Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

A case report of malignant primary pericardial mesothelioma with atypical imaging appearance: multimodality imaging with histopathological correlation

A case report of malignant primary pericardial mesothelioma with atypical imaging appearance:... Downloaded from https://academic.oup.com/ehjcr/advance-article-abstract/doi/10.1093/ehjcr/ytaa034/5762450 by guest on 03 March 2020 CASE REPORT European Heart Journal - Case Reports doi:10.1093/ehjcr/ytaa034 Cardiac imaging A case report of malignant primary pericardial mesothelioma with atypical imaging appearance: multimodality imaging with histopathological correlation 1 1 2 Audra Banisauskaite , Antanas Jankauskas *, Valdas Sarauskas , and 3,4 Monika Arzanau  skaite 1 2 Department of Radiology, Lithuanian University of Health Sciences, Eiveniu ˛ str. 2, Kaunas 50161, Lithuania; Department of Pathology, Lithuanian University of Health Sciences, 3 4 Eiveniu ˛ str. 2, Kaunas 50161, Lithuania; Department of Radiology and Imaging, Liverpool Heart and Chest Hospital, Thomas Dr, Liverpool L14 3PE, UK; and Cardiovascular Program ICCC, IR, HSCiSP, IIB-Sant Pau, Barcelona, Spain Received 31 August 2019; first decision 17 October 2019; accepted 28 January 2020 Background Primary pericardial mesothelioma is a rare primary cardiac malignancy, with three main histopathological types, sarcoma- toid histotype being the rarest. The imaging features were atypical due to concomitant extensive calcification, which resulted in aggravated differential diagnosis. ............................................................................................................................................................................................................... Case summary A 45-year-old man presented to our hospital with non-specific clinical symptoms. According to clinical history, a mediastin- al tumour had been suspected with a previous unsuccessful attempt of transthoracic computed tomography-guided biopsy at an overseas hospital with limited data of performed imaging procedures. Multimodality imaging at our centre revealed extensively calcified solid masses in the pericardium, invading the left atrium. As the imaging features suggested an atypical primary pericardial malignancy, a diagnostic thoracoscopy was performed. Histopathological analysis of specimen revealed sarcomatoid type of pericardial mesothelioma with areas of necrosis and foci of osteogenic differentiation. Despite planned treatment, 2 weeks after histological diagnosis, the patient passed away due to perforated peptic ulcer-related sepsis. ............................................................................................................................................................................................................... Discussion The presence of extensive calcification in the lesion resulted in a challenging imaging workup and diagnosis. Initial differen- tial diagnosis included primary or metastatic calcification-prone tumour, secondary calcification due to haemorrhage after previous interventional procedure and other pathologies, such as tuberculous pericarditis, calcified amorphous tumour, among others. Calcification may be part of the histological tumour characteristics; however, proper history taking is cru- cial as concomitant diseases, previous treatment, and interventional procedures may alter the imaging pattern. Keywords Case report Pericardium Mesothelioma Multimodality imaging Pericardial calcification • • • • Learning points Primary pericardial tumours are very rare, resulting in lack of systematic data of their imaging features. Calcification of the tumour may be due to its histological properties; however, proper history taking is crucial as concomitant diseases, pre- vious treatment, and interventional procedures may alter the imaging pattern. Multimodality imaging helps to effectively narrow the differential diagnoses of pericardial masses. * Corresponding author. Tel: þ3706574554, Email: jankauskas.antanas@gmail.com Handling Editor: Alberto Aimo Peer-reviewers: Marco De Carlo and Ciro Santoro Compliance Editor: Anastasia Vamvakidou Supplementary Material Editor: Peysh A. Patel V The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/ehjcr/advance-article-abstract/doi/10.1093/ehjcr/ytaa034/5762450 by guest on 03 March 2020 2 A. Bani sauskaite_ et al. Introduction While primary cardiac tumours are very rare, primary pericardial neoplasms are even less frequent, the most common being malignant 1–3 primary mesothelioma. The diagnosis is often delayed due to non- specific and late clinical manifestation. We report an unusual appear- ance of sarcomatoid pericardial mesothelioma in a 45-year-old man. Timeline Events ................................................................................................. Figure 1 Transthoracic echocardiography shows a solid mass in Six months prior to History of a suspected mediastinal tumour with the left atrium (asterisk), extending into the pericardial cavity. admission unsuccessful computed tomography (CT)- guided biopsy, complicated by pericardial tamponade, after which patient rejected fur- ther management in the previous hospital, . troponin I 0.16 mg/L (normal