International Journal of Surgery Case Reports 114 (2024) 109187

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International Journal of Surgery Case Reports

journal homepage: www.elsevier.com/locate/ijscr

Case report

Uncommon manifestation of multiple myeloma: A case report of

intracardiac plasmacytoma presenting with severe heart failure and
bilateral pleural effusion
Seyed Mohammadali Mousavizadeh Ahmadabadi a, b, Sepideh Banar a, b, Ehsan Parvas c,
Narges Shahbazi c, Hamidreza Biranvand c, *
a
Heart Valve Disease Research Center, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran
b
Shahid Rajaei Research & Training Hospital, Next to Mellat Park, Vali-Asr Ave, Tehran Postal code: 1995614331, Iran
c
Tehran University of Medical Sciences, Tehran Heart Center, North Kargar Ave, Tehran Postal code: 1431715673, Iran

A R T I C L E I N F O A B S T R A C T

Keywords: Introduction and importance: Extramedullary intracardiac plasmacytoma is a rare type of plasma cell neoplasm
Multiple myeloma that involves the heart. This article presents a case report of a patient with this condition and describes the
Plasmacytoma surgical management of the disease.
Case report
Case presentation: The patient was a 65-year-old male with a history of multiple myeloma in remission for two
Uncommon manifestation
years who presented with shortness of breath and chest pain. Imaging studies revealed a mass in the right atrium
of the heart, which was confirmed to be extramedullary intracardiac plasmacytoma on biopsy. The patient
underwent surgical resection of the mass.
Clinical discussion: Extramedullary intracardiac plasmacytoma is a rare condition that can occur in patients with
multiple myeloma. Early diagnosis and prompt treatment are crucial for improving the prognosis of affected
individuals. Surgical resection may be a viable treatment option for some patients.
Conclusion: This case report highlights the potential risks and complications associated with surgical intervention
in patients with extramedullary intracardiac plasmacytoma. Further research is needed to determine the best
treatment approach for this rare condition and to improve patient outcomes.

1. Introduction and importance
Extramedullary plasmacytoma (EMP) is a type of plasma cell
neoplasm (PCN) which consists of two main groups: plasmacytoma and
multiple myeloma (MM) [1]. While plasmacytoma is usually a solitary
tumor of the bone, EMP can occur in various extramedullary sites, such
as the head and neck region [2,3]. Despite being rare, intracardiac EMPs
can present with severe symptoms, including pericardial tamponade,
arrhythmia, superior vena cava syndrome, and heart failure, and have a
poor prognosis, with over 50 % of patients dying within 2 days to 15
months after diagnosis [4–6]. Diagnosing intracardiac EMP requires
pathological investigation, which can be challenging due to the condi­
tion's rarity and difficulty distinguishing plasmacytoma from other,
more common masses [2]. Treatment options for intracardiac EMP are
not well-established due to the rarity of the disease [3,5,7]. A rare case
of this tumor was presented to the Tehran Heart Center in Tehran, Iran.
Surgical intervention was necessary due to the tumor's widespread
extension. The present study has been reported in line with the SCARE
criteria [8].
2. Case presentation
A 67-year-old male patient, known case of multiple myeloma five
years earlier, taking weekly thalidomide and dexamethasone, was
admitted due to fatigue with the diagnosis of multiple myeloma relapse.
After ten days, the patient's condition worsened, presenting respiratory
distress and low blood pressure. Echocardiography revealed a large left
atrium mass, and then the patient was referred to a cardiac center. On
arrival, the patient was unresponsive with central cyanosis, Spo2 was 75
%, and blood pressure was 80/60 mm Hg with the pulse rate of 145 and
respiratory rate of 60. Subsequently, the patient was intubated and
admitted to ICU. On investigation, pulmonary CT angiography showed

* Corresponding author.
E-mail address: hr.biranvand@gmail.com (H. Biranvand).

