RESUMO
An 11-year-old, female, spayed, soft-coated Wheaten terrier presented for acute onset of neurological signs. On presentation, neurological examination showed right thoracic and pelvic limb proprioceptive deficits, absent right menace reflex, and weak right nasal septum response. A left thalamocortical lesion was localized. On thoracic auscultation, an arrhythmia was noted, and electrocardiography showed frequent ventricular premature complexes and rare runs of ventricular tachycardia. Echocardiography identified an interventricular septal mass extending into the lumen of the left ventricle. Thalamocortical metastasis secondary to the cardiac mass was suspected to be the cause of the patient's neurological signs. Humane euthanasia was elected by the owner due to the patients clinical status and poor prognosis. A postmortem examination diagnosed hemangiosarcoma of the interventricular septum, the right ventricular free wall, and left ventricular free wall. The left ventricle adjacent to the paraconal groove showed myocardial necrosis and inflammation. Metastases to the brain and secondary intracranial hemorrhage were found which were suspected to be the cause of the antemortem neurological signs. Concurrent pulmonary and hepatic metastases were noted. This report describes a rare presentation of an intracardiac hemangiosarcoma of the interventricular septum, right ventricle, and left ventricle in a patient presenting with neurological signs.
Assuntos
Neoplasias Encefálicas , Doenças do Cão , Neoplasias Cardíacas , Hemangiossarcoma , Cães , Neoplasias Cardíacas/veterinária , Neoplasias Cardíacas/secundário , Neoplasias Cardíacas/patologia , Hemangiossarcoma/veterinária , Hemangiossarcoma/patologia , Hemangiossarcoma/secundário , Doenças do Cão/patologia , Doenças do Cão/diagnóstico , Animais , Feminino , Neoplasias Encefálicas/veterinária , Neoplasias Encefálicas/secundário , Neoplasias Encefálicas/patologia , Ventrículos do Coração/patologia , Ecocardiografia/veterinária , Evolução FatalRESUMO
Benign cardiac neoplasms are relatively uncommon. Cardiac leiomyomas are usually diagnosed as a benign metastasizing leiomyoma or as a part of intravenous leiomyomatosis spectrum. Primary cardiac leiomyomas are extremely rare and should be diagnosed after ruling out the involvement of systemic leiomyomas. Only nine cases were found in the literature that described De novo occurrence of primary intra-cardiac leiomyoma. In this study, we present a case of 60-year-old female patient with a large pedunculated mass located in the left ventricle. Histopathology examination and immunohistochemistry aid confirmed the diagnosis of benign leiomyoma. No evidence of extra cardiac lesions was detected in the patient. The patient remained healthy with no signs of recurrence four years after the surgical resection. Benign cardiac tumors are not often seen, but when they do occur, they can present a serious risk to life. This is particularly significant because these tumors can detach and cause embolization, leading to the development of strokes. Moreover, these individuals do not show any clinical symptoms, making their detection quite challenging. When there is a suspicion, it is advised to utilize echocardiography and other imaging techniques to verify the presence of a tumor. In this report, we present a rare case and provide differential diagnoses, along with a review of the literature.
Assuntos
Neoplasias Cardíacas , Leiomioma , Humanos , Feminino , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/cirurgia , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/diagnóstico por imagem , Pessoa de Meia-Idade , Leiomioma/cirurgia , Leiomioma/diagnóstico , Leiomioma/patologia , Leiomioma/diagnóstico por imagem , Ecocardiografia , Ventrículos do Coração/diagnóstico por imagem , Diagnóstico DiferencialRESUMO
BACKGROUND: To report the diagnosis and treatment of a rare disease of intravenous leiomyomatosis (IVL) originating from the uterus, growing in the inferior vena cava (IVC) and extending into the right atrium (RA) associated with a pelvic arteriovenous fistula (AVF). This is the first reported case of IVL in the IVC and RA with pulmonary benign metastasizing leiomyoma (PBML) secondary to a pelvic AVF despite the use of GnRH agonists in a nonmenopausal woman. CASE PRESENTATION: The patient was a 50-year-old premenopausal woman with a history of surgical resection for and antiestrogen conservative drug for pulmonary benign metastasizing leiomyoma (PBML) 5 years. The patient nevertheless developed IVL in the IVC, internal iliac vein and RA accompanied by AVF. Vaginal ultrasound combined with echocardiography and computerized tomographic venography imaging assists in the diagnosis of IVL combined with AVF, with histopathology and immunohistochemistry ultimately confirming the diagnosis. The patient ultimately was performed with a combination of hysterectomy, bilateral adnexectomy, and resection of tumors in the IVC and RA without cardiopulmonary bypass and sternotomy. CONCLUSION: BML may be difficult to control with incomplete removal of the uterus and ovaries even with the use of antiestrogenic medications, and medically induced AVF resulting from fibroid surgery may accelerate this process and the development of IVL.