range <0.04 mg/L). Electrocardiography was started on dexamethasone for symptom (ECG) showed sinus rhythm, ST depression in II, III, aVF leads, and T- alleviation wave inversion in I, aVL, and V4–V6 leads. Chest radiograph revealed Clinical presenta- Dyspnoea, palpitations, chest pain, peripheral . left pleural effusion, left lower lobe consolidation, and enlarged car- tion at admission numbness, and hypotension diac silhouette. Transthoracic echocardiography showed a large (5 Electrocardiography (ECG): SR, ST-segment de- 4  2 cm), heterogeneous, hyperechoic mass in the left atrial (LA) pression in leads II, III, aVF, and T-wave inver- projection, extending into the pericardial cavity (Figure 1) with com- sion in leads I, aVL, and V4–V6 pression of the LA and mild mitral regurgitation. Additionally, there Imaging workup Transthoracic echocardiography, contrast- was hypokinesia of the left ventricular (LV) inferior and lateral within 2 weeks enhanced ECG-gated CT, dedicated ECG- walls—this was felt to result from limited pericardial adhesions; there after admission gated cardiovascular magnetic resonance . were no other features of constriction. LV systolic function was mild- imaging with gadolinium administration ly impaired with ejection fraction of 45%. Electrocardiography-gated Four weeks after Diagnostic thoracoscopy and subsequent histo- CT revealed a large (14  8  12 cm), heterogeneous, contrast- admission pathology successfully performed enhancing, markedly calcified pericardial lesion along with the left Five weeks after Histopathological diagnosis of primary pericar- heart extending into the LA walls, occluded left pulmonary veins and admission dial sarcomatoid mesothelioma small bilateral pleural effusions (Figure 2). Cardiovascular magnetic Eight weeks after Chemotherapy scheduled resonance (CMR) imaging, performed for further tissue characteriza- admission Developed a peptic ulcer complicated by peri- tion, confirmed a large (14  7  14 cm) solid pericardial mass tonitis and sepsis with fatal outcome extending along the anterior and lateral LV walls, invading the LA with adjacent intra-atrial thrombus (Figure 3). The mass was hetero- geneous on T1W with fat saturation (Figure 3A), enhanced after Gadolinium administration (Figure 3B) and had low values on ADC Case presentation map of diffusion-weighted imaging (DWI) (Figure 3C). The left upper A 45-year-old man presented with a 1-year history of dyspnoea, palpi- and lower pulmonary veins were invaded. There were satellite peri- tations, chest pain, peripheral numbness, weight loss, and hypotension. cardial nodules along the right atrial wall and the coronary sinus and a A mediastinal mass had been detected on chest computed tomography separate nodule in the left cardiophrenic angle. (CT) study. A transthoracic CT-guided biopsy was performed in an- Initial differential entities included primary or metastatic other hospital 6 months prior; however, the procedure was compli- calcification-prone tumour, among them a teratoma or metastatic cated by a cardiac tamponade and the tissue sample was osteosarcoma. Secondary calcification due to haemorrhage after pre- uninformative. The patient then refused further management and was vious biopsy and other pathologies, such as tuberculous pericarditis only administered dexamethasone for symptom alleviation. He had no were considered. other medical conditions and was not on any other regular medication. The benign nature was excluded after CT and CMR showed tumor- Physical examination revealed bilateral lower leg oedema, a loud sys- ous tissue plane invasion, pleural effusion, contrast enhancement, and tolic murmur, heart rate of 66 b.p.m. and respiratory rate of 23 breaths . satellite nodules. Moreover, the tuberculosis screen was negative, and per minute. The lung auscultation, blood oxygen saturation, and blood . abdominal ultrasound study was unremarkable. Due to predominant . pericardial location, a primary pericardial malignancy was suggested. pressure were normal (93% and 100/60 mmHg, respectively). Haematologic and biochemical analyses were unremarkable (in . As the previous biopsy was unsuccessful, thoracoscopy was per- particular, C-reactive protein was 15.9 mg/L (normal range <3mg/L), formed to remove the left cardiophrenic angle nodule. Downloaded from https://academic.oup.com/ehjcr/advance-article-abstract/doi/10.1093/ehjcr/ytaa034/5762450 by guest on 03 March 2020 Atypical imaging appearances of malignant pericardial mesothelioma 3 Figure 2 Contrast-enhanced computed tomography in systemic arterial phase, axial plane in soft tissue window. Solid mass along the left heart with prominent calcifications in the pericardium (A, arrowheads), bilateral pleural effusion (A, stars), and invasion of the left atrium (B,white arrows). Figure 3 Tissue characterization by cardiovascular magnetic resonance with electrocardiography-gating and breath-holding in axial (A–C) and cor- onal (D) planes. A heterogeneous contrast-enhancing solid mass on T1W VIBE with fat saturation (A) and inversion recovery 10 min after gadolinium administration (B) with low values on apparent diffusion coefficient (ADC) map of diffusion-weighted imaging (C) was seen. Additionally, there was a satellite nodule in the left cardiophrenic