https://doi.org/10.1016/j.ijscr.2023.109187
Received 1 November 2023; Received in revised form 15 December 2023; Accepted 18 December 2023
Available online 29 December 2023
2210-2612/© 2023 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
S.M. Mousavizadeh Ahmadabadi et al. International Journal of Surgery Case Reports 114 (2024) 109187

severe bilateral pleural effusion with compressive collapse of lower

lobes and a lobulated 79 mm mass attached to the left atrium that
protruded in the mitral valve and extended to the right atrium by fo­
ramen oval (Fig. 1). Echocardiography demonstrated a large immobile
mass in the left atrium (40 * 35 mm) with protrusion to the right atrium
and extension to SVC. Based on his condition, the patient was a candi­
date for surgical intervention. The surgery was performed on the 6th day
of admission, during which the sternum and pericardium were opened at
first.
Then Bicaval cannulation and antegrade cardio infusion were carried
out. The tumor found to invade RA, LA, and pericardium from outside.
The interatrial septum, RA wall, and a part of LA wall which were
partially removed, were repaired using the bovine pericardial patch, and
the resected tumor was sent for pathologic investigation (Fig. 2). The
microscopic description of the specimen was reported as a neoplasm
consisting of round pleomorphic cells in sheets and alveolar pattern. The
cells contained round hyperchromatic nuclei with occasional conspic­
uous nucleoli. A large population of neoplastic cells had eccentric nuclei
and conspicuous eosinophilic cytoplasms. Huge areas of necrosis were
present, and mitotic figures were frequent; some showed atypical fea­
Fig. 2. Gross view of the resected tumor.
tures (Fig. 3). For further confirmation of the nature of the neoplasm,
immunohistochemical staining was performed which revealed diffuse
membranous positivity with CD138 antibody (Fig. 4) and nuclear pos­
itivity with MUM1 antibody (Fig. 5).
Three hours after surgery, due to bleeding from the surgical wound,
the patient was returned to OR for hemostasis and bleeding control. The
patient's postoperative vital signs were unstable, and on the 8th day of
admission, he expired due to cardiac arrest. The cause of death was RV
dysfunction due to pulmonary artery embolism caused by the tumor
based on follow-up echocardiography which demonstrated severe TR
and elevated PAP.

3. Clinical discussion

Malignant tumors involving the heart are rare pathologies. Most of

them invade the myocardium directly or by venous, arterial, or
lymphatic metastases. In contrast, primary cardiac tumors are mostly
benign cardiac myxomas, and primary malignant tumors are rare, with a
reported incidence of less than 0.1 % among 12,000 autopsies, while
metastatic malignant tumors of the heart are 20 times more common

Fig. 3. A neoplasm consisting of round pleomorphic cells in sheets and alveolar

pattern, the cells contain round hyperchromatic nuclei with occasional con­
spicuous nucleoli. A large population of neoplastic cells have eccentric nuclei
and conspicuous eosinophilic cytoplasms. Huge areas of necrosis are present,
and mitotic figures are frequent; some show atypical features.

Fig. 1. Radiologic view of the tumor in CT Angiography.
[9].
Plasma cell neoplasms (PCN) are immunoprolifrative monoclonal
disorders of B-Cells caused by the malignant transformation of plasma
cells. These tumors consist of two main groups: plasmacytoma and
multiple myeloma (MM). Plasmacytoma can be either a solitary tumor of
the bone or an extramedullary plasmacytoma (EMP), which can be
primary or secondary to another PCN like MM [1].
More than 80 % of EMPs are present in the head and neck region,
mostly in the nasal cavity, paranasal sinuses, and oronasopharynx [1].
Seldom EPM can occur in the thyroid, hypopharynx, larynx, and other
areas like the gastrointestinal tract. Cardiac involvement in EPMs is
extremely rare, as only about 50 cases have been reported between 1977
and 2020 [2,3].
In terms of clinical symptoms, extramedullary intracardiac plasma­
cytoma can present with various signs and symptoms depending on the

