Intracardiac Extension Research Articles

Echocardiographic Characteristics and Contrast-Enhanced Imaging of Intravenous Leiomyomatosis With Intracardiac Extension.

Intravenous leiomyomatosis (IVL) is a histologically benign but biologically aggressive tumor. This study aimed to summarize the echocardiography and contrast-enhanced ultrasound (CEU) characteristics of IVL to provide a basis for clinical diagnosis and therapy. Fourteen IVL patients with uterus leiomyoma history (female, 46.4 ± 5.6 years) were enrolled in this study from March 2008 to December 2020 in our hospital. Preoperative imaging examination data were collected, including echocardiography computed tomography data; six patients also underwent CEU. All patients underwent successful resection, confirmed by histopathology. Echocardiographic characteristics: The mean sizes of intracardiac parts of IVL tumors were 54.0 ± 17.9 mm (length) and 24.6 ± 9.8 mm (width). IVL tumors exhibited two echocardiography types: isoechoic solid mass (71.4%, 10/14) and anechoic cystic conduits (28.6%, 4/14), with enlargements of the right atrium (57.1%,8/14), right ventricle (1 patient, 7.1%), and inferior vena cava (57.1%, 8/14). About 21.4% of the patients (3/14) had right ventricular dysfunction. Right heart obstruction was observed in 42.8% (6/14) of the patients. CEU characteristics: the solid mass type exhibited an earlier perfusion and lower perfusion intensity than the conduits type. CEU was helpful in determining origins and pathways: from the internal iliac vein (pathway I, 71.4%), from the ovarian vein (pathway II, 14.3%), or both (14.3%). The echocardiographic appearances of the 14 cases were consistent with the features of the resection specimens. Combined echocardiography and CEU can provide a more valuable information for the diagnosis of IVL and essential basis for treatment.

Thymic carcinoma with extrinsic occlusion of the left anterior descending artery: a distinctive case of myocardial infarction in a young woman

BackgroundThymic epithelial tumour (TET) is the most common tumour affecting the anterior mediastinum in adults. The cardiac extension is often limited to the pericardium, and intracardiac extension is rare. We present a unique case of encasement and displacement of the left anterior descending coronary artery by the large mediastinal tumour leading to myocardial ischemia.Case presentationOur patient is a 28-year-old lady with stage 4 TET. She presented with acute chest pain associated with 12-lead ECG changes and a significant rise in serial troponin I. Multimodality cardiac imaging revealed encasement and displacement of the left anterior descending coronary artery by the large mediastinal tumour. CT-FFR demonstrates evidence of ischemia which would account for her acute presentation. Following detailed MDT discussions between cardiologists, oncologists and cardiothoracic surgeons, the decision was made to treat this lady with palliative chemotherapy. Given the extent of the tumour invasion and failure of the initial therapy, her prognosis and the outcome were poor.ConclusionsTET could cause atrial compression, myocardial infiltration, and invasion of the pulmonary and caval veins; however, to the best of our knowledge, this is the first case reported of coronary artery displacement and encasement by TET.

Intravenous Leiomyoma with Intracardiac Extension: A Rare Late Presentation After Hysterectomy

Intravenous leiomyoma is a rare, benign smooth muscle tumor arising from intrauterine venules or the myometrium. It can rarely present as intracardiac mass long after hysterectomy. In this case report we describe a 40-year-old female with previous history of hysterectomy, presenting with an intracardiac mass which was successfully managed with single stage tumor resection under cardiopulmonary bypass. Subsequent histopathology showed features of leiomyoma. The diagnosis of intravenous leiomyoma with cardiac extension should be kept in a female patient presenting with intracardiac mass with previous history of myomectomy or hysterectomy.

Surgical treatment of intravenous leiomyomatosis with inferior vena cava and intracardiac extension

Leiomyoma is the most frequent neoplasm in the female genital tract; however, leiomyoma located intravenously with intracardiac extension is a rare entity that necessitates surgical excision to alleviate symptoms and prevent life-threating complications. We present a case of a retroperitoneal leiomyoma invading the inferior vena cava (IVC) and extending to the right ventricle.