angle, shown on balanced steady-state free precession (SSFP) image (D, arrow) and a small left pleural effusion. Histopathology revealed a well-vascularised tumour, formed by poly- appointment with an oncologist regarding chemotherapy was sched- morphic spindle-shaped cells with a moderate amount of cytoplasm, uled. During the admission, the patient was treated with dexametha- large oval nuclei, frequent mitoses, some of which were atypical. sone (1 mg/day) and diuretics orally (torasemide 20 mg/day and There were wide necrotic areas and foci of osteogenic differenti- spironolactone 25 mg/day). A week after the histopathological diag- ation. Immunohistochemistry was positive for cytokeratin, epithelial nosis, patient’s condition worsened due to a development of a left membrane antigen (EMA), and negative for CD34, S100, smooth lower lobe pneumonia, which responded well to antibacterial treat- muscle actin (SMA), CK20, BCL-2, Anti-TLE, and CD99 (Figure 4). ment (Sulfamethoxazole–Trimethoprim 400/80 mg/b.i.d.). However, The final pathological diagnosis was thus a primary pericardial sarco- several days later, the patient experienced an acute severe abdominal matoid mesothelioma. The tumour anatomically was too advanced pain and was diagnosed with peritonitis due to a perforated peptic for surgical removal and, after multidisciplinary discussion, ulcer (confirmed by esophagogastroduodenoscopy), which may have . Downloaded from https://academic.oup.com/ehjcr/advance-article-abstract/doi/10.1093/ehjcr/ytaa034/5762450 by guest on 03 March 2020 4 A. Bani sauskaite_ et al. Figure 4 Histological and immunohistochemical analysis of the specimen: spindle shaped cells with frequent and atypical mitoses (A), positive immunohistochemical reaction with cytokeratin 20 (B), positive immunohistochemical reaction with EMA (C), and positive immunohistochemical reaction with cytokeratin 10 (D). had developed after long-term dexamethasone treatment. Patient . Echocardiography is the first-line investigation to assess for peri- underwent abdominal surgery but passed away due to sepsis 2 days cardial constriction as per ESC guidelines ; it is useful to differentiate later. fluid from solid tumours, including biphasic or sarcomatoid type peri- cardial mesotheliomas. However, it is challenging in distinguishing epithelial PPM from fluid—both are anechoic. Cross-sectional imaging can provide structural and functional in- Discussion . formation about the pericardium and heart in any anatomical plane has greater soft-tissue contrast than echocardiography and is less op- Primary pericardial mesothelioma (PPM) is an exceedingly rare malig- nant tumour (prevalence of <0.0022%) and accounts for 2–3% of pri- erator-dependent. 3,5 Computed tomography is sensitive in evaluating density of effusion mary cardiac and pericardial malignancies. Sarcomatoid histotype is and calcification and differentiating malignancy from pericarditis by the rarest, while epithelial and biphasic histotypes are more common 6 . revealing solid nodules. While ECG-gated CT is useful for assessing (75–80% of all PPM cases). . . the involvement of pericardial layers, epicardial fat, and myocardium, Primary pericardial mesothelioma can form a local solid tumour, plaque-like pericardial thickening or diffusely infiltrate the pericar- CMR is superior due to excellent tissue characterization and haemo- dynamic analysis and thus is the imaging technique of choice for dium and adjacent structures. 15,16 Although some pleural osteoblastic sarcomatoid mesotheliomas myocardial and pericardial pathology. Protocols include standard have been reported to have osteoid components, primary calcifica- T1- and T2-weighted imaging with and without fat suppression and tion of PPM is not typical. Thus a possible reason for calcification in . first-pass perfusion for vascularity; early and late Gadolinium phase our case was a component of a chronic iatrogenic haematoma after . assessment helps to define solid components, necrosis, adjacent . thrombi and to differentiate healthy myocardium from tumorous in- the first biopsy. Haematoma, experimentally produced in animals by injecting blood into pericardium, resolves without calcification. filtration and other pathology. Although cardiac DWI is not com- monly performed in clinical practice due to motion artefacts, in our Calcified haematomas occur mainly after high energy trauma, which is believed to alter processes of fibrinolysis and absorption. case, the excellent quality images showed diffusion restriction. Spin- Extensive calcification may mask imaging features of vascularity and echo sequences help to characterize pericardial effusion, especially . 