MP48. Primary Intimal Sarcoma of the Mitral Valve
Presented During: Moderated E-Posters
Rush University Medical Center
Chicago, IL
United States - Contact Me
Hi,
My name is Michael Bishop. I am a first year cardiothoracic surgery fellow at RUSH university medical center in Chicago, IL. I am interested in heart failure and mechanical support.
Thursday, May 4, 2023: 6:30 PM - Saturday, May 6, 2023: 2:29 AM
New York Hilton Midtown
Room: Grand Ballroom Foyer
Description
Objectives
The objective of this paper is to differentiate types of mitral valve masses. Transesophageal echo is the most common modality used to identify intracardiac lesions. TEE is an adequate way of identifying intracardiac tumors but is unable to differentiate a benign myxoma from a malignant intracardiac sarcoma. Greater than 75% of cardiac tumors are benign. Our case, however, presents a mass suggestive of a myxoma in the left atrium, which was found to be a primary intimal sarcoma of the mitral valve. Primary intimal sarcomas are extremely rare, mostly found in the right atrium, infiltrate surrounding tissue, and cause symptoms related to their location.
Methods
A 37 year old woman was transferred to our hospital after 2 weeks of worsening SOB. The patient was intubated in the ED due to hypoxic respiratory failure. A transthoracic echo was performed with findings of severe mitral valve stenosis with a mass, concerning for endocarditis. CT PE protocol showed a large filling defect in the left atrium, pulmonary edema, and bilateral pleural effusions. A TEE showed a mobile mass on the posterior leaflet measuring 3.1 x 3.2 cm. Acoustic contrast showed complete opacification and a small stalk, consistent with a left atrial myxoma.
Results
The patient was taken to the OR and sternotomy was performed with percutaneous bicaval cannulation. An incision was made through Sondergaard's groove with identification of a mitral valve mass that involved the anterior and posterior leaflets with no clear stalk. The mass and mitral valve leaflets were excised and replaced with a 29 mm St. Jude mechanical valve. The final pathology revealed a 4.0 x 2.2 x 1.5 cm tumor composed of many features consistent with primary intimal sarcoma. Her PET scan revealed no residual intracardiac tumor or extracardiac extension.
Conclusions
It is challenging to differentiate a primary intimal sarcoma from a myxoma via TEE. Whether benign or malignant-appearing, tumor removal is essential for tissue sampling and patient management. Usually, less than 50% of tumors can undergo complete surgical resection. Due to the aggressive nature of the tumor, primary intimal sarcomas require prompt surgical resection, postoperative chemotherapy and radiation. These tumors often recur and metastasize. The prognosis is very poor with a 3 month to 1 year survival rate. Our case is an example of what appeared to be a benign myxoma, but was found to be an aggressive primary intimal sarcoma.
The objective of this paper is to differentiate types of mitral valve masses. Transesophageal echo is the most common modality used to identify intracardiac lesions. TEE is an adequate way of identifying intracardiac tumors but is unable to differentiate a benign myxoma from a malignant intracardiac sarcoma. Greater than 75% of cardiac tumors are benign. Our case, however, presents a mass suggestive of a myxoma in the left atrium, which was found to be a primary intimal sarcoma of the mitral valve. Primary intimal sarcomas are extremely rare, mostly found in the right atrium, infiltrate surrounding tissue, and cause symptoms related to their location.
Methods
A 37 year old woman was transferred to our hospital after 2 weeks of worsening SOB. The patient was intubated in the ED due to hypoxic respiratory failure. A transthoracic echo was performed with findings of severe mitral valve stenosis with a mass, concerning for endocarditis. CT PE protocol showed a large filling defect in the left atrium, pulmonary edema, and bilateral pleural effusions. A TEE showed a mobile mass on the posterior leaflet measuring 3.1 x 3.2 cm. Acoustic contrast showed complete opacification and a small stalk, consistent with a left atrial myxoma.
Results
The patient was taken to the OR and sternotomy was performed with percutaneous bicaval cannulation. An incision was made through Sondergaard's groove with identification of a mitral valve mass that involved the anterior and posterior leaflets with no clear stalk. The mass and mitral valve leaflets were excised and replaced with a 29 mm St. Jude mechanical valve. The final pathology revealed a 4.0 x 2.2 x 1.5 cm tumor composed of many features consistent with primary intimal sarcoma. Her PET scan revealed no residual intracardiac tumor or extracardiac extension.
Conclusions
It is challenging to differentiate a primary intimal sarcoma from a myxoma via TEE. Whether benign or malignant-appearing, tumor removal is essential for tissue sampling and patient management. Usually, less than 50% of tumors can undergo complete surgical resection. Due to the aggressive nature of the tumor, primary intimal sarcomas require prompt surgical resection, postoperative chemotherapy and radiation. These tumors often recur and metastasize. The prognosis is very poor with a 3 month to 1 year survival rate. Our case is an example of what appeared to be a benign myxoma, but was found to be an aggressive primary intimal sarcoma.