Pulmonary Vein Tumor Thrombus With Intracardiac Extension Secondary to Poorly Differentiated Bronchogenic Carcinoma

Pulmonary vein thrombosis (PVT) is a rarely encountered disease entity with varied clinical presentations. It has been reported to be associated with underlying lung malignancy in multiple case reports. Diagnosis can be challenging due to nonspecific symptoms on presentation. Herein, we report a 67-year-old male patient with a history of extensive smoking and chronic obstructive pulmonary disease (COPD) who presented with multiple hemoptysis episodes. CT scan of the chest with contrast showed multiple right lower lobe (RLL) lung masses and a thrombus in the inferior pulmonary vein. After various imaging modalities and transthoracic biopsy of the lung mass, the patient was diagnosed with pulmonary vein tumor thrombus secondary to poorly differentiated bronchogenic carcinoma with intracardiac extension. The patient was started on Eliquis for anticoagulation and is currently in the process of beginning chemo/radiation therapy for the underlying malignancy.

Pulmonary Artery Sarcoma is a Predominantly Bilateral Disease: Insight from a 55 Case Single Center Experience

Pulmonary artery sarcoma (PAS) is an exceedingly rare disease with historically poor survival. PAS is often mistaken for chronic thromboembolic pulmonary hypertension (CTEPH) owing to similarities in clinical presentation. Controversy exists regarding need to definitively differentiate PAS from CTEPH prior to surgical therapy. Furthermore lung resection would seem inferior palliation to tumor endarterectomy in a predominantly bilateral disease dominated by hemodynamic compromise. Retrospective review of all patients who underwent pulmonary endarterectomy (PEA) and tumor resection surgery at a single institution between March 1992 and February 2019, identified 55 patients diagnosed with PAS. In this series of 55 patients (23 male, 32 female, age 23-82 years), median time from symptom onset to diagnosis was 8 months (IQR 5-12 months). Many patients were severely limited on initial assessment (median WHO Functional Class III). Forty-six patients (83.6%) had suspected PAS diagnosis prior to surgery. Preoperative testing identified bilateral disease (37/50 V/Q scans, 39/46 CTA) and PA mass (35/55) in majority of cases. Fifty patients (90.9%) had pathologically-confirmed bilateral pulmonary involvement, while 20 also had valvular or intracardiac tumor extension resected at time of surgery. A majority of sarcomas (87%) were classified as undifferentiated or intimal subtype and 64% were high-grade. PEA resulted in significant improvement in hemodynamics [mPAP decreased by 6 mmHg (p < 0.001), CO increased by 1.2 L/min (p<0.001), PVR decreased by 332 dynes/sec/cm5 (p<0.001)]. 30-day mortality was 10.9%. Overall median survival was 13 months following surgery (n=53), but increased to 23.5 months in those receiving adjuvant therapy (n=23). PAS is a rare disease with poor prognosis. Tumor endarterectomy may alleviate symptoms and increase life-expectancy. Knowledge that PAS is a predominantly bilateral disease (90.9% in our series), suggests lung resection would rarely if ever be curative, whereas PEA can be diagnostic and effective treatment of hemodynamic compromise. Furthermore, earlier surgical treatment may enable earlier referral and tolerability of adjuvant chemoradiation or immunotherapy resulting in improved survival.

Intravenous leiomyomatosis with intracardiac extension managed by one-stage surgical excision: a case presentation

BackgroundIntravenous leiomyomatosis is characterized by the extension of pathologically benign smooth muscle tumor into venous channels. Its extension into the heart is a very rare occurrence, which may lead to heart failure and occasionally sudden death. Due to its rarity, the condition is poorly understood and often misdiagnosed.Case presentationWe report a case of intravenous leiomyomatosis with intracardiac extension in a 40 years old woman who underwent hysterectomy for uterine leiomyoma (one year earlier), managed successfully through one-stage surgical approach with main focus on its imaging features.ConclusionThe diagnosis of intravenous leiomyomatosis should be considered in women who present with cardiac symptoms associated with a right atrial mass and who also have a pelvic mass or have previously undergone hysterectomy because of uterine leiomyoma.