Assuntos
Fístula Arteriovenosa , Átrios do Coração , Leiomiomatose , Neoplasias Pulmonares , Neoplasias Uterinas , Neoplasias Vasculares , Veia Cava Inferior , Humanos , Feminino , Veia Cava Inferior/patologia , Veia Cava Inferior/cirurgia , Veia Cava Inferior/diagnóstico por imagem , Pessoa de Meia-Idade , Neoplasias Uterinas/patologia , Neoplasias Uterinas/cirurgia , Fístula Arteriovenosa/cirurgia , Fístula Arteriovenosa/etiologia , Fístula Arteriovenosa/diagnóstico por imagem , Fístula Arteriovenosa/patologia , Átrios do Coração/patologia , Átrios do Coração/cirurgia , Átrios do Coração/diagnóstico por imagem , Leiomiomatose/patologia , Leiomiomatose/cirurgia , Leiomiomatose/diagnóstico por imagem , Neoplasias Pulmonares/secundário , Neoplasias Pulmonares/patologia , Neoplasias Vasculares/patologia , Neoplasias Vasculares/cirurgia , Neoplasias Vasculares/diagnóstico por imagem , Neoplasias Cardíacas/secundário , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/cirurgia , Neoplasias Cardíacas/complicações , Resultado do Tratamento , Histerectomia , Veia Ilíaca/patologia , Veia Ilíaca/diagnóstico por imagemRESUMO
BACKGROUND: Primary cardiac synovial sarcoma is a rare condition with limited treatment options for advanced stages. Surgery and chemotherapy are currently the mainstay treatments; however, survival rates remain low. CASE PRESENTATION: A 64-year-old woman presenting with symptoms of chest tightness and shortness of breath was found to have an obstructive right atrial mass, along with pulmonary infarction and metastasis. She was ultimately diagnosed with advanced primary cardiac synovial sarcoma. Following surgery, the patient's symptoms improved, and she underwent chemotherapy and anti-angiogenic therapy, but unfortunately, her survival time was only 8 months. CONCLUSION: This case report aims to enhance clinicians' understanding of the diagnosis and treatment of primary cardiac synovial sarcoma. Enhancing both survival outcomes and quality of life in individuals with primary cardiac synovial sarcoma continues to present a significant challenge.
