the chordae of the tricuspid valve and later shown to be histopathologically consistent with uterine leiomyoma, was successfully resected through a right atriotomy. This case suggests that benign metastasizing leiomyoma should be considered in the differential diagnosis of right-sided cardiac tumors.     

Intracardiac extension of intravenous leiomyoma

Intravenous leiomyoma with intracaval and intracardiac extension is a rare benign neoplasm affecting women. We report a case in which an intravenous leiomyoma originated from the right iliac vein and extended through the inferior vena cava into the right atrium. The correct diagnosis was made intraoperatively, after resection of the intracardiac extension of the tumor, which was 28 cm in length. At the 2nd stage of the operation, a right common iliac venotomy enabled the removal of all residual tumor from the inferior vena cava.     

Intracardiac leiomyomatosis: echocardiographic features

Intravenous leiomyomatosis is a histologically benign smooth-muscle tumor arising from either a uterine myoma or the walls of a uterine vessel with extension into veins. Echocardiographic features of two cases of intravenous leiomyomatosis with extensive spread into the right-sided cardiac chambers and pulmonary arteries are described. Both patients were middle-aged women, with prior history of hysterectomy 12 and 10 years earlier who presented with cardiac symptoms and signs. Distinctive echocardiographic features include 1) elongated mobile masses extending from the veins of the lower body, including inferior vena cava and azygos vein; 2) multiple venous attachments or metastases; and 3) filling of venous vessels and right-heart chambers. Intracardiac leiomyomatosis should be considered in a female patient presenting with an extensive mass in the right-sided cardiac chambers.     

Intravenous leiomyomatosis: A rare cause of intracardiac mass

Intravenous leiomyomatosis is an unusual clinical condition characterized by histologically benign smooth muscle lesions extending from the uterus into pelvic and systemic veins and, more rarely, into the right cardiac chambers. We report the case of a 45-year-old woman who presented with a three-week history of dyspnea on exertion, shortness of breath and fatigue. Echocardiography showed a large mobile mass in the right atrium prolapsing into the right ventricle and extending to the inferior vena cava. A computed tomography scan revealed a large mass extending from the right atrium to the inferior vena cava and through the systemic veins as far as the popliteal veins. A presumptive diagnosis of large thrombus was made; the correct diagnosis of intravenous leiomyomatosis with intracardiac involvement was obtained only after surgical resection and histologic examination.     

Intravenous leiomyomatosis with extension to the heart: echocardiographic features: a case report

A 40-year-old female was admitted to the hospital for an operation with a presumptive diagnosis of right atrial myxoma. She had had a hysterectomy for leiomyoma 8 months earlier and has since experienced progressive dyspnea on exertion, pedal edema, and two syncope episodes in the past 2 months. Cardiac murmur was detected and two-dimensional echocardiography was arranged. A mobile right atrial mass was discovered, which was thought to be a myxoma. The patient subsequently received open heart surgery. Histologic examination of the resected mass confirmed intravenous leiomyomatosis. Magnetic resonance imaging performed on the eighth postoperative day revealed an intravascular mass from the right common iliac vein to the upper abdominal inferior vena cava. The patient underwent a second operation one and half months after the first operation, with resection of the tumor mass in the inferior vena cava and resection of multiple uterine myoma. She recovered well. Repeat magnetic resonance imaging 6 months following the second operation showed that the inferior vena cava and right atrium were clear of tumor.     

静脉内平滑肌瘤病延伸至右侧心腔的诊断和外科治疗

目的探讨静脉内平滑肌瘤延伸至右侧心腔的诊断与外科治疗。方法2001年1月至2006年5月我院收治静脉内平滑肌瘤延伸至右侧心腔患者2例,均分期手术。首先行常规全麻中低温体外循环下心脏肿物切除术,然后二期切除子宫、附件及子宫病变组织,进行盆腔清扫术。结果无围术期并发症,2例患者随访至今情况良好。结论提高对多发性子宫肌瘤或右心房肿物的警惕性,一期直视下心脏内肿物切除术,二期切除子宫病变组织和盆腔清扫术,可获满意的效果。     

Ungew?hnlicher Befund einer rechtsatrialen Raumforderung

A 36-year-old woman was admitted for performance of a cardiac MRI scan because of a suspected myxoma in the right atrium. A large intracardiac tumor, reaching from the right atrium to the inferior vena cava, was found on the MRI scan. The morphologic structure, growth pattern, and feature of late gadolinium enhancement were unusual for intracardiac myxoma, so a CT scan of the abdomen and CT-guided biopsy were performed. The histological examination showed an intravenous leiomyoma; this finding was explained by a hysterectomy the patient had undergone 18?months previously due to uterus myomatosus. An injury of the pelvic veins during extirpation of the uterus had been described. The intracardiac and intra-abdominal tumor excision was successfully performed using cardiopulmonary bypass. The present report describes a rare finding of intracardiac mass.     