2
S.M. Mousavizadeh Ahmadabadi et al.
Fig. 4. Immunohistochemical staining, diffuse membranous positivity with
CD138 antibody.
Fig. 5. Immunohistochemical staining, nuclear positivity
MUM1 antibody.
size and localization of the mass, such as pericardial tamponade,
arrhythmia, SVC syndrome, or severe heart failure [4,6]. In this case,
due to involvement of the left and right atrium as well as the extension to
the SVS, the patient presented with bilateral pleural effusion and severe
heart failure leading to an unstable hemodynamic state.
The definite diagnosis of plasmacytoma requires pathological
investigation using specimens from transvenous biopsy or surgical
resection [2]. In our case, based on microscopic and macroscopic
characteristics of the mass, we could not distinguish plasmacytoma from
rhabdomyosarcoma; thus, an Immunohistochemical study was done to
International Journal of Surgery Case Reports 114 (2024) 109187
establish the diagnosis.
Among radiologic examinations, echocardiography and CT scan are
the most common and convenient imaging modalities used to determine
the size and shape of the intracardiac mass. However, they are not
capable of differentiating plasmacytoma from other more common
masses, like thrombus, myxoma, or lymphoma. Some studies suggest
that contrast echocardiography may facilitate the classification of
intracardiac tumors and thrombi [10]. Cardiac magnetic resonance
imaging (CMRI) provides excellent soft-tissue characterization and helps
us to localize and evaluate cardiac masses and their physiologic effects
[11]. Furthermore, positron emission tomography (PET)/CT would also
demonstrate concomitant bone involvement [12]. In our case, because
of the patient's unstable condition, MRI was not carried on. We con­
ducted spiral pulmonary CT angiography, which revealed a lobulated
79 mm mass attached to the interatrial septum with protrusion in the
mitral valve and extension to the right atrium through the foramen oval.
In general, EMP has a better prognosis compared to plasmacytoma
with bone involvement [13], but the situation is different for intracar­
diac EMPs in MM as they show a poor prognosis. Over 50 % of the pa­
tients die within 2 days to 15 months after diagnosis [2,5,14].
Considering the extreme rarity of intracardiac EMP, there is no
consensus on the first choice of treatment. Some options have been re­
ported, such as surgical resection, radiotherapy, and chemotherapy,
alone or in combination, most of which achieved mass debulking and
cardiovascular symptom control but not long-term survival [3,5,7]. In
our case, because of the extension of the tumor to both atrial cavities, the
mitral valve, and SVC, the patient presented with severe heart failure
and an unstable hemodynamic state, so surgical intervention was inev­
itable. One of the unique features in our case was the infiltration of the
tumor to the interatrial septum, which was partially resected during the
surgery and repaired using the bovine pericardial patch.
4. Conclusion
As we experienced in this case, although the only choice for
improving cardiovascular symptoms was surgical resection due to the
emergent condition of the patient, the risk of this procedure was
extremely high, especially considering the tumor's widespread exten­
sion. It is possible that greater awareness may be needed regarding
intracardiac EMP in patients with MM. More studies are mandatory to
determine the success rate of treatment in multiple myeloma patients
with early diagnosis of intracardiac EMP using some routine cardio­
vascular examination like echocardiography.
Consent
Written informed consent was obtained from the patient's repre­
sentative for publication and any accompanying images. A copy of the
written consent is available for review by the Editor-in-Chief of this
journal on request.
Ethical approval
with
Our case report is exempt from ethical approval in our institution.
The reason is, it is case report only.
Funding
This research did not receive any specific grant from funding
agencies in the public, commercial, or not-for-profit sectors.
Guarantor
Hamidreza Biranvand.
3
S.M. Mousavizadeh Ahmadabadi et al.
CRediT authorship contribution statement
HB, EP, and NSh were involved in the study concept or design. SM,
EP, and SB were involved in data collection. HB, EP, and NSh were
involved in data interpretation. SM and SB were involved in writing the
paper under the supervision of HB, EP, and NSh.
Declaration of Generative AI and AI-assisted technologies in the
writing process
All authors disclose any use of generative AI and AI-assisted
technologies.
Declaration of competing interest
None.
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