Assuntos
Neoplasias Cardíacas , Infarto Pulmonar , Sarcoma Sinovial , Humanos , Sarcoma Sinovial/patologia , Sarcoma Sinovial/complicações , Sarcoma Sinovial/diagnóstico , Feminino , Pessoa de Meia-Idade , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/complicações , Infarto Pulmonar/patologia , Infarto Pulmonar/etiologia , Septo Interatrial/patologiaAssuntos
Cardiomiopatia Restritiva , Neoplasias Cardíacas , Hemangiossarcoma , Humanos , Hemangiossarcoma/patologia , Hemangiossarcoma/complicações , Hemangiossarcoma/diagnóstico , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico por imagem , Cardiomiopatia Restritiva/patologia , Cardiomiopatia Restritiva/etiologia , Masculino , Pessoa de Meia-Idade , Feminino , Evolução FatalRESUMO
BACKGROUND Cervical cancer ranks fourth globally among women's cancers. Squamous cell carcinoma constitutes 70% of cervical cancer cases, often metastasizing to lungs and paraaortic nodes. Uncommon sites include the brain, skin, spleen, and muscle, while pericardial fluid metastasis is highly rare. We report a case of squamous cell carcinoma of the uterine cervix that was metastatic to the pericardium and was detected on cytologic evaluation of pericardial fluid. CASE REPORT A 42-year-old woman who was previously treated for stage III squamous cell carcinoma of the cervix presented with symptoms of cough, fever, and shortness of breath for 8 days, and chest pain for 3 days. Clinical workup revealed pericardial effusion, with spread to the lungs and mediastinal and hilar lymph nodes. Cytological analysis of the fluid showed malignant cells, consistent with metastatic squamous cell carcinoma. Immunohistochemistry demonstrated cells positive for p63 and p40, while negative for GATA-3, D2-40, calretinin, and WT1. These findings in conjunction with patient's known history of cervical squamous cell carcinoma was consistent with a cytologic diagnosis of metastatic squamous cell carcinoma to pericardial fluid. CONCLUSIONS History and clinical correlation plays a vital role in determining the primary site causing malignant pericardial effusions. While the occurrence of cervical cancer metastasizing to the pericardium is uncommon, it should be considered, particularly in cases involving high-grade, invasive tumors, recurrences, or distant metastases. This possibility should be included in the list of potential diagnoses when encountering pericardial effusions with squamous cells in female patients.
Assuntos
Carcinoma de Células Escamosas , Derrame Pericárdico , Neoplasias do Colo do Útero , Humanos , Feminino , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/secundário , Carcinoma de Células Escamosas/complicações , Derrame Pericárdico/etiologia , Neoplasias do Colo do Útero/patologia , Neoplasias do Colo do Útero/complicações , Adulto , Neoplasias Cardíacas/secundário , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/patologiaRESUMO
The antemortem diagnosis of secondary cardiac involvement by lymphoma remains suboptimal. Prognosis is worse with delayed diagnosis as the tumor burden increases with the multicompartment participation. Chemotherapy may improve survival, but there is a risk of mortality due to treatment-related complications, such as myocardial rupture and fatal arrhythmias. Modified chemotherapy regimens may prevent such complications, but the data are limited. We report the case of a 72-year-old woman diagnosed with diffuse large B-cell lymphoma with cardiac involvement, where early diagnosis prevented cardiac complications from the disease and its treatment as well. The aim of this case report is to highlight the fact that cardiac involvement in lymphoma is frequent and can be easily missed, leading to complications. Treatment requires an individualized approach.
Assuntos
Neoplasias Cardíacas , Linfoma Difuso de Grandes Células B , Humanos , Feminino , Idoso , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/complicações , Ecocardiografia , Septo Interatrial/patologia , Septo Interatrial/diagnóstico por imagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêuticoRESUMO
Transthoracic echocardiography and cardiac magnetic resonance imaging revealed a well-defined globular mass attached to the anterolateral papillary muscle. The mass was hyperintense on T1 and T2 weighted images with suppression of signal on fat saturated and short tau inversion recovery (STIR) images. This imaging established the diagnosis of cardiac lipoma attached to the anterolateral papillary muscle, Papillary muscle is a very rare location of lipoma, which is rarely reported in the literature.