Intracardiac extension of intravenous leiomyomatosis in a pregnant woman: A case report and review of the literature

Intravenous leiomyomatosis is an uncommon clinical entity characterized by the growth of a benign, smooth muscle tumour within the venous system. Intracardiac extension of this tumour is rare, and approximately 35 cases have been reported in the literature. The second case of massive intracardiac extension of intravenous leiomyomatosis is reported in a pregnant patient diagnosed after the unusual presentation of seizure activity. Tumours were successfully removed from the right internal iliac vein, inferior vena cava, and right atrium and ventricle in a single-stage operation using cardiopulmonary bypass and circulatory arrest. Concomitant total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. Nine months after diagnosis, the patient was evaluated for recurrent disease. A review of the literature from 1994 to 1998 is presented.     

Intravenous leiomyomatosis of the uterus with extension into the heart

A 42 year old woman presented with impaired filling of the right atrium and right ventricle two years after removal of uterine leiomyomas. At the time of hysterectomy, intravenous leiomyomatosis was noted, with extension of the tumor into the inferior vena cava. The tumor subsequently extended into the right atrium and coronary sinus, and protruded through the tricuspid orifice. It was successfully removed from the heart, and proved to be histologically benign.     

Intravascular leiomyomatosis: report of a case and review of the literature

So far, only 13 cases with extension of an intravascular leiomyoma into the heart have been reported. We present the second known case of leiomyomatosis with extension of the tumor through the tricuspid valve into the right ventricle, which was first removed successfully. Ten years after an abdominal hysterectomy for a myomatous uterus, a 53-year old woman presented with a intracavitary mass in the right-sided heart chambers which could be followed retrogradely into the inferior vena cava and right pelvic veins, representing an intravasal extension from the pelvic leiomyoma. The clinical picture, the two-stage operative management, and the postoperative course are reviewed and the literature on the subject is presented.     

Renal adenocarcinoma with intramyopericardial and right atrial metastasis, latter via coronary sinus: report of a case

Primary renal tumors with intracardiac metastasis are not infrequent. Most of the secondary spread is blood-borne and occurs via inferior vena cava. Patients with such a spread often present with cardiac symptoms. We presume that a metastatic spread in the right atrium through coronary sinus has never been reported in the literature according to the result of a Medline search at the time of writing this report.     

Echocardiographic characteristics of intravenous leiomyomatosis with intracardiac extension: a single-institution experience

Background: Intravenous leiomyomatosis (IVL) is a rare smooth‐muscle proliferation arising from a uterine myoma and occasionally extending into cardiac chambers. Methods and Results: A series of 10 consecutive patients with histologically and surgically proven intracardiac IVL between 2000 and 2010 in our hospital were reviewed. The echocardiographic features of 10 cases with IVL and extensive spread into the right‐sided cardiac chambers were described for the first time. All patients were female and the mean age was 42 ± 7 years old. The first symptoms of six patients (60%) were exertional dyspnea and palpitation of cardiac origin. Echocardiography showed that all the tumors originated from the inferior vena cava (IVC) and located in cardiac right chambers (70% in right atrium alone, 30% in right ventricle and atrium). Eight masses (80%) were oval, whereas the others (20%) were serpentine, all with well‐demarcated borders and most (70%) with heteroechogenic texture. Five tumors (50%) intermittently prolapsed into right ventricle through the tricuspid valve. Two patients with nodules adhering to the top of the tumors had pulmonary tumorous thromboembolism. Conclusion: Echocardiography is a simple and important technique to diagnose IVL with intracardiac extension. This disease should be considered in a female patient presenting with an extensive mass from IVC with well‐demarcated border in the right‐sided cardiac chambers. (Echocardiography 2011;28:934‐940)     

Early Detection of a Cavopulmonary Tumor Embolus with the Use of Transesophageal Echocardiography

Pulmonary tumor embolization from renal cell carcinoma is associated with severe cardiopulmonary morbidity and high perioperative mortality rates. We report the case of a 71-year-old woman who presented with right-sided abdominal pain. Magnetic resonance images revealed a mass originating from the upper pole of the right kidney and extending into the infrahepatic portion of the inferior vena cava. Transesophageal echocardiography was continuously used to monitor the mass during intended radical nephrectomy and tumor resection. When the right kidney was mobilized, intracaval thrombus detached and migrated to the patient''s right atrium, causing severe hemodynamic instability. After emergent sternotomy and during the initiation of cardiopulmonary bypass, the mass was no longer echocardiographically detectable in the heart; it was soon removed completely from the left pulmonary artery. The mass was a renal cell carcinoma.We recommend the use of transesophageal echocardiography as an efficient diagnostic tool in the early detection of pulmonary tumor embolization during the resection of renal cell carcinoma that involves the inferior vena cava.     