P1696 Intravascular leiomyomatosis with inferior vena cava and intracardiac extension, with severe obstruccion to the right ventricle.The fundamental role of transesophageal echocardiography

Abstract Introduction Intravascular leiomyomatosis (IL) is a rare type of tumor common and was first described by Birch-Hirschfeld in 1896. There are currently few cases reported in the literature involving this type of vena cava tumor and the right atrium. This tumors are characterized by nodular, intravascular, histologically benign masses of smooth muscle with variable extensión. The most frequently affected women are pre-menopausal and multiparous. There are no specific clinical manifestations of vascular leiomyomas. Clinical case This is 51-year-old woman with a history of morbid obesity (body mass index of 47kg / m2SC) in bariatric surgery protocol, mixed dyslipidemia, is presented to the emergency service of our Institute, with uterine myomatosis diagnosed 6 months prior to admission. She was admitted for a 2-week evolution characterized by dyspnea of medium efforts that progressed at rest on the day of his admission with acute heart failure. A transthoracic and transesophageal echocardiograhy three-dimensional (TTE, TEE 3D) was made in which a solid-appearing, regular-border, heterogeneous tumor was reported from the inferior vena cava to the right atrium without being attached to the interatrial septum or other structures, with severe protrusion to the right ventricle, highly mobile, heterogeneous, without vascularity with color doppler, causing intermittent obstruction and tricuspid stenosis with a mean gradient of 8mmHg, pulmonary artery systolic pressure calculated at 46 mmHg. TAC showed invasion from the uterine leiomyoma to the inferior vena cava and right atrium. The patient was taken to emergency surgery, the vascular surgery and cardiothoracic surgery performed a long intervention. Macroscopic description reported uterine tumor of 25x19x18cm. Weight of 3.90Kg, with multiple nodular lesions, the largest being 18x15cm. Intracavitary tumor with fragment of tissue. The pathological diagnosis was uterine leiomyomatosis, intravascular leiomyomatosis in right periadnexal vessels. The patient was taken to intensive therapy, although the postoperative period was prolonged, she was discharged in functional class II NYHA. Discussion This case demonstrates the importance of recognizing this rare disease and suspecting it in patients who present with the a symptoms and the role of the echocardiography is fundamental for the characterization of the tumor and the invasion of neighboring structures. The reported cases of intravascular leiomyomas with intracardiac extension are approximately 10% and to date, less than 300 cases have been reported in the literatura. Vascular leiomyomas arise from uterine leiomyomas with vascular invasión, the etiology still remains unknown, it has been associated with high levels of estrogen. The key in its treatment is the complete resection. The prognosis is good after its resection. The recurrence rate is up to 30%. Anti-estrogenic therapy has been considered but its efficacy is controversial. Abstract P1696 Figure. Intravascular leiomyomatosis

P169 Uterine leiomyoma extension into right side cardiac

Abstract Funding Acknowledgements governmental grants Uterine leiomyoma is a commom disease in women, however, intravenous leiomyomatosis of uterine origin extending via inferior vena cava into the right side of the heart,known as intracardiac leiomyomatosis is a rare condition (3%). In 1907, Durk reported the first case of intracardiac extension. The patient was a Woman, 35 years old, admitted to our emergency department for an intracardiac mass. She had shortness of breath,fatigue and chest pain. The transthoracic two dimensional echocardiography showed an echogenic oval mass mobile in right atrium and projected through right ventricle in diastole. This mass was observed to extend from inferior vena cava to the right atrium. The echotransesophageal three dimensional showed a large mobile mass that extended from inferior vena cava to the right atrium. A Computer tomographic (CT) scan showed a hypodense multilobulated mass in the pelvis, which had invaded the inferior vena cava and right atrium. The patient underwent a two stages surgery. In first stage (transatrial tumor resection). The operation was performed normal temperature with establisment of cardiopulmonary bypass (CPB). Subsequently, the pathological report was confirmed uterin smooth muscle origin. The second stage surgery ( total histerectomy) was done four weeks later for removing lobulated mass uterin with dimensions 20x15x7.5cm with confirmed histopathological of leiomyoma. Because of it is nonspecific clinical presentation and rarity, an intracardiac Leiomyomatosis continues to be a misdiagnosed as either thrombus or myxoma. The cardiac imaging techniques like a transthoracic echocardiography 2d and transesophageal echocardiography 3d have been used to define the presence, extension of tumor as appearance of the mass and involvement of adjacent structures. Abstract P169 Figure. Echotransesophageal 3D (bicaval view)

Perioperative management of Wilms’ tumor with intracardiac extension: Report of two cases with review of literature

Wilms’ tumor (WT) is one of the solid tumor that affects children. It involves the kidney and may extend to the lungs and liver. WT conquers the adjacent blood vessels such as renal veins and inferior vena cava (IVC); its extension to IVC and right-sided heart chambers is unusual. Furthermore, when the tumor extends to heart chambers, its surgical management becomes challenging and demands multidisciplinary medical and surgical specialties including pediatric cardiac surgery. In this report, we discuss the surgical management and perioperative treatment of two unusual cases of WT with IVC and intracardiac extension.

