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R. Vintha and P. N. Sethi
unknown; however, it could have been caused by myocardial injury to the RV apex
wall caused by the metastatic tumor [7]. Similarly, metastasis, tumor invasion into
the heart, tumor emboli within a coronary artery, or a metastatic lesion around a
coronary artery can all cause a neoplastic process.
This patient did not have the typical enzyme and electrocardiography changes
associated with acute myocardial infarction. A metastatic tumor had inltrated the
pericardium and myocardium, causing the ST segment elevation. Continuous myocardial injury, which prevents the formation of new cardiac cell membranes,
stretched adjacent muscle bers, inammatory reaction, ionic potassium transfer
from necrotic tissue to the adjacent myocardium, and potassium transfer from damaged tissue to the adjacent myocardium, all of which produce electric potential differences, are suggested mechanisms for these pseudo-infarction electrocardiography
patterns [8]. An electrocardiography anomaly comparable to ST segment elevation
in myocardial infarction was reported by Na etal. in a case of mimicking ST segment elevation in myocardial infarction [9]. In another example, it was reported that
the electrocardiogram revealed a specic anomaly (ST segment).
With metastatic heart disease, there is an increase and T inversion in leads V1–
V6. Detailed history, physical examination, basic laboratory testing, or imaging
studies can all help diagnose urothelial carcinoma [10]. In such circumstances,
acute myocardial infarction should be ruled out [11]. As a result, invasive procedures can be avoided.
Conclusion
In conclusion, this is a case of metastatic cancer of the heart that was identied
primarily as an acute myocardial infarction. However, this diagnosis was refuted by
the clinical presentation, serial cardiac enzyme values, and persistent ST elevation
observations. When electrocardiography alterations without classic angina are seen
in a patient with malignancy and negative cardiac enzymes in the blood, metastatic
myocardial inltration due to the tumor should be suspected.
Especially in patients with unusual presentations, such as loss of consciousness
and no chest pain, echocardiography is essential for conrming the diagnosis of
myocardial infarction or ischemia when patients show electrocardiography alterations that are suggestive of these conditions. Patients with chronic ST elevation and
unexplained hypercalcemia, particularly those with known or suspected malignancies, should have metastatic malignancy considered in the differential diagnosis.
Furthermore, coronary catheterization was recommended to distinguish between
true coronary occlusion and fake infarction electrocardiogram patterns in patients
with high cardiac enzyme levels, notwithstanding the rarity of myocardial infarction
caused by cancer.
12 Metastatic Heart Cancer Misdiagnosed asAcute Myocardial Infarction
83
References
1. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6964163/
2. Singh H, Giardina TD, Meyer AN, Forjuoh SN, Reis MD, Thomas EJ.Types and origins of
diagnostic errors in primary care settings. JAMA Intern Med. 2013;173(6):418.
3. Black AD.Non-infectious mimics of community-acquired pneumonia. Pneumonia (Nathan).
2016;8:2. Published 2016 Apr 12. https://doi.org/10.1186/s41479- 016- 0002- 1.
4. Pertseva TO, Kіreєva TV, Bєlosludtseva KO. Maski tyazhkikh pnevmonіy: algoritmi
dіagnostiki ta lіkuvannya. Herda, Dnіpropetrovs’k. 2014:64.
5. Pertseva TO, Kyreeva TV, Bielosludtceva KO.Retrospective analysis of lethal severe community acquired pneumonia cases: masks of severe pneumonia. Ukr. Pulmonol. J. 2013;2:26–30.
6. Kirieieva TV, Basina BO, Bielosludtseva KO.Pulmonary embolism in the therapeutic practice,
risk assessment on the example of clinical case. 2018;7(10):599–602.
7. Pertseva TA, Kyreyeva TV, Kravchenko NK.Difcult diagnosis. Clinical analysis of pneumonia overdiagnosis case Medicni perspektivi Medical perspectives. 2013;18(3):25–9.