Secondary tumors of the right heart. Echocardiographic aspects. Apropos of 6 cases in the adult

Secondary cardiac tumours are rare but but are now more frequently diagnosed by echocardiography. We report 6 cases of intracardiac metastases affecting the right heart which were diagnosed by 2D echocardiography. In 3 cases, a very mobile, oval-shaped tumour was visualised within the right atrium prolapsing into the tricuspid orifice in diastole like a myxoma but associated in 2 cases with signs of invasion of the inferior vena cava. Two other non-mobile tumours were observed causing massive invasion of the right atrium and the last case was of an infiltrating tumour of the right ventricle resulting in pulmonary infundibular obstruction. In the light of our experience and a review of the literature, it is difficult to distinguish secondary tumours of the right atrium from myxomas especially when the tumours are mobile and when it is impossible to visualise a pedicle inserted on the interatrial septum or tumoral invasion of the inferior vena cava. At the ventricular level, the diagnostic signs differ according to whether there is tumoral invasion of the cavity or infiltration of the muscular wall. These cases illustrate the value of 2D echocardiography in the diagnosis of intracardiac metastases, sometimes even in the absence of clinical signs.     

An unusual case of recurrent mass in the right atrium: intravenous leiomyomatosis

Intravenous leiomyomatosis with a cardiac extension is an extremely rare condition. In this report, a case of a 43-year-old female patient is described: she was operated for right atrial mass protruding into the inferior vena cava, which was later diagnosed as leiomyoma. After a 3-year symptom-free period, recurrence of the extension through the inferior vena cava was observed. After abdominal ultrasonographic examination, which revealed bilateral ovarian and retroperitoneal mass, bilateral oopherectomy, retroperitoneal, and right atrial mass excision was done. The retroperitoneal and right atrial mass was reported as leiomyoma. On her last admission, she had complaints of dizziness, abdominal pain, and bilateral leg edema; and right atrial mass extending through the common iliac vein was noted, but the patient did not accept any further treatment modality.     

Surgical management of a uterine leiomyoma extending through the inferior vena cava into the right heart

 We present a case of a 38-year-old woman with intravenous leiomyomatosis originating from the uterus involving the right ventricle, right atrium, inferior vena cava, and iliac veins. Tumors in the heart and the inferior vena cava were removed using cardiopulmonary bypass and circulatory arrest; 10 days after the cardiac operation, hysteroadnexectomy was performed. Received: April 26, 2002 / Accepted: August 13, 2002 Correspondence to M. Gaudino     

Intra-caval leiomyomatosis. Surgical treatment

The authors report a case of intracaval leiomyomatosis diagnosed in a 11-year old female, who had been initially operated for a massive tumor of uterus (35 X 45 X 25) and who had had subtotal hysterectomy performed. The histologic diagnosis at was intravenous leiomyomatosis. Investigation of extent of neoplastic spread included echography, cavography and revealed inferior vena cava involvement. Cavotomy removed a tumor fragment. Intravenous leiomyomatosis has always its source in the uterus and spreads by progressive invasion of the vena cava. The right cardiac cavities and, at times, the pulmonary artery are also involved. Management of cardiac invasion is a determinant factor of the severity of the disease. Indication for surgery is systematic and should result in complete tumor excision.     

Surgery of hepatoma with intracavitary cardiac extension

Summary A case of primary liver carcinoma with intracavitary cardiac extension is presented. A 36-year-old female was admitted to our surgical clinic with dyspnea and generalized edema. Echocardiography and superior vena cavography demonstrated a large filling defect in the right atrium. After a diagnosis of acute cardiac failure due to an intracardiac tumor, the patient was operated upon immediately. A right atriotomy exposed a large yellow mass within the right atrium, which was not adherent to the atrial wall. The mass was in continuity with similar material in the inferior vena cava and right hepatic vein. With a suspicion of hepatic malignancy, the atrial tumor was removed, and debulking of the mass in the inferior vena cava and right hepatic vein was performed. A postoperative histological examination of the tumor showed hepatocellular carcinoma. Her postoperative course was uneventful, and she was discharged from the hospital.Intracardiac extension of hepatoma is rarely encountered. In this clinical setting, long-term survival cannot be anticipated from any surgery, but palliative clearing of the atrium and inferior vena cava may be of value in preventing cardiac arrest causing sudden death.     

An autopsy case of invasive thymoma extending to the right atrium with superior vena cava syndrome as the initial manifestation]

We report a case of invasive thymoma presenting with superior vena caval obstruction and intracardiac extension. A 74-year-old man was admitted in July 2002 with swelling of the face and right upper extremity. Computed tomography of the chest revealed a small anterior mediastinal mass, which infiltrated the lumen of the superior vena cava extending into the right atrium. Invasive thymoma was strongly suspected, but he refused any medical treatment. His health declined steadily, with repeated right-sided heart failure. He died due to cardiac tamponade 50 months after his first visit. On autopsy, the tumor was diagnosed as a thymoma classified as type B3 according to the WHO histological classification. Formation of a tumor thrombus in the superior vena cava and the right atrium is a rare mode of extension of thymoma. In this respect, our case may be valuable for improving the understanding of the natural course of invasive thymoma.