15 can lead to a false-positive diagnosis of tuberculous pericarditis. . septated or complex. Diastolic septal flattening during deep inspir- Other calcification-prone lesions, such as calcified amorphous tu- . ation is diagnostic for haemodynamic compromise in pericardial con- mour, were excluded due to atypical location and morphology. striction. Phase-contrast velocity imaging is used to assess for Downloaded from https://academic.oup.com/ehjcr/advance-article-abstract/doi/10.1093/ehjcr/ytaa034/5762450 by guest on 03 March 2020 Atypical imaging appearances of malignant pericardial mesothelioma 5 chamber or vessel obstruction by the tumour and can be applied to Slide sets: A fully edited slide set detailing this case and suitable for detect the expiratory diastolic caval flow reversal in pericardial con- local presentation is available online as Supplementary data. striction. Volumetric module can accurately assess cardiac size and Consent: The author/s confirm that written consent for submission function particularly related to chemotherapy. . and publication of this case report including image(s) and associated . text has been obtained from the patient in line with COPE guidance. Conflict of interest: none declared. Conclusion Although malignant PPM is very rare, knowledge of imaging features References . 1. Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a helps to narrow the differential entities and to achieve a timely diag- review of 12,485 consecutive autopsies. Arch Pathol Lab Med 1993;117: nosis, particularly in atypical appearances. Tissue sampling and histo- . . 1027–1031. pathological analysis are crucial for the diagnosis and treatment . 2. Restrepo CS, Vargas D, Ocazionez D, Martı´nez-Jime ´ nez S, Betancourt Cuellar SL, Gutierrez FR. Primary pericardial tumors. Radiographics 2013;33:1613–1630. planning. 3. Gossinger HD, Siostrzonek P, Zangeneh M, Neuhold A, Herold C, Schmoliner R, Laczkovics A, Tscholakoff D, Mo ¨ sslacher H. Magnetic resonance imaging findings in a patient with pericardial mesothelioma. Am Heart J 1988;115:1321–1322. 4. Godar M, Liu J, Zhang P, Xia Y, Yuan Q. Primary pericardial mesothelioma: a rare entity. Case Rep Oncol Med 2013;2013:283601. 5. Battifora H, Mccaughey WTE, eds. Atlas of Tumor Pathology (AFIP Series): Tumors of Lead author biography . . the Serosal Membranes, Fascicle 15, Third Series. Washington, DC: Armed Forces Institute of Pathology, 1995. Dr Audra Bani sauskaitei _ s a . 6. Mensi C, Romano A, Berti A, Dore R, Riboldi L. A second case of pericardial finalyear radiology trainee mesothelioma mimicking systemic lupus erythematosus in the literature in over at Hospital of University of 30 years: a case report. J Med Case Rep 2017;11:85. 7. Sardar MR, Kuntz C, Patel T, Saeed W, Gnall E, Imaizumi S, Lande L. Primary Health Sciences Kaunas . pericardial mesothelioma unique case and literature review. Tex Heart Inst J 2012; Clinics. She graduated from 39:261–264. 8. Mortimer AM, Rowlands J, Murphy P. Coarse pleural calcification in a mesotheli- Lithuanian University of . . oma patient raises the possibility of a rare tumour subtype: osteoblastic sarco- Health Sciences in 2016. . matoid mesothelioma. Br J Radiol 2011;84:e106–e108. Audra is particularly inter- 9. Sbokos CG, Karayannacos PE, Kontaxis A, Kambylafkas J, Skalkeas GD. . Traumatic hemopericardium and chronic constrictive pericarditis. Ann Thorac ested in chest and cardio- Surg 1977;23:225–229. vascular imaging, presented 10. Manhas AH, Martin RT, Reul GJ, Stainback RF. Heart failure due to a post- at international congresses . traumatic calcified pericardial hematoma. Tex Heart Inst J 2008;35:345–348. 11. Basso C, Rizzo S, Valente M, Thiene G. Cardiac masses and tumours. Heart including European . 2016;102:1230–1245. Congress of Radiology and . 12. Adler Y, Charron P, Imazio M, Badano L, Baro ´ n-Esquivias G, Bogaert J, Brucato is a co-author of an article A, Gueret P, Klingel K, Lionis C, Maisch B, Mayosi B, Pavie A, Risti c AD, Sabate ´ Tenas M, Seferovic P, Swedberg K, Tomkowski W. 2015 ESC Guidelines for the published in national scientific journal. She is also an active member . diagnosis and management of pericardial diseases: the task force for the diagnosis of national and international radiology societies. and management of pericardial diseases of the European Society of Cardiology (ESC). Eur Heart J 2015;36:2921–2964. . 13. Rizzardi C, Barresi E, Brollo A, Cassetti P, Schneider M, Melato M. Primary peri- cardial mesothelioma in an asbestos-exposed patient with previous heart sur- Supplementary material gery. Anticancer Res 2010;30:1323–1325. 14. Suman S, Schofield P, Large S. Primary pericardial mesothelioma presenting as Supplementary material is available at European Heart Journal - Case pericardial constriction: a case report. Heart 2004;90:e4. 15. Peebles CR, Shambrook JS, Harden SP. Pericardial disease–anatomy and function. Reports online. Br J Radiol 2011;84 Spec No 3(Spec Iss 3):S324–S337. 16. Pennell DJ, Sechtem UP, Higgins CB, Manning WJ, Pohost GM, Rademakers FE, van Rossum AC, Shaw LJ, Yucel EK. Clinical indications for cardiovascular mag- . netic resonance (CMR): consensus panel report. Eur Heart J 2004;25:1940–1965. Acknowledgements 17. Miller CA, Dormand H, Clark D, Jones M, Bishop P, Schmitt M. Comprehensive The authors are grateful to Indre_ CeponieneM _ D, PhD and Jurgita . . characterization of constrictive pericarditis using multiparametric CMR. JACC Zaveckiene_ MD, PhD, for their contributions to this article. Cardiovasc Imaging 2011;4:917–920. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Heart Journal - Case Reports Oxford University Press

A case report of malignant primary pericardial mesothelioma with atypical imaging appearance: multimodality imaging with histopathological correlation

Download PDF
5 pages

Loading next page...