Assuntos
Neoplasias Cardíacas , Lipoma , Imageamento por Ressonância Magnética , Músculos Papilares , Humanos , Músculos Papilares/diagnóstico por imagem , Músculos Papilares/patologia , Músculos Papilares/cirurgia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Neoplasias Cardíacas/patologia , Lipoma/diagnóstico por imagem , Lipoma/cirurgia , Lipoma/patologia , Ecocardiografia , Masculino , Pessoa de Meia-Idade , Feminino , Valor Preditivo dos TestesRESUMO
Cardiac myxoma is a commonly encountered tumor within the heart that has the potential to be life-threatening. However, the cellular composition of this condition is still not well understood. To fill this gap, we analyzed 75,641 cells from cardiac myxoma tissues based on single-cell sequencing. We defined a population of myxoma cells, which exhibited a resemblance to fibroblasts, yet they were distinguished by an increased expression of phosphodiesterases and genes associated with cell proliferation, differentiation, and adhesion. The clinical relevance of the cell populations indicated a higher proportion of myxoma cells and M2-like macrophage infiltration, along with their enhanced spatial interaction, were found to significantly contribute to the occurrence of embolism. The immune cells surrounding the myxoma exhibit inhibitory characteristics, with impaired function of T cells characterized by the expression of GZMK and TOX, along with a substantial infiltration of tumor-promoting macrophages expressed growth factors such as PDGFC. Furthermore, in vitro co-culture experiments showed that macrophages promoted the growth of myxoma cells significantly. In summary, this study presents a comprehensive single-cell atlas of cardiac myxoma, highlighting the heterogeneity of myxoma cells and their collaborative impact on immune cells. These findings shed light on the complex pathobiology of cardiac myxoma and present potential targets for intervention.
Assuntos
Neoplasias Cardíacas , Mixoma , Microambiente Tumoral , Humanos , Mixoma/patologia , Mixoma/genética , Mixoma/imunologia , Microambiente Tumoral/imunologia , Microambiente Tumoral/genética , Neoplasias Cardíacas/genética , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/imunologia , Macrófagos/imunologia , Macrófagos/patologia , Proliferação de Células/genética , Masculino , FemininoAssuntos
Achados Incidentais , Imagem Multimodal , Humanos , Feminino , Fibroelastoma Papilar Cardíaco/diagnóstico por imagem , Fibroelastoma Papilar Cardíaco/patologia , Fibroelastoma Papilar Cardíaco/cirurgia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/patologia , Pessoa de Meia-Idade , Fibroma/diagnóstico por imagem , Fibroma/patologia , Fibroma/cirurgiaRESUMO
ABSTRACT: Lipomatous hypertrophy of the interatrial septum (LHIS) is a rare but benign cardiac tumor that can be found on cardiac imaging such as echocardiogram, or during surgery or an autopsy. Cardiac MRI is the best imaging modality to determine the borders of the tumor and its extension into the intraventricular septum and ventricular free wall. Patients require close monitoring because LHIS may cause right or left outflow tract obstruction or superior vena cava obstruction, requiring cardiac surgical intervention. This article describes a patient with LHIS who underwent cardiac surgery because of her increasing symptomatology.
Assuntos
Septo Interatrial , Neoplasias Cardíacas , Lipomatose , Humanos , Feminino , Septo Interatrial/diagnóstico por imagem , Septo Interatrial/patologia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/patologia , Lipomatose/diagnóstico por imagem , Lipomatose/diagnóstico , Lipomatose/cirurgia , Imageamento por Ressonância Magnética , Hipertrofia , Ecocardiografia , Pessoa de Meia-Idade , Lipoma/cirurgia , Lipoma/diagnóstico por imagem , Lipoma/diagnósticoRESUMO
Generally, sarcomas arising from benign soft tissue are rare. Cardiac myxoma (CM) is a benign tumor, and few reports have described its malignant transformation. Herein, we documented a case of an 89-year-old man with prostate cancer and a 5-year history of a right atrium tumor without Carney complex. The tumor was resected surgically and had a myxomatous or gelatinous appearance. Microscopically, the tumor had two components: a sarcomatous area and myxomatous area. In the myxomatous area, typical myxoma cells were demonstrable and were strongly immunoreactive for immunohistochemistry (IHC) of calretinin. In the sarcomatous area, the epithelioid- to spindle-shaped cells with prominent atypia proliferated densely. The IHC profile of cells in the sarcomatous area was different from that of cells in the myxomatous area; MDM2-positive cells were found only in the sarcomatous area. Especially, the Ki-67 index and number of p53-positive cells in the sarcomatous area were higher than those in the myxomatous area. The transition of the two components was seamless. Thus, we made a diagnosis of CM with malignant transformation corresponding to undifferentiated pleomorphic sarcomas. This case suggests that CM may transform into sarcoma, albeit rarely.
