Superior Vena Cava Syndrome Secondary to a Fibrin-Associated Diffuse Large B Cell Lymphoma Arising From a Superior Vena Cava-Right Atrium Thrombus
Presented During:
Saturday, May 6, 2023: 5:00PM - Tuesday, May 9, 2023: 5:00PM
Los Angeles Convention Center
Posted Room Name:
ePoster Area, Exhibit Hall
Abstract No:
6280
Submission Type:
Cardiothoracic Resident Case Report Competition
Authors:
Andres Diaz (1), Paolo Bosco (1)
Institutions:
(1) St Thomas' Hospital, London, United Kingdom
Submitting Author:
Andres Diaz
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St Thomas' Hospital
St Thomas' Hospital
Co-Author:
Paolo Bosco
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St Thomas' Hospital
St Thomas' Hospital
Presenting Author:
Andres Diaz
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Abstract:
Abstract
Primary cardiac lymphomas are rare and subvariants like fibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) are even more uncommon. They are mostly found within cardiac myxomas or associated with cardiac prosthesis. They do no tend to form a mass making the diagnosis challenging. Symptoms are unspecific and their behaviour is indolent. Surgical resection alone seems to offer promising results without the need of adjuvant therapy.
We present a 24-year-old female with FA-DLBCL located within an SVC thrombus causing SVC syndrome.
TTE revealed the presence of a large mass filling the entire right atrium, extending from the SVC. Subsequently she underwent a successful removal of the SVC and RA mass.
To our knowledge this is the first case of a FA-DLBCL located in the SVC causing SVC syndrome.
Patient and methods
A 24 year-old immunocompetent female who presented with SVC obstruction symptoms was referred to our cardiac surgery service for debulking of an SVC and RA mass.
She presented initially with dyspnoea, headaches and chest tightness. A CT revealed bilateral pulmonary embolism and she received anticoagulation therapy. However, over the following months she developed facial, neck, upper limb and upper chest swelling, mostly when bending forward. She underwent a biopsy of the mass that revealed a FA-DBLCL. Chemotherapy was initially offered but her symptoms continued to deteriorate rapidly with increasing facial swelling and cyanosis. A transthoracic echocardiography showed a 4.7x 5.5cm mass filling the entire right atrium, extending from the SVC (completely blocked) and causing severe TV obstruction. The patient was immediately admitted from the clinic. Following a multidisciplinary team meeting decision was made to perform urgent surgery to remove the mass. The preoperative planning considered the need for deep hypothermic circulatory arrest due to the localization of the tumour obstructing the RA and SVC.
Median sternotomy was performed. The right atrium was distended and hard on palpation. Ascending aorta cannulation was performed. A two-stage venous cannula was carefully inserted in the the right atrium after initial digital palpation of the mass to ascertain if there was space available for the cannula. Full cardiopulmonary bypass was commenced and the patient was cooled to 18 degrees Celsius. The venous cannula was removed and the right atrium was opened revealing an atrial mass measuring 7x4 cm occupying almost the entire chamber. The SVC was hard on palpation and completely obliterated by the tumour. The right atrial mass was resected en-bloc. The SVC was partially freed from the tumour obtaining a mass measuring 6x4 cm. We removed as much of the mass as possible to guarantee that the SVC lumen was not obstructed. This part of the procedure was the most challenging as we were aiming to remove the mass in an expeditious manner without causing injury and considering the DHCA time to keep it to a minimum. The procedure was uneventful, the patient recovered well and was discharged home after 1 cycle of chemotherapy. During follow up there has been no signs of recurrence.
We present a unique case of a young female immunocompetent adult with a FA-DLBCL arising from an SVC thrombus presenting with SVC syndrome. She underwent a successful resection of the mass under a brief period of deep hypothermic circulatory arrest and made an uneventful recovery. Clinical outcome seems favourable after surgical resection
Primary cardiac lymphomas are rare and subvariants like fibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) are even more uncommon. They are mostly found within cardiac myxomas or associated with cardiac prosthesis. They do no tend to form a mass making the diagnosis challenging. Symptoms are unspecific and their behaviour is indolent. Surgical resection alone seems to offer promising results without the need of adjuvant therapy.
We present a 24-year-old female with FA-DLBCL located within an SVC thrombus causing SVC syndrome.
TTE revealed the presence of a large mass filling the entire right atrium, extending from the SVC. Subsequently she underwent a successful removal of the SVC and RA mass.
To our knowledge this is the first case of a FA-DLBCL located in the SVC causing SVC syndrome.
Patient and methods
A 24 year-old immunocompetent female who presented with SVC obstruction symptoms was referred to our cardiac surgery service for debulking of an SVC and RA mass.
She presented initially with dyspnoea, headaches and chest tightness. A CT revealed bilateral pulmonary embolism and she received anticoagulation therapy. However, over the following months she developed facial, neck, upper limb and upper chest swelling, mostly when bending forward. She underwent a biopsy of the mass that revealed a FA-DBLCL. Chemotherapy was initially offered but her symptoms continued to deteriorate rapidly with increasing facial swelling and cyanosis. A transthoracic echocardiography showed a 4.7x 5.5cm mass filling the entire right atrium, extending from the SVC (completely blocked) and causing severe TV obstruction. The patient was immediately admitted from the clinic. Following a multidisciplinary team meeting decision was made to perform urgent surgery to remove the mass. The preoperative planning considered the need for deep hypothermic circulatory arrest due to the localization of the tumour obstructing the RA and SVC.
Median sternotomy was performed. The right atrium was distended and hard on palpation. Ascending aorta cannulation was performed. A two-stage venous cannula was carefully inserted in the the right atrium after initial digital palpation of the mass to ascertain if there was space available for the cannula. Full cardiopulmonary bypass was commenced and the patient was cooled to 18 degrees Celsius. The venous cannula was removed and the right atrium was opened revealing an atrial mass measuring 7x4 cm occupying almost the entire chamber. The SVC was hard on palpation and completely obliterated by the tumour. The right atrial mass was resected en-bloc. The SVC was partially freed from the tumour obtaining a mass measuring 6x4 cm. We removed as much of the mass as possible to guarantee that the SVC lumen was not obstructed. This part of the procedure was the most challenging as we were aiming to remove the mass in an expeditious manner without causing injury and considering the DHCA time to keep it to a minimum. The procedure was uneventful, the patient recovered well and was discharged home after 1 cycle of chemotherapy. During follow up there has been no signs of recurrence.
We present a unique case of a young female immunocompetent adult with a FA-DLBCL arising from an SVC thrombus presenting with SVC syndrome. She underwent a successful resection of the mass under a brief period of deep hypothermic circulatory arrest and made an uneventful recovery. Clinical outcome seems favourable after surgical resection
Category:
Adult Cardiac
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