 
/lp/oxford-university-press/a-case-report-of-malignant-primary-pericardial-mesothelioma-with-htrAEXXyN9

References (39)

Publisher
Oxford University Press
Copyright
© The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology.
eISSN
2514-2119
DOI
10.1093/ehjcr/ytaa034
Publisher site
See Article on Publisher Site

Abstract

Downloaded from https://academic.oup.com/ehjcr/advance-article-abstract/doi/10.1093/ehjcr/ytaa034/5762450 by guest on 03 March 2020 CASE REPORT European Heart Journal - Case Reports doi:10.1093/ehjcr/ytaa034 Cardiac imaging A case report of malignant primary pericardial mesothelioma with atypical imaging appearance: multimodality imaging with histopathological correlation 1 1 2 Audra Banisauskaite , Antanas Jankauskas *, Valdas Sarauskas , and 3,4 Monika Arzanau  skaite 1 2 Department of Radiology, Lithuanian University of Health Sciences, Eiveniu ˛ str. 2, Kaunas 50161, Lithuania; Department of Pathology, Lithuanian University of Health Sciences, 3 4 Eiveniu ˛ str. 2, Kaunas 50161, Lithuania; Department of Radiology and Imaging, Liverpool Heart and Chest Hospital, Thomas Dr, Liverpool L14 3PE, UK; and Cardiovascular Program ICCC, IR, HSCiSP, IIB-Sant Pau, Barcelona, Spain Received 31 August 2019; first decision 17 October 2019; accepted 28 January 2020 Background Primary pericardial mesothelioma is a rare primary cardiac malignancy, with three main histopathological types, sarcoma- toid histotype being the rarest. The imaging features were atypical due to concomitant extensive calcification, which resulted in aggravated differential diagnosis. ............................................................................................................................................................................................................... Case summary A 45-year-old man presented to our hospital with non-specific clinical symptoms. According to clinical history, a mediastin- al tumour had been suspected with a previous unsuccessful attempt of transthoracic computed tomography-guided biopsy at an overseas hospital with limited data of performed imaging procedures. Multimodality imaging at our centre revealed extensively calcified solid masses in the pericardium, invading the left atrium. As the imaging features suggested an atypical primary pericardial malignancy, a diagnostic thoracoscopy was performed. Histopathological analysis of specimen revealed sarcomatoid type of pericardial mesothelioma with areas of necrosis and foci of osteogenic differentiation. Despite planned treatment, 2 weeks after histological diagnosis, the patient passed away due to perforated peptic ulcer-related sepsis. ............................................................................................................................................................................................................... Discussion The presence of extensive calcification in the lesion resulted in a challenging imaging workup and diagnosis. Initial differen- tial diagnosis included primary or metastatic calcification-prone tumour, secondary calcification due to haemorrhage after previous interventional procedure and other pathologies, such as tuberculous pericarditis, calcified amorphous tumour, among others. Calcification may be part of the histological tumour characteristics; however, proper history taking is cru- cial as concomitant diseases, previous treatment, and interventional procedures may alter the imaging pattern. Keywords Case report Pericardium Mesothelioma Multimodality imaging Pericardial calcification • • • • Learning points Primary pericardial tumours are very rare, resulting in lack of systematic data of their imaging features. Calcification of the tumour may be due to its histological properties; however, proper history taking is crucial as concomitant diseases, pre- vious treatment, and interventional procedures may alter the imaging pattern. Multimodality imaging helps to effectively narrow the differential diagnoses of pericardial masses. * Corresponding author. Tel: þ3706574554, Email: jankauskas.antanas@gmail.com Handling Editor: Alberto Aimo Peer-reviewers: Marco De Carlo and Ciro Santoro Compliance Editor: Anastasia Vamvakidou Supplementary Material Editor: Peysh A. Patel V The Author(s) 2020. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/ehjcr/advance-article-abstract/doi/10.1093/ehjcr/ytaa034/5762450 by guest on 03 March 2020 2 A. Bani sauskaite_ et al. Introduction While primary cardiac tumours are very rare, primary pericardial neoplasms are even less frequent, the most common being malignant 1–3 primary mesothelioma. The diagnosis is often delayed due to non- specific and late clinical manifestation. We report an unusual appear- ance of sarcomatoid pericardial mesothelioma in a 45-year-old man. Timeline Events ................................................................................................. Figure 1 Transthoracic echocardiography shows a solid mass in Six months prior to History of a suspected mediastinal tumour with the left atrium (asterisk), extending into the pericardial cavity. admission unsuccessful computed tomography (CT)- guided biopsy, complicated by pericardial tamponade, after which patient rejected