Cardiac Masses - Cases
Bandar A. Alyami, MD
Medical resident
west virginia university
Morgantown, West Virginia, United States
Bandar A. Alyami, MD
Medical resident
west virginia university
Morgantown, West Virginia, United States
Ahmed Abdelhaleem, MD
Advanced Cardiac Imaging Fellow
West Virginia University Heart & Vascular Institute
Morgantown, West Virginia, United States
Ion Presneac, MD
Pathology Resident
West Virginia University, United States
Anas Alharbi, MD
Cardiology Fellow
West Virginia University, United States
Yasar Sattar, MD
Cardiology Fellow
West Virginia University, United States
Mohamed Alhajji, MD
Chief Resident
Augusta University/University of Georgia
Lawrenceville, Georgia, United States
Dhivya Velu, MD
Advanced Cardiac Imaging Fellow
West Virginia University, United States
Zakeih chaker, MD
Cardiology Fellow
West Virginia University, United States
Matthew K. Shotwell, MD
Physician
University of Louisville
Prospect, Kentucky, United States
Kesavan Sankaramangalam, MD
Cardiology Fellow
East Carolina University, United States
Sudharshan Balla, MD
Associate Professor of Cardiology
West Virginia University, United States
Yasmin Hamirani, MD
Assistant professor of Cardiology
West Virginia University Heart & Vascular Institute
Morgantown, West Virginia, United States
A 57-year-old man presented with fatigue, exertional dyspnea, and cough one week prior to presentation. His co-morbid conditions included: non-insulin-dependent diabetes mellitus, previous history of smoking, and pneumoconiosis. His physical examination was unremarkable.
Diagnostic Techniques and Their Most Important Findings:
A chest CT angiogram was performed due to suspicion for pulmonary embolism which depicted a LA mass (measuring 6.1 x 2.4 cm) invading the left inferior pulmonary vein (LIPV) and extending to the posterior mediastinum along with sub-carinal lymphadenopathy suspicious of malignancy.
An extensive work-up including bronchoscopy, mediastinoscopy with lymph node biopsy, and CT abdomen and brain revealed no other extra-cardiac masses. On Cardiovascular MRI (CMR) 1.5 T scanner (Siemens Healthineers, Erlangen, Germany) performed for tissue characterization (Figure 1 A-G), the mass was hyperintense on T2w and isointense on T1w images. Increased T1(ShMOLLI) and T2 times were noted on mapped images of 1540 msec and 68 msec, respectively. The mass was predominantly avascular with some vascular components on first-pass perfusion imaging. It was partly hypo-intense on long T1 sequence with heterogenous enhancement on late gadolinium images. A differential diagnosis of hemangioma or angiosarcoma was made.
Subsequently, a whole-body PET/CT FDG scan was performed that revealed uptake only in the mass in the LA and LIPV (Figure 2 A-D). There was no evidence of extra-cardiac metastasis. Mediastinal lymph node biopsies revealed benign granuloma. Eventually, an intracardiac echocardiogram (ICE)-guided trans-atrial septal biopsy of the mass was performed that revealed the diagnosis of poorly differentiated intimal sarcoma, and showed nuclear positivity for both MDM2 and CDK4, consistent with amplification of these genes on the long arm of chromosome 12 (Figure 3 A&B).
A multidisciplinary decision was to treat the patient initially with neoadjuvant treatment with improvement in the size of the mass. This was followed by successful LA subtotal mass resection with en-bloc resection of the LIPV, left lower lung lobectomy, and LA reconstruction with a bovine pericardial patch (Figure 3 C). Unfortunately, after 6 months the patient developed tracheal, brain, and spinal metastases. Referral to hospice was made and the patient died after 2.5 years from the initial presentation.
Learning Points from this Case:
Intimal sarcoma is an extremely rare primary cardiac tumor that typically originates from the major cardiac vessels and is associated with a dismal prognosis.1 Our case is rare with its origin in the LIPV. CMR can play a pivotal role in establishing the diagnosis with confirmation using biopsy.