8. https://read.qxmd.com/read/28515823/lupus- negative- libman- sacks- endocarditis-
complicated- by- catastrophic- antiphospholipid- syndrome
9. https://read.qxmd.com/read/17522239/nonbacterial- thrombotic- endocarditis- in- cancer-
patients- pathogenesis- diagnosis- and- treatment
10. https://read.qxmd.com/read/17881239/non- bacterial- thrombotic- endocarditis
11. https://read.qxmd.com/read/15115997/prosthetic- valve- thrombosis- twenty- year- experience-
at- the- montreal- heart- institute
Chapter 13
Primary Cardiac Synovial Sarcoma
Misdiagnosed asTuberculoma
ShitijShrivastava
Learning Objectives
By the end of this presentation, the clinician will be able to:
1. Discuss the reasons which led to a false diagnosis of a tuberculoma.
2. Describe and explain the histopathology of primary cardiac synovial sarcoma.
3. Learn about the epidemiology of cardiac tumors.
4. Enumerate the diagnostic methods used to diagnose cardiac sarcomas.
5. Distinguish the clinical signs in patients with primary cardiac synovial sarcomas.
Introduction
Primary cardiac synovial sarcoma comprises 1in every 20 cardiac sarcomas and
less than 0.1% of all primary cardiac tumors [1]. Synovial sarcomas (or malignant
synovioma) are seen in all age groups in males and females but usually present in
young males. The distal extremities are most commonly involved. It is a rare disease
and not understood very well. The predominant literature on cardiac tumors comprises case reports rather than their microscopic prole [2]. Case reports on cardiac
synovial sarcomas (including those of secondary origin) date back to 1958 on
PubMed [3].
In 1988, Shefeld etal. described a case of a primary cardiac synovial sarcoma
arising from a benign mesothelioma of the atrioventricular (AV) node [4]. Until
April 1997, only six cases of primary synovial sarcomas of the heart had been
described [5]. They most commonly arise from the right side of the heart, and most
patients have general, unclear symptoms which coincide with other cardiac
S. Shrivastava (*)
BronxCare Health System, Bronx, NY, USA
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
H. Tohid et al. (eds.), The Misdiagnosis Casebook in Clinical Medicine,
https://doi.org/10.1007/978-3-031-28296-6_13
85
86
S. Shrivastava
pathologies. This makes diagnosing primary cardiac synovial sarcomas challenging. They can be either biphasic or monophasic. The mean age of presentation is
32.5 years, and age ranges from 13 to 66 years [6, 7].
Clinical Case Presentation
A 31-year-old, female, Caucasian, presented to the emergency department complaining of fatigue and increasingly severe dyspnea for 2 weeks. Her past medical
history was insignicant. The patient reported anorexia, 8kg weight loss, and physical weakness during the last 3 months. The patient started having New York Heart
Association Class IV dyspnea and general fatigue in the previous 10 days. Her
brother, whom she lived with, had been receiving medical treatment for pulmonary
tuberculosis (TB), although the patient was not taking prophylactic drugs. The
patient required admission for her symptoms. Vital signs were as follows: heart rate,
130 bpm; blood pressure, 100/64 mmHg; and oxygen saturation, 94%. On the physical exam, mufed heart sounds were heard. No murmurs, bruits, rubs, or gallops
were audible. Jugular venous pressure was normal and peripheral edema was absent.
