Huge Intravascular Tumor Extending to the Heart: Leiomyomatosis.
ABSTRACT: Intravenous leiomyomatosis (IVL) is a rare neoplasm characterized by histologically benign-looking smooth muscle cell tumor mass, which is growing within the intrauterine and extrauterine venous system. In this report we aimed to present an unusual case of IVL, which is originating from iliac vein and extended throughout to right cardiac chambers. A 49-year-old female patient, who was treated with warfarin sodium due to right iliac vein thrombosis, was admitted to our department with intermittent dyspnea, palpitation, and dizziness. Physical examination was almost normal except bilateral pretibial edema. On magnetic resonance venography, there was an intravenous mass, which is originated from right internal iliac vein and extended into the inferior vena cava. Transthoracic echocardiography and transesophageal echocardiography revealed a huge mass extending from the inferior vena cava through the right atrium, with obvious venous occlusion. Thoracic, abdominal, and pelvic MR showed an intravascular mass, which is concordant with leiomyomatosis. Surgery was performed through median sternotomy. A huge mass with 25-cm length and 186-gr weight was excised through right atrial oblique incision, on beating heart with cardiopulmonary bypass. Histopathologic assessment was compatible with IVL. Exact strategy for the surgical treatment of IVL is still controversial. We used one-stage approach, with complete resection of a huge IVL extending from right atrium to right iliac vein. In such cases, high recurrence rate is a significant problem; therefore it should be kept in mind.
Project description:Uterine leiomyomatosis is a common disease in women; however, intravenous leiomyomatosis with intracaval and intracardiac tumor extension is rare. We sought to analyze the clinical and echocardiographic features of intracardiac leiomyomatosis. From January 2003 through July 2012, 7 women (age range, 24-59 yr) underwent surgical resection of histopathologically diagnosed intracardiac leiomyomas at our hospital. Most of the patients had histories of hysterectomy or uterine leiomyoma. We retrospectively analyzed their preoperative echocardiograms. We found that the tumors had no stalks, did not adhere to the wall of the right side of the heart, were highly mobile, and moved back and forth in the right atrium near the tricuspid orifice. All tumors originated from the inferior vena cava and had borders well demarcated from that structure's wall. Most of the masses extended into the inferior vena cava and right atrium through the right internal and common iliac veins. Computed tomograms revealed pelvic tumors and contiguous filling defects in 6 patients. When echocardiograms reveal a right-sided cardiac mass that originates from the inferior vena cava, particularly in women who have a history of hysterectomy or uterine leiomyoma, intracardiac leiomyomatosis should be suspected. If the mass has no stalk and freely moves within the inferior vena cava and right-sided cardiac chambers without attachment to the endothelial surface or endocardium, intracardiac leiomyomatosis should be diagnosed. We discuss our findings and briefly review the relevant medical literature.
Project description:Purpose:This study aimed to retrospectively review the diagnosis and surgical treatment of uterine intravenous leiomyomatosis (IVL). Methods:The clinical data of 14 patients with uterine IVL admitted to our hospital between 2013 and 2018 were retrospectively analyzed, including their demographics, imaging results, surgical procedures, perioperative complications, and follow-up results. Results:The tumors were confined to the pelvic cavity in 7 patients, 1 into the inferior vena cava, 4 into the right atrium, and 2 into the pulmonary artery (including 1 into the superior vena cava). Only one case was misdiagnosed as right atrial myxoma before the operation, which was found during the surgery and was treated by staging surgery; all the other patients underwent one-stage surgical resection. Three patients underwent complete resection of the right atrial tumor through the abdominal incision, and one patient died of heart failure in the process of resection of heart tumor without abdominal surgery. During the 6-60 months of follow-up, 4 patients developed deep venous thrombosis of the lower extremity, and 1 patient developed ovarian vein thrombosis and pulmonary embolism. After anticoagulation treatment, the symptoms disappeared. One patient refused hysterectomy and the uterine fibroids recurred 4 years after the operation. Conclusion:Specific surgical plans for uterine IVL can be formulated according to cardiac ultrasound and computed tomography (CT). For the first type of tumor involving the right atrium, the right atrium tumor can be completely removed through the abdominal incision alone to avoid thoracotomy. The disease is at high risk of thrombosis and perioperative routine anticoagulation is required.
Project description:A nineteen-month-old girl was taken up for patent ductus arteriosus (PDA) device closure. A diagnostic catheter from the right femoral venous access entered the superior vena cava (SVC), through the azygos vein suggesting interruption of inferior vena caval with azygos continuity. Therefore, the PDA device was closed from the right jugular venous access. However, a postprocedure echocardiogram (echo) showed a patent inferior vena caval connection into the right atrium. An angiogram from femoral veins showed communication between the iliac veins and the azygos system, in addition to normal drainage into the inferior vena cava (IVC). Congenital communication between the iliac veins and the azygos system can mimic IVC interruption. An attempt to theoretically explain the embryological origin of the communication has been made.