Assuntos
Biomarcadores Tumorais , Transformação Celular Neoplásica , Átrios do Coração , Neoplasias Cardíacas , Imuno-Histoquímica , Mixoma , Sarcoma , Humanos , Masculino , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/cirurgia , Mixoma/patologia , Mixoma/cirurgia , Transformação Celular Neoplásica/patologia , Sarcoma/patologia , Sarcoma/cirurgia , Sarcoma/química , Átrios do Coração/patologia , Átrios do Coração/cirurgia , Átrios do Coração/química , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/análise , Biomarcadores Tumorais/metabolismo , Proteínas Proto-Oncogênicas c-mdm2/análise , Proteínas Proto-Oncogênicas c-mdm2/metabolismo , Neoplasias da Próstata/patologia , Neoplasias da Próstata/cirurgiaRESUMO
A 78-year-old Japanese man presented to the emergency department with a sore throat and fever that worsened over 3 weeks. A tonsil biopsy led to the diagnosis of pleomorphic mantle cell lymphoma (MCL) that had infiltrated the right adrenal gland, inferior vena cava, and right atrium (RA). Although the patient's cardiac tumor had high mobility, his hemodynamic state was stable, and he did not present with fatal arrhythmia. Therefore, we first introduced chemotherapy. However, the patient developed recurrent pulmonary embolisms (PEs) and died after starting chemotherapy. An autopsy revealed that the MCL had invaded the large vessels, causing the PEs. Although the high mobility of cardiac tumors is known to increase the risk of PE in diffuse large B-cell lymphoma (DLBCL), optimal management of cardiac MCL remains to be elucidated owing to its rarity. To the best of our knowledge, this is the first report of cardiac MCL with posttreatment PE development in a Japanese patient. It is worth considering preventive surgery before treatment not only in DLBCL, but also in MCL based on the mobility of the cardiac tumors. Our case highlights the need for close communication between hematologists and cardiologists to treat cardiac MCL.
Assuntos
Autopsia , Neoplasias Cardíacas , Linfoma de Célula do Manto , Embolia Pulmonar , Humanos , Masculino , Idoso , Linfoma de Célula do Manto/tratamento farmacológico , Linfoma de Célula do Manto/complicações , Linfoma de Célula do Manto/patologia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/patologia , Embolia Pulmonar/etiologia , Embolia Pulmonar/induzido quimicamente , Embolia Pulmonar/patologia , Evolução FatalAssuntos
Átrios do Coração , Neoplasias Cardíacas , Sarcoma , Humanos , Átrios do Coração/patologia , Átrios do Coração/diagnóstico por imagem , Sarcoma/patologia , Sarcoma/cirurgia , Sarcoma/diagnóstico , Sarcoma/diagnóstico por imagem , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Túnica Íntima/patologia , Túnica Íntima/diagnóstico por imagem , Masculino , Feminino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Primary cardiac tumors, while rare, present significant clinical challenges due to their diverse pathology and presentation. Lung cancer frequently metastasizes to the heart; however, cases involving primary cardiac tumors of different origins alongside primary lung cancer are exceedingly rare in the literature. CASE PRESENTATION: We report the case of a 53-year-old female who presented with hemoptysis and was subsequently diagnosed with a left atrial myxoma, pulmonary squamous cell carcinoma, and a thymic cyst. This coexistence of multiple non-homologous tumors in a single patient is exceedingly rare. CONCLUSION: This case underscores the complexity of diagnosing and managing patients with multiple distinct tumors. The simultaneous occurrence of a primary cardiac myxoma, pulmonary squamous cell carcinoma, and thymic cyst is unprecedented, providing valuable insights for future clinical practice.