Chest X-ray, echocardiogram, and electrocardiogram (EKG) were ordered on Day
1. Chest X-ray revealed a cardiothoracic ratio of 0.7, along with striking cardiomegaly. The EKG was signicant for diffuse T-wave inversion with normal sinus
rhythm. A mass with the dimensions of 27mm × 35mm was observed in the right
atrium. Echocardiogram also showed large-sized pericardial effusion. The left ventricular ejection fraction (LVEF) was 65%, and the inferior vena cava (IVC) measured 18 mm. The IVC vessel was compliant. Antinuclear antibody (ANA) and
rheumatoid factor (RF) were negative. Additional routine blood tests were within
normal limits except for serum C-reactive protein, which was elevated to 24.5
mg/L.Given the shortness of breath in light of pericardial effusion, pericardiocentesis was performed. One liter of bloody, exudative uid was removed. The pericardial uid was lymphocytic predominant and protein-rich (66 g/L). Adenosine
deaminase (ADA) level was 17 UI/L. However, neoplastic cells were lacking in
cytologic analysis [8]. On the third day of admission, cardiac magnetic resonance
imaging (CMR) demonstrated an immobile intracavitary solid mass of the right
atrium, measuring 39 mm × 28.5 mm (Fig. 13.1). The mass displayed lobulated
contours and was xed to the wall opposite the emergence of the IVC.CMR was
negative for vascular, local, or systemic inltration. The authors presumptively
diagnosed the mass as tuberculoma, owing to the patient’s history of living with her
brother and not receiving TB preventive treatment (TBT). The next day she was
scheduled for a surgical biopsy. Numerous metastatic lesions and vesicles were discovered on the heart, large vessels, and pericardium. Further surgery was deferred
after frozen examination established their neoplastic origin. Pathological examination of biopsy specimens of neoplastic masses was notable for monophasic cardiac
synovial sarcoma. A translocation investigation was not done; she died 3 days later
from cardiac tamponade [8].
ab
13 Primary Cardiac Synovial Sarcoma Misdiagnosed asTuberculoma
Fig. 13.1 CMR in axial T2 FIESTA (a) and sagittal T2 FIESTA (b) showing an intracavitary right
atrial (yellow arrows) mass with pericardial effusion (yellow stars) [8]
87
Differential Diagnosis
1. Primary cardiac synovial sarcoma
2. Tuberculoma
What WasMisdiagnosed inThis Case andWhy?
In this case, a synovial cell carcinoma of the heart was misdiagnosed as a tuberculoma. The authors were xated on the diagnosis suggested by the patient’s social
history and, therefore, never conducted a complete diagnostic workup [8].
Discussion
In the heart, secondary lesions are more frequently encountered than primary masses
and are seen in 95% of cases. The most common histopathological subtype of
benign cardiac tumors is myxoma. It is seen in 50% of patients, followed closely by
cardiac bromas, lipomas, hemangiomas, rhabdomyomas, papillary broelastomas,
teratomas, pericardial cysts, or cystic tumor of the AV node. CMR and computed
tomography scans can be employed as adjuvant imaging modalities. Transthoracic
echocardiogram (TTE) and transesophageal echocardiogram (TEE) remain the
gold-standard diagnostic tools [9].
Patients usually present with shortness of breath, signs of heart failure, and chest
pain [10]. This patient, in our case, presented with dyspnea, fatigue, and weight loss
88
S. Shrivastava
[8]. Such nonspecic symptoms make the diagnosis of primary cardiac synovial
sarcoma very challenging. Like synovial sarcoma, a tuberculoma may also present
with vague features such as arrhythmia or congestive heart failure [11]. In this particular case, the patient’s brother was being treated for tuberculosis, and the patient
was living with him without taking prophylaxis herself. Therefore, the diagnosis of
tuberculoma was reinforced, given the patient’s social and family histories [8].
Fewer than 70 primary cardiac synovial sarcomas have been reported in the literature [12]. Pericardiocentesis was negative for neoplastic cells. However, the absence
of malignant cells does not exclude malignancy. In 2016, Ma et al. published a
prospective cohort study evaluating the causes of moderate to large pericardial effusion requiring pericardiocentesis in 140 Chinese patients. They found that neoplastic cells might be missing in 25% of malignant effusions. There was no statistically
signicant difference noted in the ADA level in the TB subset (39.56 ± 27.05) compared to the malignant subset (41.61 ± 37.52) [13]. The ADA level of 17U/I was not
high enough to classify the pericardial effusion as tuberculous. A cut-off level of 40
UI/L results in a sensitivity, specicity, and positive predictive value of 84.0%,
80.0%, and 91.0%, respectively. ADA synthesis in the body results from the factors
associated with the immune response against TB antigens [14].