fur- ther management in the previous hospital, . troponin I 0.16 mg/L (normal range <0.04 mg/L). Electrocardiography was started on dexamethasone for symptom (ECG) showed sinus rhythm, ST depression in II, III, aVF leads, and T- alleviation wave inversion in I, aVL, and V4–V6 leads. Chest radiograph revealed Clinical presenta- Dyspnoea, palpitations, chest pain, peripheral . left pleural effusion, left lower lobe consolidation, and enlarged car- tion at admission numbness, and hypotension diac silhouette. Transthoracic echocardiography showed a large (5 Electrocardiography (ECG): SR, ST-segment de- 4  2 cm), heterogeneous, hyperechoic mass in the left atrial (LA) pression in leads II, III, aVF, and T-wave inver- projection, extending into the pericardial cavity (Figure 1) with com- sion in leads I, aVL, and V4–V6 pression of the LA and mild mitral regurgitation. Additionally, there Imaging workup Transthoracic echocardiography, contrast- was hypokinesia of the left ventricular (LV) inferior and lateral within 2 weeks enhanced ECG-gated CT, dedicated ECG- walls—this was felt to result from limited pericardial adhesions; there after admission gated cardiovascular magnetic resonance . were no other features of constriction. LV systolic function was mild- imaging with gadolinium administration ly impaired with ejection fraction of 45%. Electrocardiography-gated Four weeks after Diagnostic thoracoscopy and subsequent histo- CT revealed a large (14  8  12 cm), heterogeneous, contrast- admission pathology successfully performed enhancing, markedly calcified pericardial lesion along with the left Five weeks after Histopathological diagnosis of primary pericar- heart extending into the LA walls, occluded left pulmonary veins and admission dial sarcomatoid mesothelioma small bilateral pleural effusions (Figure 2). Cardiovascular magnetic Eight weeks after Chemotherapy scheduled resonance (CMR) imaging, performed for further tissue characteriza- admission Developed a peptic ulcer complicated by peri- tion, confirmed a large (14  7  14 cm) solid pericardial mass tonitis and sepsis with fatal outcome extending along the anterior and lateral LV walls, invading the LA with adjacent intra-atrial thrombus (Figure 3). The mass was hetero- geneous on T1W with fat saturation (Figure 3A), enhanced after Gadolinium administration (Figure 3B) and had low values on ADC Case presentation map of diffusion-weighted imaging (DWI) (Figure 3C). The left upper A 45-year-old man presented with a 1-year history of dyspnoea, palpi- and lower pulmonary veins were invaded. There were satellite peri- tations, chest pain, peripheral numbness, weight loss, and hypotension. cardial nodules along the right atrial wall and the coronary sinus and a A mediastinal mass had been detected on chest computed tomography separate nodule in the left cardiophrenic angle. (CT) study. A transthoracic CT-guided biopsy was performed in an- Initial differential entities included primary or metastatic other hospital 6 months prior; however, the procedure was compli- calcification-prone tumour, among them a teratoma or metastatic cated by a cardiac tamponade and the tissue sample was osteosarcoma. Secondary calcification due to haemorrhage after pre- uninformative. The patient then refused further management and was vious biopsy and other pathologies, such as tuberculous pericarditis only administered dexamethasone for symptom alleviation. He had no were considered. other medical conditions and was not on any other regular medication. The benign nature was excluded after CT and CMR showed tumor- Physical examination revealed bilateral lower leg oedema, a loud sys- ous tissue plane invasion, pleural effusion, contrast enhancement, and tolic murmur, heart rate of 66 b.p.m. and respiratory rate of 23 breaths . satellite nodules. Moreover, the tuberculosis screen was negative, and per minute. The lung auscultation, blood oxygen saturation, and blood . abdominal ultrasound study was unremarkable. Due to predominant . pericardial location, a primary pericardial malignancy was suggested. pressure were normal (93% and 100/60 mmHg, respectively). Haematologic and biochemical analyses were unremarkable (in . As the previous biopsy was unsuccessful, thoracoscopy was per- particular, C-reactive protein was 15.9 mg/L (normal range <3mg/L), formed to remove the left cardiophrenic angle nodule. Downloaded from https://academic.oup.com/ehjcr/advance-article-abstract/doi/10.1093/ehjcr/ytaa034/5762450 by guest on 03 March 2020 Atypical imaging appearances of malignant pericardial mesothelioma 3 Figure 2 Contrast-enhanced computed tomography in systemic arterial phase, axial plane in soft tissue window. Solid mass along the left heart with prominent calcifications in the pericardium (A, arrowheads), bilateral pleural effusion (A, stars), and invasion of the left atrium (B,white arrows). Figure 3 Tissue characterization by cardiovascular magnetic resonance with electrocardiography-gating and breath-holding in axial (A–C) and cor- onal (D) planes. A heterogeneous contrast-enhancing solid mass on T1W VIBE with fat saturation (A) and inversion recovery 10 min after gadolinium administration (B) with low values on apparent diffusion coefficient (ADC) map of