Related Experiment Video
Updated: May 23, 2025
08:37
Echocardiographic Assessment Using Subxiphoid-Only Examination for Hypotensive Patients
Published on: April 18, 2025
234
Obstructive Shock Caused by Intimal Sarcoma With Pulmonary Vein Stenosis and SVC/IVC Obstruction
Ebenezer Daniel1, Jesu Krupa1, Anne Jennifer Prabhu2
1Department of Cardiology, Christian Medical College, Vellore, India.
JACC. Case Reports
|March 7, 2025
Summary
Intimal sarcoma, a rare cancer, can aggressively infiltrate heart chambers and major vessels, causing severe obstructive symptoms. Early diagnosis and understanding its rapid progression are crucial for patient management.
Area of Science:
- Cardiovascular Pathology
- Oncology
- Surgical Pathology
Background:
- Intimal sarcoma is a rare malignant tumor originating in the intima of blood vessels.
- Cardiac involvement by intimal sarcoma is exceptionally uncommon, presenting diagnostic and therapeutic challenges.
Purpose of the Study:
- This case report aims to illustrate the clinical presentation, diagnostic findings, and aggressive behavior of intimal sarcoma involving the heart and great vessels.
- To highlight the obstructive complications and rapid progression associated with this rare malignancy.
Main Methods:
- A case study of a 63-year-old woman with dyspnea.
- Diagnostic workup included imaging (echocardiography, CT, MRI) and histopathological examination of tumor biopsy.
- Immunohistochemistry was performed to confirm the diagnosis.
Main Results:
- Imaging revealed a large right atrial mass extending into the left atrium, pulmonary veins, and vena cavae.
- Histopathology showed spindle-shaped cells with high mitotic activity, positive for SMA, desmin, and MDM2, confirming intimal sarcoma.
- The patient experienced rapid tumor progression, leading to pulmonary vein stenosis, vena cava obstruction, and obstructive shock.
Conclusions:
- Intimal sarcoma of the heart is an aggressive neoplasm characterized by local infiltration and significant obstructive complications.
- Palliative care was initiated due to the tumor's inoperability and rapid progression.
- This case underscores the importance of considering rare cardiac tumors in the differential diagnosis of unexplained obstructive symptoms.
Keywords:
MDM2 amplificationcardiac sarcomacardio-oncology, inferior vena cava (IVC) obstructionintimal sarcomapulmonary vein stenosissuperior vena cava (SVC) obstructionMore Related Videos
Related Concept Videos
01:24Blood Pressure Imbalances and Circulatory Shock
728
Disorders affecting blood volume, vascular tone, or vascular function can disrupt vascular homeostasis, including conditions like hypertension, hemorrhage, and shock.
Blood Pressure: Hypertension and Hypotension
Normal blood pressure is 120/80 mm Hg. Elevated blood pressure is 120-129/under 80 mm Hg. Hypertension, warranting treatment at 130/80 mm Hg, is often asymptomatic and can lead to severe cardiovascular events, aneurysms, peripheral arterial disease, chronic renal disease, or cardiac...
Blood Pressure: Hypertension and Hypotension
Normal blood pressure is 120/80 mm Hg. Elevated blood pressure is 120-129/under 80 mm Hg. Hypertension, warranting treatment at 130/80 mm Hg, is often asymptomatic and can lead to severe cardiovascular events, aneurysms, peripheral arterial disease, chronic renal disease, or cardiac...
728
01:24Esophageal Varices-I: Introduction
56
Esophageal varices are dilated, tortuous veins which are found mainly in the submucosa of the lower esophagus but which may also appear higher up or extend into the stomach. They develop due to increased pressure in the portal venous system, often as a result of liver cirrhosis. This condition scars and damages the liver, impeding normal blood flow through the portal vein. To compensate, blood seeks alternative pathways, forming fragile new vessels (varices) in the esophagus and stomach. These...
56
01:28Esophageal Varices-II: Clinical Features and Management
34
Esophageal varices often manifest as gastrointestinal bleeding episodes, presenting symptoms like hematemesis (vomiting of blood), hematochezia (passing fresh blood via the rectum), and melena (black, tarry stools). Other signs can include weight loss, anorexia, abdominal discomfort, jaundice, pruritus, altered mental status, and muscle cramps.
In the initial assessment, a thorough review of the patient's medical history is vital to identify risk factors such as liver disease, alcohol...
In the initial assessment, a thorough review of the patient's medical history is vital to identify risk factors such as liver disease, alcohol...
34
01:28Esophageal Perforation-II: Clinical Manifestations and Management
31
Esophageal perforations manifest in various clinical forms, influenced by factors such as the perforation's cause and location (cervical, intrathoracic, or intra-abdominal), the extent of contamination, and potential injury to adjacent mediastinal structures. The timing between the perforation occurrence and treatment initiation also affects the clinical presentation.
Clinical Manifestations:
Clinical Manifestations:
31
01:30Pulmonary Hypertension: Classification and Pathogenesis
142
Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
There are various classifications for PH, each relating to different underlying causes and also...
142