Assuntos
Carcinoma de Células Escamosas , Átrios do Coração , Neoplasias Cardíacas , Neoplasias Pulmonares , Cisto Mediastínico , Mixoma , Humanos , Mixoma/complicações , Mixoma/cirurgia , Mixoma/patologia , Feminino , Pessoa de Meia-Idade , Carcinoma de Células Escamosas/cirurgia , Carcinoma de Células Escamosas/complicações , Carcinoma de Células Escamosas/patologia , Neoplasias Pulmonares/cirurgia , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/patologia , Cisto Mediastínico/cirurgia , Cisto Mediastínico/complicações , Cisto Mediastínico/patologia , Neoplasias Cardíacas/cirurgia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/patologia , Átrios do Coração/patologia , Átrios do Coração/cirurgia , Neoplasias Primárias Múltiplas/cirurgia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Primárias Múltiplas/complicaçõesRESUMO
Cardiac tumors are rare and encompass a variety of presentations. Clinica symptoms are usually nonspecific, but they can present as obstructive, embolic, or constitutional symptoms. Treatment options and prognosis vary highly depending on the subtype, tumor size, and location. Surgical resection is usually the first-line therapy, except for cardiac lymphomas, and provides favorable long-term prognosis in most benign tumors. Cardiac sarcomas, however, are usually diagnosed in advanced stages, and the treatment relies on a multimodal approach with chemotherapy and radiotherapy. Metastatic cardiac tumors are usually related to advanced disease and carry an overall poor prognosis.
Assuntos
Neoplasias Cardíacas , Sarcoma , Humanos , Neoplasias Cardíacas/terapia , Neoplasias Cardíacas/patologia , Neoplasias Cardíacas/diagnóstico por imagem , Sarcoma/terapia , Sarcoma/patologia , PrognósticoAssuntos
Fibroelastoma Papilar Cardíaco , Humanos , Fibroelastoma Papilar Cardíaco/cirurgia , Fibroelastoma Papilar Cardíaco/patologia , Fibroelastoma Papilar Cardíaco/diagnóstico por imagem , Fibroelastoma Papilar Cardíaco/diagnóstico , Feminino , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Neoplasias Cardíacas/patologia , Masculino , Fibroma/cirurgia , Fibroma/patologia , AdultoAssuntos
Neoplasias do Endométrio , Neoplasias Cardíacas , Sarcoma do Estroma Endometrial , Veia Cava Inferior , Humanos , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/patologia , Feminino , Sarcoma do Estroma Endometrial/patologia , Sarcoma do Estroma Endometrial/diagnóstico por imagem , Neoplasias do Endométrio/patologia , Neoplasias do Endométrio/cirurgia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/patologia , Invasividade Neoplásica , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Surgery is the only curative treatment for retrohepatic inferior vena cava (r-IVC) leiomyosarcoma.1 Cavo-hepatic confluence invasion is a poor prognostic situation, requiring extreme liver surgery for selected patients to achieve R0 margins (a crucial prognostic factor). Ex situ liver resection and autotransplantation (ELRA), developed by Pichlmayr et al., permits to achieve such R0 margin.2,3 METHODS: An 84-year-old patient in excellent condition (ECOG 0), without relevant past medical history, was referred for abdominal mass, bilateral lower limbs edema, and dyspnea. Workup revealed a large r-IVC leiomyosarcoma invading cavo-hepatic confluence and protruding in right atrium without any metastasis. After multidisciplinary consultation, surgical treatment was retained. Preoperative transoesophaegal echocardiography confirmed a 4-cm protruding tumoral thrombus in right atrium without abdominalisation possibility. RESULTS: A sterno-laparotomy was performed, consisting of a right nephrectomy for exposure and en bloc total hepatectomy comprising r-IVC after atriotomy for intracardiac thrombectomy under extracorporeal circulation. Tumorectomy (rIVC + segment I and IX) was performed on back table followed by a r-IVC reconstruction through a tubulized homologous venous patch. Native IVC was reconstructed as well, permitting a side-to-side cavo-caval anastomosis for liver reimplantation. Postoperative evolution was eventless except for an early bile leak that required surgical exploration. The patient was discharged on postoperative day 32. Pathological examination confirmed r-IVC-leiomyosarcoma T4N0M0 R0, FNCLCC grade 2. Eight months after surgery, general status was conserved with disappearance of symptoms, and IVC was permeable without leiomyosarcoma recurrence. CONCLUSION: Ex situ liver resection and autotransplantation with atrial thrombectomy is a surgical possibility for R0 r-IVC leiomyosarcoma invading cavo-hepatic confluence in selected patients.