diffusion-weighted imaging (C) was seen. Additionally, there was a satellite nodule in the left cardiophrenic angle, shown on balanced steady-state free precession (SSFP) image (D, arrow) and a small left pleural effusion. Histopathology revealed a well-vascularised tumour, formed by poly- appointment with an oncologist regarding chemotherapy was sched- morphic spindle-shaped cells with a moderate amount of cytoplasm, uled. During the admission, the patient was treated with dexametha- large oval nuclei, frequent mitoses, some of which were atypical. sone (1 mg/day) and diuretics orally (torasemide 20 mg/day and There were wide necrotic areas and foci of osteogenic differenti- spironolactone 25 mg/day). A week after the histopathological diag- ation. Immunohistochemistry was positive for cytokeratin, epithelial nosis, patient’s condition worsened due to a development of a left membrane antigen (EMA), and negative for CD34, S100, smooth lower lobe pneumonia, which responded well to antibacterial treat- muscle actin (SMA), CK20, BCL-2, Anti-TLE, and CD99 (Figure 4). ment (Sulfamethoxazole–Trimethoprim 400/80 mg/b.i.d.). However, The final pathological diagnosis was thus a primary pericardial sarco- several days later, the patient experienced an acute severe abdominal matoid mesothelioma. The tumour anatomically was too advanced pain and was diagnosed with peritonitis due to a perforated peptic for surgical removal and, after multidisciplinary discussion, ulcer (confirmed by esophagogastroduodenoscopy), which may have . Downloaded from https://academic.oup.com/ehjcr/advance-article-abstract/doi/10.1093/ehjcr/ytaa034/5762450 by guest on 03 March 2020 4 A. Bani sauskaite_ et al. Figure 4 Histological and immunohistochemical analysis of the specimen: spindle shaped cells with frequent and atypical mitoses (A), positive immunohistochemical reaction with cytokeratin 20 (B), positive immunohistochemical reaction with EMA (C), and positive immunohistochemical reaction with cytokeratin 10 (D). had developed after long-term dexamethasone treatment. Patient . Echocardiography is the first-line investigation to assess for peri- underwent abdominal surgery but passed away due to sepsis 2 days cardial constriction as per ESC guidelines ; it is useful to differentiate later. fluid from solid tumours, including biphasic or sarcomatoid type peri- cardial mesotheliomas. However, it is challenging in distinguishing epithelial PPM from fluid—both are anechoic. Cross-sectional imaging can provide structural and functional in- Discussion . formation about the pericardium and heart in any anatomical plane has greater soft-tissue contrast than echocardiography and is less op- Primary pericardial mesothelioma (PPM) is an exceedingly rare malig- nant tumour (prevalence of <0.0022%) and accounts for 2–3% of pri- erator-dependent. 3,5 Computed tomography is sensitive in evaluating density of effusion mary cardiac and pericardial malignancies. Sarcomatoid histotype is and calcification and differentiating malignancy from pericarditis by the rarest, while epithelial and biphasic histotypes are more common 6 . revealing solid nodules. While ECG-gated CT is useful for assessing (75–80% of all PPM cases). . . the involvement of pericardial layers, epicardial fat, and myocardium, Primary pericardial mesothelioma can form a local solid tumour, plaque-like pericardial thickening or diffusely infiltrate the pericar- CMR is superior due to excellent tissue characterization and haemo- dynamic analysis and thus is the imaging technique of choice for dium and adjacent structures. 15,16 Although some pleural osteoblastic sarcomatoid mesotheliomas myocardial and pericardial pathology. Protocols include standard have been reported to have osteoid components, primary calcifica- T1- and T2-weighted imaging with and without fat suppression and tion of PPM is not typical. Thus a possible reason for calcification in . first-pass perfusion for vascularity; early and late Gadolinium phase our case was a component of a chronic iatrogenic haematoma after . assessment helps to define solid components, necrosis, adjacent . thrombi and to differentiate healthy myocardium from tumorous in- the first biopsy. Haematoma, experimentally produced in animals by injecting blood into pericardium, resolves without calcification. filtration and other pathology. Although cardiac DWI is not com- monly performed in clinical practice due to motion artefacts, in our Calcified haematomas occur mainly after high energy trauma, which is believed to alter processes of fibrinolysis and absorption. case, the excellent quality images showed diffusion restriction. Spin- Extensive calcification may mask imaging features of vascularity and echo sequences help to characterize pericardial effusion, especially . 