Project description:We present an unusual variant of the sinus venosus defect in which an obvious window is formed between a single pulmonary vein and the superior vena cava, the pulmonary vein retaining its connection to the left atrium. Two patients were operated on via right anterior minithoracotomy. A large single right pulmonary vein was found connecting to the left atrium. There was a large side-to-side communication between the superior vena cava and the pulmonary vein resulting in partially anomalous pulmonary venous drainage. A side-biting clamp was applied in the superior vena cava and the pulmonary vein at both sides of the communication, and the vein was divided. The incision in both veins was closed with a running suture.
Project description:Background:In chronic haemodialysis patients central veins occlusion occur very often. In such patients, permanent pacemaker placement implantation can be challenging and alternative approaches should be used. Case summary:This is a case of 66-year-old male patient with complete atrioventricular block after a mitral valve (MV) surgery for endocarditis. The patient has a permanent surgically inserted haemodialysis catheter in right heart atrium after several unsuccessful attempts of endovascular recanalization of superior vena cava. A lead was implanted in the right ventricle after successful endovascular revascularization of the right iliac vein. The pacemaker was placed in a pouch on the right lower abdominal wall. Discussion:To our knowledge, this is the first reported case where a permanent single-chamber pacemaker was implanted through the right iliac vein after successful endovascular recanalization in chronic haemodialysis patient post-MV replacement.
Project description:Uterine leiomyoma (UL) is the most common benign smooth muscle tumour of the premenopausal women. Rarely it shows malignant behaviour by metastasizing through the pelvic veins into systemic veins, inferior vena cava (IVC), there it is termed as intravenous leiomyomatosis (IVL). IVL may restrict itself within the IVC or it may extend into right heart chambers reaching up to pulmonary arteries. Here we report a case of single staged excision of intracardiac(IC) extension of IVL of a 45 -year -old premenopausal women, who have undergone abdominal hysterectomy five years ago, with the complaints of shortness of breath aggravated on bending forward for the past two years.
Project description:A 71-year-old woman presented with a right adnexal solid mass invading the right gonadal vein and inferior vena cava up to the hepatic veins revealed by CT and confirmed by MRI. A thin-walled cyst and a solid mass were unexpectedly found in the right atrium by transesophageal echocardiography (TEE) in the operating room. Using color Doppler and air bubbles as contrast material a circumscribed cyst was confirmed and localized close to the IVC. The cyst was connected to the mass in the inferior vena cava. The tumor, including the cyst, was removed without using cardiopulmonary bypass and described as a low-grade endometrial stromal sarcoma, a rare slowly growing tumor. This is the first TEE description of endometrial stromal sarcoma manifesting as a right atrial cyst.
Project description:To characterize a expression patterns in the heart, we used rat. 12 Wistar male rats (8 - 11 weeks) were sacrificed. Left atrium (LA) adjacent to the pulmonary vein (PV), a mass of left ventricle (LV), and free-wall of the right ventricle (RV) was isolated. Each LV mass was dissected into three pieces as samples. Because the SAN isolation procedure takes approximately 20 minutes, SA was isolated separately. The SA region was delimited by the borders of the crista terminalis, the interatrial septum, the superior vena cava, and right atrium (RA). In addition to PV, LV, RV, SA, and RA samples, pulmonary arteries were added to the samples for the rat microarray. Overall design: 23 samples
Project description:Invasion of a renal cell carcinoma thrombus into the inferior vena cava and right atrium is infrequent. Reaching and completely excising a tumor from the inferior vena cava is particularly challenging because the liver covers the surgical field. We report the case of a 61-year-old man who underwent surgery for a renal cell carcinoma of the right kidney that extended into the inferior vena cava and right atrium. During dissection of the liver to expose the inferior vena cava, transesophageal echocardiograms revealed right atrial mass migration into the tricuspid valve. On emergency sternotomy, the tumor embolized into the main pulmonary artery. We used a selective upper-body perfusion technique involving moderately hypothermic cardiopulmonary bypass, cardioplegic arrest, and clamping of the descending aorta, which provided a bloodless surgical field for precise removal of the mass and resulted in minimal blood loss. Our technique might be useful in other patients with tumor thrombus extending into the right atrium because it reduces the need for transfusion and avoids the deleterious effects of deep hypothermic circulatory arrest. Our case also illustrates the importance of continuous transesophageal echocardiographic monitoring to detect thrombus embolization.
Project description:Scimitar syndrome is a rare congenital anomaly where the right pulmonary veins return to the inferior vena cava (IVC) just below the diaphragm. On chest X-ray (CXR), an IVC catheter will be in a bizarre location outside the heart if it inadvertently passes into the scimitar vein rather than into the right atrium.