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ARTICLE IN PRESS
doi:
10.25259/IJNM_169_24

Atypical Relapse of Multiple Myeloma as an Atrial Mass

Department of Radiology, University Hospital Our Lady of Candelaria, Santa Cruz de Tenerife, Spain
Department of Cardiology, University Hospital Our Lady of Candelaria, Santa Cruz de Tenerife, Spain
Department of Hematology, University Hospital Our Lady of Candelaria, Santa Cruz de Tenerife, Spain
Department of Pathology, University Hospital Our Lady of Candelaria, Santa Cruz de Tenerife, Spain
Department of Nuclear Medicine, University Hospital Our Lady of Candelaria, Santa Cruz de Tenerife, Spain

*Corresponding author: Dr. Jon-Andoni Uña-Gorospe, Department of Nuclear Medicine, Hospital Universitario Nuestra Señora de Candelaria, Carretera del Rosario 145, Santa Cruz de Tenerife 38010, Spain. junagor@gobiernodecanarias.org

Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: González-González ML, López F, Moreno-Peña T, Vannini L, Afonso JM, Uña-Gorospe JA. Atypical Relapse of Multiple Myeloma as an Atrial Mass. Indian J Nucl Med. doi: 10.25259/ IJNM_169_24.

Abstract

We present the case of a patient diagnosed with multiple myeloma in 2021 and currently undergoing second-line treatment. On admission secondary to acute cholecystitis, she presented chest pain and cardiac tamponade. Echocardiography showed pericardial effusion and deformation of the right atrial wall with infiltrating mass. A computed tomography (CT) scan was performed that reported a voluminous filling defect in the atrium. Positron emission tomography (PET) showed high fluorodeoxyglucose (FDG) metabolism in the auricular mass, as well as multiple bone lesions. Extramedullary intracardiac expression of myeloma is extremely rare, with a worse prognosis. To our knowledge, this is the fifteenth case described in the literature that initially relapsed as atrial plasmacytoma.

Keywords

Fluorodeoxyglucose (FDG)
Heart
Myeloma
Positron emission tomography (PET)
Positron emission tomography-computed tomography
(PET-CT)
Plasmocytoma

We present the case of a patient diagnosed with monoclonal gammopathy of undetermined significance (MGUS) who was lost to follow-up and developed symptoms seven years later. The Hematology Department confirmed IgA kappa multiple myeloma ISS-R 1 and initiated first-line treatment. Following disease progression, second-line treatment was started.

Upon admission for acute cholecystitis, the patient presented with chest pain and cardiac tamponade. Echocardiography revealed an infiltrative mass. Contrast-enhanced computed tomography (CECT) and magnetic resonance imaging (MRI) were performed, revealing an aggressive mass. Positron emission tomography (PET) showed high 18F-fluorodeoxyglucose metabolism in the atrial mass, as well as multiple bone lesions [Fig 1 and 2].

PET showed high 18F-FDG metabolism in the atrial mass with heterogeneous uptake, whose standard uptake value (SUV) reached a value of 7.35. Contrast-enhanced computed Tomography showed a voluminous filling defect in the right atrium (RA).
Fig 1:
PET showed high 18F-FDG metabolism in the atrial mass with heterogeneous uptake, whose standard uptake value (SUV) reached a value of 7.35. Contrast-enhanced computed Tomography showed a voluminous filling defect in the right atrium (RA).
Whole body positron emission tomography (PET)–computed tomography maximum intensity projection (MIP) image demonstrating multiple bone lesions with high 18F-fluorodeoxyglucose (FDG) uptake. The sum of diagnostic tests allowed us to delimit the local extent of the lesion, as well as the body extension of the disease using 18F-FDG PET.
Fig 2:
Whole body positron emission tomography (PET)–computed tomography maximum intensity projection (MIP) image demonstrating multiple bone lesions with high 18F-fluorodeoxyglucose (FDG) uptake. The sum of diagnostic tests allowed us to delimit the local extent of the lesion, as well as the body extension of the disease using 18F-FDG PET.

A biopsy was performed via the right jugular vein without complications, with a histopathological diagnosis of plasma cell neoplasm [Fig 3]. Multiple myeloma is a rare entity, most frequently located in the bone marrow. Extramedullary intracardiac manifestation is extremely rare, with a worse prognosis.[1] Several imaging tests are considered useful, including ultrasound, positron emission tomography, and histopathological examination.[2] To our knowledge, this is the fifteenth case described in the literature that initially recurred as atrial plasmacytoma.[3-14] The combination of diagnostic tests allowed us to delineate the local extent of the lesion, as well as the systemic extent of the disease using 18F-fluorodeoxyglucose positron emission tomography.secretion, and negativity to cyclin-D1 [Fig 3e and 3f]

A biopsy was performed via the right jugular vein using a biotome for myocardial biopsy guided by transesophageal echocardiography without complications, with a pathological diagnosis of myeloma-type plasma cell neoplasia, with monotypic secretion of kappa light chains. (a,b) Diffuse proliferation of plasma cells with intermixed blood vessels and characterized by wide eosinophilic cytoplasm and eccentric nuclei with dark chromatin, sometimes with conspicuous nucleoli and some mitotic figures. Immunohistochemistry tests revealed (c) CD138 and (d) CD56 positivity, (e,f) monotypic kappa light chain secretion and negativity to cyclin-D1. For (a) (H&E) stain, magnification 10×; (b) (H&E) stain, magnification 20×; IHC image magnification 20×. H&E: Hematoxylin and Eosin; IHC: Immunohistochemistry
Fig 3:
A biopsy was performed via the right jugular vein using a biotome for myocardial biopsy guided by transesophageal echocardiography without complications, with a pathological diagnosis of myeloma-type plasma cell neoplasia, with monotypic secretion of kappa light chains. (a,b) Diffuse proliferation of plasma cells with intermixed blood vessels and characterized by wide eosinophilic cytoplasm and eccentric nuclei with dark chromatin, sometimes with conspicuous nucleoli and some mitotic figures. Immunohistochemistry tests revealed (c) CD138 and (d) CD56 positivity, (e,f) monotypic kappa light chain secretion and negativity to cyclin-D1. For (a) (H&E) stain, magnification 10×; (b) (H&E) stain, magnification 20×; IHC image magnification 20×. H&E: Hematoxylin and Eosin; IHC: Immunohistochemistry

Author contribution:

MLG: and JAU: Performed the PET-CT and the selection of PET-CT images; FL LV, and TM: Attended the clinic and performed cardiac ultrasound and CT; JM: Analyzed the pathological anatomy and selected significant staining images; JAU: Coordinated the preparation of the manuscript. All authors participated in the writing of the manuscript.

Ethical approval:

Institutional Review Board approval is not required.

Declaration of patient consent:

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given consent for their images and other clinical information to be reported in the journal. The patient understand that the patient’s names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Conflicts of interest:

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation:

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Financial support and sponsorship: Nil.

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