15 can lead to a false-positive diagnosis of tuberculous pericarditis. . septated or complex. Diastolic septal flattening during deep inspir- Other calcification-prone lesions, such as calcified amorphous tu- . ation is diagnostic for haemodynamic compromise in pericardial con- mour, were excluded due to atypical location and morphology. striction. Phase-contrast velocity imaging is used to assess for Downloaded from https://academic.oup.com/ehjcr/advance-article-abstract/doi/10.1093/ehjcr/ytaa034/5762450 by guest on 03 March 2020 Atypical imaging appearances of malignant pericardial mesothelioma 5 chamber or vessel obstruction by the tumour and can be applied to Slide sets: A fully edited slide set detailing this case and suitable for detect the expiratory diastolic caval flow reversal in pericardial con- local presentation is available online as Supplementary data. striction. Volumetric module can accurately assess cardiac size and Consent: The author/s confirm that written consent for submission function particularly related to chemotherapy. . and publication of this case report including image(s) and associated . text has been obtained from the patient in line with COPE guidance. Conflict of interest: none declared. Conclusion Although malignant PPM is very rare, knowledge of imaging features References . 1. Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a helps to narrow the differential entities and to achieve a timely diag- review of 12,485 consecutive autopsies. Arch Pathol Lab Med 1993;117: nosis, particularly in atypical appearances. Tissue sampling and histo- . . 1027–1031. pathological analysis are crucial for the diagnosis and treatment . 2. Restrepo CS, Vargas D, Ocazionez D, Martı´nez-Jime ´ nez S, Betancourt Cuellar SL, Gutierrez FR. Primary pericardial tumors. Radiographics 2013;33:1613–1630. planning. 3. Gossinger HD, Siostrzonek P, Zangeneh M, Neuhold A, Herold C, Schmoliner R, Laczkovics A, Tscholakoff D, Mo ¨ sslacher H. Magnetic resonance imaging findings in a patient with pericardial mesothelioma. Am Heart J 1988;115:1321–1322. 4. Godar M, Liu J, Zhang P, Xia Y, Yuan Q. Primary pericardial mesothelioma: a rare entity. Case Rep Oncol Med 2013;2013:283601. 5. Battifora H, Mccaughey WTE, eds. Atlas of Tumor Pathology (AFIP Series): Tumors of Lead author biography . . the Serosal Membranes, Fascicle 15, Third Series. Washington, DC: Armed Forces Institute of Pathology, 1995. Dr Audra Bani sauskaitei _ s a . 6. Mensi C, Romano A, Berti A, Dore R, Riboldi L. A second case of pericardial finalyear radiology trainee mesothelioma mimicking systemic lupus erythematosus in the literature in over at Hospital of University of 30 years: a case report. J Med Case Rep 2017;11:85. 7. Sardar MR, Kuntz C, Patel T, Saeed W, Gnall E, Imaizumi S, Lande L. Primary Health Sciences Kaunas . pericardial mesothelioma unique case and literature review. Tex Heart Inst J 2012; Clinics. She graduated from 39:261–264. 8. Mortimer AM, Rowlands J, Murphy P. Coarse pleural calcification in a mesotheli- Lithuanian University of . . oma patient raises the possibility of a rare tumour subtype: osteoblastic sarco- Health Sciences in 2016. . matoid mesothelioma. Br J Radiol 2011;84:e106–e108. Audra is particularly inter- 9. Sbokos CG, Karayannacos PE, Kontaxis A, Kambylafkas J, Skalkeas GD. . Traumatic hemopericardium and chronic constrictive pericarditis. Ann Thorac ested in chest and cardio- Surg 1977;23:225–229. vascular imaging, presented 10. Manhas AH, Martin RT, Reul GJ, Stainback RF. Heart failure due to a post- at international congresses . traumatic calcified pericardial hematoma. Tex Heart Inst J 2008;35:345–348. 11. Basso C, Rizzo S, Valente M, Thiene G. Cardiac masses and tumours. Heart including European . 2016;102:1230–1245. Congress of Radiology and . 12. Adler Y, Charron P, Imazio M, Badano L, Baro ´ n-Esquivias G, Bogaert J, Brucato is a co-author of an article A, Gueret P, Klingel K, Lionis C, Maisch B, Mayosi B, Pavie A, Risti c AD, Sabate ´ Tenas M, Seferovic P, Swedberg K, Tomkowski W. 2015 ESC Guidelines for the published in national scientific journal. She is also an active member . diagnosis and management of pericardial diseases: the task force for the diagnosis of national and international radiology societies. and management of pericardial diseases of the European Society of Cardiology (ESC). Eur Heart J 2015;36:2921–2964. . 13. Rizzardi C, Barresi E, Brollo A, Cassetti P, Schneider M, Melato M. Primary peri- cardial mesothelioma in an asbestos-exposed patient with previous heart sur- Supplementary material gery. Anticancer Res 2010;30:1323–1325. 14. Suman S, Schofield P, Large S. Primary pericardial mesothelioma presenting as Supplementary material is available at European Heart Journal - Case pericardial constriction: a case report. Heart 2004;90:e4. 15. Peebles CR, Shambrook JS, Harden SP. Pericardial disease–anatomy and function. Reports online. Br J Radiol 2011;84 Spec No 3(Spec Iss 3):S324–S337. 16. Pennell DJ, Sechtem UP, Higgins CB, Manning WJ, Pohost GM, Rademakers FE, van Rossum AC, Shaw LJ, Yucel EK. Clinical indications for cardiovascular mag- . netic resonance (CMR): consensus panel report. Eur Heart J 2004;25:1940–1965. Acknowledgements 17. Miller CA, Dormand H, Clark D, Jones M, Bishop P, Schmitt M. Comprehensive The authors are grateful to Indre_ CeponieneM _ D, PhD and Jurgita . . characterization of constrictive pericarditis using multiparametric CMR. JACC Zaveckiene_ MD, PhD, for their contributions to this article. Cardiovasc Imaging 2011;4:917–920.

Journal

European Heart Journal - Case Reports – Oxford University Press

Published: Jun 1, 2020

There are no references for this article.