Multidisciplinary Surgery in a Young Woman with Endometrial Stromal Sarcoma with Inferior Vena Cava and Intracardiac Extension

Abstract Background: Endometrial stromal sarcoma (ESS) is a rare tumor. Extension into the inferior vena cava (IVC) and heart has been described infrequently. Case: A 35-year-old female had low-gra...

Combined transthoracic echocardiography and contrast‐enhanced ultrasonography to trace intravenous leiomyomatosis with intracardiac extension

Intravenous leiomyomatosis (IVL) with intracardiac extension is a rare condition, which the benign tumor invades into the right heart through inferior vena cava. We described the findings of ultrasonography in eight patients with this disease. Transthoracic echocardiography (TTE) can reveal the intracardiac lesion burden and associated compromise. Contrast-enhanced ultrasonography (CEUS) can trace the origin of the lesion and extension. Therefore, combining TTE with CEUS is used to better characterize this complex lesion and plays a crucial role in guidance of surgical decision.

39. Uterine intravenous leiomyomatosis with intracardiac extension: Report of two cases

Background: Intravenous leiomyomatosis (IVL) is a histomorphologically benign smooth muscle neoplasm characterised by spindle-cell proliferation primarily located within the uterine and pelvic venous system, inferior vena cava, and in rare instances, extending to the heart.

A Rare Case Report of Intravenous Leiomyomatosis with Intracardiac Extension: Role of Transesophageal Echocardiography

A Rare Case Report of Intravenous Leiomyomatosis with Intracardiac Extension: Role of Transesophageal Echocardiography

‘Now You See It, Now You Don’t’: A Rare Case of Pulmonary Artery Occlusion and Circulatory Collapse Due to Intravascular Extension of a Renal Cell Carcinoma to the Right Heart With Tumour Embolisation

Intravascular extension of renal cell carcinoma to the right heart via the inferior vena cava (IVC) occurs in <1% of cases. A 47-year-old male with a known history of an inoperable right renal cell carcinoma was referred to this institution with intravascular, intracardiac extension of the tumour. On routine transthoracic echocardiography (TTE), a large multi-loculated mass (measuring 28 mm × 10 mm) was seen arising from the IVC and extending into the right atrium. The tumour mass prolapsed from the right atrium, across the tricuspid valve, entering the right ventricle during diastole. There was near complete occlusion of the IVC, as demonstrated on two-dimensional assessment and colour flow Doppler. The patient was initially treated with chemotherapy and immunotherapy, so as to avoid cardiothoracic surgery in the setting of advanced malignancy. Unfortunately, the patient re-presented to the Emergency Department with syncope and was found to be in obstructive cardiogenic shock. On TTE, it was noticed that the mass in the right-sided cardiac chambers was absent. Tumour embolisation was suspected, and an urgent computed tomography pulmonary angiogram was performed. This confirmed that the left main pulmonary artery was completely occluded with the tumour thrombus obstructing all flow to his left lung. The patient was transferred for urgent cardiothoracic surgery, during which time the tumour was successfully excised and his pulmonary artery repaired. This rare case of intravascular, intracardiac extension of a right-sided renal cell carcinoma highlights the potential catastrophic complications of intracardiac malignancies.