There are three subtypes of synovial sarcomas: biphasic, monophasic, and undifferentiated. The architecture of biphasic type is made up of gland-like epithelial
structures and spindle cells. The epithelial part consists of amphophilic cytoplasm.
Monophasic synovial sarcomas have inltrative borders and scant amphophilic
cytoplasm, whereas the poorly differentiated subtype has high mitotic activity,
round cells, and hyperchromatic nuclei [15]. In the histological sections of the
biopsy specimens, hematoxylin and eosin staining showed spindle cell proliferation, and immunohistochemistry revealed focal expression of epithelial membrane
antigen and diffuse expression of cluster of differentiation– 99 antigen and B-cell
lymphoma 2 protein [8]. CMR and echo are not very useful in discerning a tuberculoma from a cardiac synovial sarcoma [12], but CMR may help with surgical plantication [16]. A biopsy followed by histologic analysis, immunohistochemistry,
and genetic investigation is the key to diagnosis [12].
Proper guidelines for treating primary cardiac synovial sarcoma are yet to be
established. Further research is suggested in this area, but the rarity of the disease is
a limitation for research. Although rarely possible, complete surgical excision is the
gold standard of treatment [17]. Orthotopic heart transplantation may be performed,
and its role continues to be discussed [18]. Bench surgery can be useful in accomplishing a more thorough tumor excision [19]. The authors note that their approach
to diagnosis could have been different. TEE might have provided more insight on
the tumor’s connection to the pericardium, epicardium, or the endocardium. Positron
emission tomography would have given some clues on additional masses and lymph
node involvement, while coronary angiography would have detected the extent of
vascularization of the mass. In this case, the mass could not be resected due to local
invasion, and the resulting cardiac tamponade proved fatal [8].
13 Primary Cardiac Synovial Sarcoma Misdiagnosed asTuberculoma
89
Plan ofAction: ThePoints Clinician Should Consider–
Pitfalls toAvoid
It is crucial to have a thorough medical history when dealing with intracardiac
masses. Subsequent imaging must not be delayed. The most important kinds of
imaging are TTE, TEE, coronary angiography, and cardiac MRI. Then, a biopsy
must be ordered to diagnose cardiac synovial cell carcinoma. Surgical resection is
the mainstay of treatment.
Pearls ofKnowledge toConsider
Elevated ADA levels are seen in cardiac sarcomas as well as tuberculomas.
Neoplastic disease should not be ruled out in the absence of neoplastic cells in the
pericardial uid.
Conclusion
This case speaks volumes on the importance of having an extensive scale of differential diagnoses from the start when conrming the etiology of cardiac masses. Of
special note, attention must be paid to the location of the tumor, the age of the
patient, and clinical symptoms, and a team approach must be utilized. Though hard
to think of, rare pathologies can prove to be fatal, especially if not recognized early.
Local invasion can make it impossible to resect a primary cardiac synovial cell sarcoma, hence reinforcing the need for prompt diagnosis.
References
1. Miller D, Deb A, Edwards W, Zehr K, Oliveira A. Primary synovial sarcoma of the mitral
valve. Cardiovasc Pathol. 2005;14(6):331–3. https://doi.org/10.1016/j.carpath.2005.05.002.
2. Urbini M, Astol A, Indio V, Nannini M, Pizzi C, Paolisso P, etal. Genetic aberrations and
molecular biology of cardiac sarcoma. Ther Adv Med Oncol. 2020;12:175883592091849.
https://doi.org/10.1177/1758835920918492.
3. Cupp C, Woolvin S, Inmon T. Chaotic heart due to metastatic synovioma. Am Heart
J. 1958;56(5):695–700. https://doi.org/10.1016/0002- 8703(58)90212- 6.
4. Lie J. Synovial sarcoma of the heart arising from a so-called mesothelioma of the atrioventricular node. Histopathology. 1988;13(4):191–201. https://doi.org/10.1111/j.1365-
2559.1988.tb02069.x.
