A Rare Case of Pericardial Inflammatory Myofibroblastic Tumor Presenting With Dyspnea
Recommended Citation
Munir M, Arif M, Rashi F, Yerramsetty DL. A Rare Case of Pericardial Inflammatory Myofibroblastic Tumor Presenting With Dyspnea. Am J Respir Crit Care Med 2025; 211(Supplement 1).
Document Type
Conference Proceeding
Publication Date
5-1-2025
Publication Title
Am J Respir Crit Care Med
Keywords
C reactive protein, corticosteroid, cyclooxygenase 2, nonsteroid antiinflammatory agent, vasculotropin, adult, body weight loss, cardiomegaly, case report, cell proliferation, child, clinical article, computer assisted tomography, conference abstract, decreased appetite, diagnosis, drug therapy, dyspnea, echocardiography, electrocardiography, fatigue, female, fever, fluorescence in situ hybridization, gene rearrangement, heart palpitation, heart sound, human, human tissue, jugular vein, loss of appetite, medical history, moderate persistent asthma, neck swelling, night sweat, oral drug administration, pediatric patient, pericardial biopsy, pericardial effusion, pericardiocentesis, pericardiotomy, pericarditis, pericardium, physical examination, plasma cell granuloma, pleura effusion, rheumatoid arthritis, school child, sinus tachycardia, spindle cell, surgery, tachycardia, young adult
Abstract
Inflammatory Myofibroblastic Tumor (IMT) is a rare and unpredictable tumor composed of spindle cells and an inflammatory component. It is most commonly found in children and young adults, typically affecting the lungs or abdomen. The patient is a 68-year-old female with a medical history significant for seronegative rheumatoid arthritis (RA) and moderate persistent asthma. She presented with generalized fatigue, palpitations, and shortness of breath, which had been ongoing for one month.The patient reported multiple visits to her primary care provider over the past month due to fatigue, night sweats, fevers up to 102°F, loss of appetite, and a 5-10 pound weight loss. Upon arrival at the hospital, she was tachycardic, and physical examination revealed distended neck veins and diminished heart sounds. Her ECG showed sinus tachycardia at a rate of 102 bpm. A CT scan of the chest with IV contrast revealed a large pericardial effusion, cardiomegaly, small left pleural effusion, trace right pleural effusion, and small right pericardial lymph nodes. An echocardiogram confirmed a small to moderate-sized pericardial effusion with no evidence of tamponade. Laboratory findings were notable for an elevated ESR (58) and CRP (8.8).The patient underwent a subxiphoid pericardial window procedure with drain placement, performed by cardiothoracic surgery (CTS), which resulted in symptom resolution and improvement in tachycardia. A pericardial biopsy was sent for analysis and revealed low-grade spindle cell proliferation, suggestive of an inflammatory pseudotumor/myofibroblastic tumor. Testing for ALK gene rearrangement by FISH was negative, and the pericardial fluid was negative for malignant cells.This case underscores the importance of considering cardiac IMT-associated pericardial effusion as a potential cause of dyspnea. Fewer than 60 cases of cardiac IMTs have been documented in the literature, most of which are endocardial in origin. A literature review identified only two case reports involving inflammatory myofibroblastic tumor-associated pericardial effusion, one with an endocardial origin and the other with a pericardial origin, both in pediatric patients.Surgical resection of the tumor is considered the definitive treatment, as IMT can lead to recurrent pericardial effusions. In the meantime, symptomatic pericardial effusions can initially be managed with pericardiocentesis or a pericardial window. Some studies suggest that NSAIDs and corticosteroids may be effective in treating IMT, given the role of COX-2 and VEGF expression in tumor proliferation. However, further research is needed before these therapies can become standard practice.
Volume
211
Issue
Supplement 1