Massive pelvic recurrence of uterine leiomyomatosis with intracaval-intracardiac extension: video case report and literature review

BackgroundUterine leiomyomas represent the gynecological neoplasm with the highest prevalence worldwide. This apparently benign pathological entity may permeate into the venous system causing the so-called intravenous leiomyomatosis of the uterus (IVL). IVL may seldom extend to large caliber veins and reach the right cardiac chambers or pulmonary arteries and cause signs of right sided congestive heart failure and sudden death. Due to its low incidence, however, IVL with intracardiac extension is often misdiagnosed resulting in deferred treatment. No consensus has been obtained regarding the standard surgical approach to be used for this rare condition. We describe the case of a massive pelvic recurrence of uterine leiomyomatosis with intracardiac extension and provide a review of the literature, analyzing management and surgical outcomes.Case presentationWe present the case of a 46-year-old premenopausal woman presenting with lower-extremity edema, recurrent syncopes and a history of subtotal hysterectomy for multiple uterine fibroids. She was diagnosed with pelvic recurrence of uterine leiomyomatosis and IVL with cardiac involvement. A two-stage surgical excision of the intracardiac-intracaval mass and pelvic leiomyomatosis was performed. The patient had an uneventful recovery and no evidence of recurrence was observed on follow-up.ConclusionsBy virtue of the rarity of the present pathology, awareness is widely scarce and diagnosis is often delayed. Early recognition is difficult due to initial aspecific and subtle clinical manifestations. Nevertheless, suspicion should be held high in premenopausal women with known history of uterine leiomyomata, presenting with cardiovascular symptoms and evidence of a free-floating mass within the right cardiac chambers. In-depth imaging is crucial for defining its anatomical origin and relations. Prompt surgical treatment with radical excision of pelvic and intravenous leiomyomatosis guarantees favorable outcomes and excellent prognosis with low rates of recurrence, whereas delayed diagnosis and treatment exposes to increased risk of congestive heart failure and sudden death.

Intravenous leiomyomatosis with intracardiac extension.

Intravenous leiomyomatosis with intracardiac extension.

Surgical management and outcomes of 12 cases of Wilms tumour with intracardiac extension from a single centre.

To review demographics, effect of preoperative chemotherapy on tumour thrombus, imaging, operative strategy, and outcomes of 12 patients presenting with intracardiac extension of Wilms tumour thrombus. A retrospective audit was undertaken on patients with intracardiac extension of Wilms tumour. Patients were identified from the oncology database and information obtained on clinical presentation, stage, preoperative treatment, surgical procedures and complications, histology, and survival status. Ethics approval was obtained from the University of Cape Town Human Research Ethics Committee. From 1984 to 2016, 337 children with Wilms tumour were treated. Twelve (3.6%) had intracardiac extension of tumour thrombus, nine into the right atrium, and three into the right ventricle. Ultrasound, computerized tomography, magnetic resonance imaging, and echocardiograms were used to assess thrombus level. Patients were staged as stage III(8) and IV(4). All patients received preoperative chemotherapy. Thrombus retracted from the heart in two cases. One patient died preoperatively. Eleven underwent laparotomy, median sternotomy, and cardiopulmonary bypass (CPB). Four underwent cavectomy. Five required cavoatrial patches. Thrombus extending into the hepatic veins was extracted in five patients. There was one intraoperative complication and one perioperative death. Thrombus histology showed viable tumour in 9 of 11 patients. Three patients died of progressive disease. Seven patients are currently disease free. A combination of imaging is required to determine thrombus extent, and this facilitates surgical planning. Preoperative chemotherapy may cause thrombus regression, thus avoiding CPB. CPB offers appropriate conditions for safe tumour thrombus excision. Full management in centres with appropriately experienced staff and facilities for CPB is recommended.

Intra-cardiac extension of a Wilms’ tumor

Wilms᾿ tumor, or renal nephroblastoma, is the commonest malignant renal tumor in children and young adults. Intravascular and intracardiac extension of this tumor is a very rare and challenging clinical entity. The case is reported of a 6-year-old boy diagnosed with nephroblastoma, in whom the tumor involved the inferior vena cava and the right atrium. The successful multimodal surgical management consisted of combined, synchronous surgical resection of both renal and intracardiac tumors. The boy had an uneventful postoperative course and recovery, followed by relapse-free living. Intra-cardiac extension of Wilm’s tumor poses a surgical challenge where the favorable outcome is provided only by the combined and well-coordinated, skilled multidisciplinary care of pediatric surgeons and pediatric cardiac surgeons.

Patient with neuroblastoma complicated by giant thrombus of an inferior vena cava with intracardiac extension

In this article, we presented a rare case of neuroblastoma complicated by giant thrombus of an inferior vena cava with intracardiac extension in a 3 years old child.