5. Nicholson AG, Rigby M, Lincoln C, Meller S, Fisher C. Synovial sarcoma of the heart.
Histopathology. 1997;30(4):349–52. https://doi.org/10.1046/j.1365- 2559.1997.d01- 616.x.
90
6. Orlandi A, Ferlosio A, Roselli M, Chiariello L, Spagnoli L. Cardiac sarcomas: an update. J
Thorac Oncol. 2010;5(9):1483–9. https://doi.org/10.1097/jto.0b013e3181e59a91.
7. Varma T, Adegboyega P. Primary cardiac synovial sarcoma. Arch Pathol Lab Med.
2012;136(4):454–8. https://doi.org/10.5858/arpa.2011- 0008- RS.
8. Ouarrak S, El Ouali Z, Elkebir A, Moumna K, Karkouri M, Azzouzi L, Habbal R.Diagnostic
approach to a cardiac mass: a case report of misdiagnosed cardiac synovial sarcoma. Eur Heart
J. 2021;5(3):ytab039. https://doi.org/10.1093/ehjcr/ytab039.
9. Petris AO, Alexandrescu DM, Costache II.Cardiac tumors. Rev Med Chir Soc Med Nat Iasi.
2014;118(2):289–92.
10. McGilbray T, Schulz T. Primary cardiac synovial sarcoma. Lancet Oncol. 2003;4(5):283.
https://doi.org/10.1016/s1470- 2045(03)01075- 1.
11. Jagia P, Gulati GS, Sharma S, Goyal NK, Gaikwad S, Saxena A.MRI features of tuberculoma
of the right atrial myocardium. Pediatr Radiol. 2004;34(11):904–7. https://doi.org/10.1007/
s00247- 004- 1222- 8.
12. Wang J, Li N. Primary cardiac synovial sarcoma. Ann Thorac Surg. 2013;95(6):2202–9.
https://doi.org/10.1016/j.athoracsur.2013.01.030.
13. Ma W, Liu J, Zeng Y, Chen S, Zheng Y, Ye S, Lan L, Liu Q, Weig HJ, Liu Q.Causes of moderate to large pericardial effusion requiring pericardiocentesis in 140 Han Chinese patients.
Herz. 2012;37(2):183–7. https://doi.org/10.1007/s00059- 011- 3428- 5.
14. Reuter H, Burgess LJ, Carstens ME, Doubell AF.Adenosine deaminase activity—more than a
diagnostic tool in tuberculous pericarditis. Cardiovasc J S Afr. 2005;16(3):143–7.
15. Obeidin F, Alexiev BA.Synovial sarcoma. PathologyOutlines.com. https://www.pathology-
outlines.com/topic/softtissuesynovialsarc.html. Accessed 14 May 2022.
16. Duran-Moreno J, Kampoli K, Kapetanakis EI, Mademli M, Koufopoulos N, Foukas PG,
Kostopanagiotou K, Tomos P, Koumarianou A. Pericardial synovial sarcoma: case report,
literature review and pooled analysis. In Vivo. 2019;33(5):1531–8. https://doi.org/10.21873/
invivo.11633.
17. Coli A, Chiariello GA, Novello M, Colizzi C, Massetti M.Treatment of cardiac synovial sarcoma: experience of two cases. J Cardiothorac Surg. 2018;13(1):84. https://doi.org/10.1186/
s13019- 018- 0771- 0.
18. Gowdamarajan A, Michler RE. Therapy for primary cardiac tumors: is there a
role for heart transplantation? Curr Opin Cardiol. 2000;15(2):121–5. https://doi.
org/10.1097/00001573- 200003000- 00010.
19. Hoffmeier A, Deiters S, Schmidt C, Tjan TD, Schmid C, Drees G, Fallenberg EM, Scheld
HH.Radical resection of cardiac sarcoma. Thorac Cardiovasc Surg. 2004;52(2):77–81. https://
doi.org/10.1055/s- 2004- 817809.
S. Shrivastava
Chapter 14
Severe Eccentric Mitral Regurgitation
Misdiagnosed asSevere Aortic Stenosis
ShitijShrivastava
Learning Objectives
By the end of this presentation, the clinician will be able to:
1. Discuss the cause of the misdiagnosis of mitral regurgitation as aortic stenosis.
2. Describe how transesophageal and transthoracic echocardiography can be used
to identify mitral regurgitation and aortic stenosis.
3. Identify the factors responsible for the improvement in mitral regurgitation postsurgical aortic valve replacement.
4. Enumerate the causes of mitral regurgitation.
5. Describe and list the hemodynamic changes in patients with mitral
regurgitation.
Introduction
Aortic stenosis gradients are often underestimated in presence of severe mitral regurgitation on echocardiography [1]. Rheumatic mitral regurgitation may present with
mixed valvular pathology including severe AS.However, the hemodynamic interplay
between the two may lead to improper estimation of the severity of cardiac echocardiography [2]. Transesophageal echocardiography or transcatheter assessment should
be considered for proper measurement of gradients or MR gradings. Mitral valve
regurgitation, simply known as mitral regurgitation, is the most common valvular
heart disease followed by aortic stenosis in the western population [3]. Rheumatic
pathology is the most common cause of multivalvular disease involving younger people. Degenerative disease affects the older population involving the mitral and aortic
S. Shrivastava (*)
BronxCare Health System, Bronx, NY, USA
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
H. Tohid et al. (eds.), The Misdiagnosis Casebook in Clinical Medicine,
https://doi.org/10.1007/978-3-031-28296-6_14
91
92
valves. MR increases the volume overload of the left ventricle and also decreases the
cardiac output and stroke volume. This creates a low-ow and low-gradient situation
misleading the correct gradient across the aortic valve [4]. Aortic stenosis causes pressure overload of the left ventricle thus overestimating regurgitant volume of MR jet.
The aortic valve area may provide better information about coexisting AS in presence
of severe MR.In our case, the patient was a 75-year- old female with chronic asthma
and progressive dyspnea. She was misdiagnosed with severe aortic stenosis and moderate mitral regurgitation of degenerative etiology. Transthoracic echocardiography
(TTE) also conrmed the same diagnosis. However, TEE carried out in the operating
room (OR) revealed a normal aortic valve without any gradients and showed severe
MR with ail posterior mitral leaet due to ruptured chordae tendineae [5].
S. Shrivastava
Clinical Case Presentation
In this publication by Kumar etal., an elderly female presented with long-standing
asthma and ongoing dyspnea. She was referred to the authors as a diagnosed case of
severe aortic stenosis and moderate mitral regurgitation. She was not in failure.
On TTE, her peak vs mean gradients across the aortic valve were 113 vs 75
mmHg. The patient also had moderate mitral regurgitation with normal left ventricular function and moderate pulmonary artery hypertension. Auscultatory ndings revealed a systolic murmur at the apex and right second intercostal space
radiating to the left axilla. Before surgery, coronary angiography was done which
revealed normal coronaries.
The patient was planned for aortic valve replacement after due consent. In the OR,
TEE was performed for a detailed evaluation of the pathology. Unexpectedly, the
TEE showed ail posterior mitral leaet and ruptured chordae causing severe eccentric mitral regurgitation. The aortic leaets were opening well with good coaptation,
and the gradients measured were peak/mean as 57/41 mmHg. TTE was repeated to
understand the reason for misdiagnosis. The position of the continuous wave (CW)
Doppler was adjusted diligently to avoid the MR jet while calculating the AS gradient. This time the true gradient could be measured which turned out to be low with
near-normal aortic valve hemodynamics. Hence the surgical plan was revised after
providing the new information to the patient’s family and mitral valve replacement
was carried out. Post-surgery, a TEE was done which demonstrated normal functioning of the mitral prosthesis, and no signicant aortic valve gradients were observed.
Her post-op course was unremarkable and she was discharged after 1 week [5].
Differential Diagnosis
1. Mitral valve regurgitation
2. Aortic valve stenosis