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Primary Cardiac Epithelioid Angiosarcoma in a Latin American Patient: Case Report and Literature Review

Primary Cardiac Epithelioid Angiosarcoma in a Latin American Patient: Case Report and Literature... Hindawi Case Reports in Oncological Medicine Volume 2019, Article ID 2641976, 4 pages https://doi.org/10.1155/2019/2641976 Case Report Primary Cardiac Epithelioid Angiosarcoma in a Latin American Patient: Case Report and Literature Review 1 2 1 Dan Morgenstern-Kaplan , Carlos Manuel Aboitiz-Rivera, Ruben Blachman-Braun , 3 2 María Eugenia Vázquez-Manríquez, Benito Sarabia-Ortega, and Mario Enrique Baltazares-Lipp Centro de Investigación en Ciencias de la Salud (CICSA), Facultad de Ciencias de la Salud, Universidad Anáhuac Mexico Campus Norte, Huixquilucan, Edo. de Mexico, Mexico Echocardiography and Hemodynamics Service, Instituto Nacional de Enfermedades Respiratorias, Tlalpan, Mexico City, Mexico Department of Pathology, Instituto Nacional de Enfermedades Respiratorias, Tlalpan, Mexico City, Mexico Correspondence should be addressed to Ruben Blachman-Braun; rubenblachman@gmail.com Received 17 April 2019; Accepted 29 June 2019; Published 30 July 2019 Academic Editor: Jose I. Mayordomo Copyright © 2019 Dan Morgenstern-Kaplan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Cardiac angiosarcoma is a rare and clinically challenging pathology. It is a high-grade primary malignant tumor of the heart tissue that has many variants, of which the epithelioid variant is rarely present in the heart or the great vessels. As with many other cardiac tumors, it is mainly a diagnosis of exclusion and the initial diagnostic test is an echocardiogram followed by a biopsy with immunohistochemistry analysis to ascertain the type of tumor. The differential diagnosis of cardiac tumors is challenging due to the overlapping clinical manifestations with different cardiac tumors and systemic diseases. Cardiac angiosarcomas are often aggressive with a poor prognosis even with treatment. Herein, we present a case of the epithelioid variant of a cardiac angiosarcoma in addition to a thorough review of the recent literature on the clinical manifestation, diagnosis, and treatment of this type of tumors. 1. Introduction mented reports of this type of tumor [4, 5]. Herein, we pres- ent a case of a primary cardiac epithelioid angiosarcoma and a review of the literature. Cardiac tumors are rare, and, of these, metastatic tumors to the heart are nearly 30 times more common than primary cardiac 2. Case Report tumors. The estimated prevalence for primary cardiac tumors ranges from 0.0017% to 0.33% [1, 2]; of these, 90% are benign, A 20-year-old Hispanic male was referred for cardiologic eval- with myxomas being the most frequent. Of the 10% that are uation with the presence of thoracic oppressive pain and pro- malignant tumors, the most common are sarcomas, with gressively worsening dyspnea, orthopnea, dysphagia, and 8 kg undifferentiated pleomorphic sarcoma and angiosarcoma of weight loss in the last month. A chest computerized tomog- being the most prevalent histological subtypes, accounting for raphy (CT) scan was performed, reporting the presence of a 40% of all the malignant primary cardiac neoplasms [2]. The hyperdense mass within the pericardial space, with infiltration epithelioid variant is rarely present in cardiac tissue (less than into the myocardium, displacing and compressing adjacent 3% of all angiosarcomas are in the heart and/or great vessels), structures, with lymphadenopathy adjacent to the mass. In and it has been reported in many other tissues and organs addition to the mediastinal mass, several hyperdense masses (i.e., soft tissues and skin, especially in the extremities) [3]. were observed in the right lung (Figure 1). Afterward, trans- Primary cardiac epithelioid angiosarcoma is extremely thoracic echocardiography was performed, reporting pericar- rare; to our knowledge, there are only another seven docu- dial effusion with the presence of a mediastinal mass that 2 Case Reports in Oncological Medicine Figure 1: Chest CT scan with IV contrast showing a hyperdense mass within the pericardial space, with infiltration into the (a) myocardium, displacing and compressing adjacent structures. compromised all four cardiac cavities, infiltrating both atria and partially obstructing the right atrium (Figure 2). A biopsy was taken (Figure 3), and, macroscopically, the specimen was a 7×4 × 2cm lung parenchyma, with multiple, well-circumscribed solid brown nodular lesions surrounded by collapsed lung tissue. Histological analysis was consistent with a malignant neoplasia of mesenchymal origin with mostly polygonal cells and some spindle-shaped cells arranged around the blood vessels. The cells were pleomor- phic with vesiculated nuclei, prominent nucleoli, and a high atypical mitotic rate. The cells were surrounded by a net of capillaries with pleomorphic endothelial cells. In immuno- (b) histochemical analysis, neoplastic cells were intensely posi- tive for CD34 and CD31, positive for vimentin, and weakly Figure 2: Transthoracic echocardiography. (a) Parasternal short positive for angiotensin-converting enzyme (ACE), cytokera- axis view showing tumoral infiltration around the aortic root tin AE1/AE3 (CKAE1/AE3), and epithelial cell adhesion without obstruction of the outflow tract. (b) Apical 4-chamber molecule (BerEP4), with a Ki-67 of 50%. This pathological view showing myocardial infiltration to the interatrial septum and the free wall of the right atrium and ventricle. analysis was consistent with the diagnosis of metastatic mul- tifocal epithelioid angiosarcoma. After the pathological diagnosis was made, the patient was readmitted with symptoms of superior vena cava syn- Cardiac tumors are generally diagnosed after excluding drome. It was then decided to start with the first cycle of che- other more common pathologies. Echocardiography is the motherapy with a combination of ifosfamide, epirubicin, and diagnostic tool of choice for the initial evaluation of cardiac cisplatin. He did not respond to treatment, and the patient neoplasms and the hemodynamic and functional effects of with his family decided to forgo treatment. The patient was the tumor. The location of the tumor is also of diagnostic discharged to palliative care at home. use; for example, the most common location for cardiac myx- omas is the left atrium, while the most common location for an epithelioid angiosarcoma is the right atrium [8]. Trans- 3. Discussion esophageal echocardiography has 97% sensitivity in detecting Cardiac angiosarcomas are rare entities, and the diagnosis cardiac masses, and it can help identify its location, size, shape, attachment, and mobility, characteristics that might and treatment of any cardiac tumor are a challenge for clini- cians. Most benign cardiac tumors originate in the atria, provide some insights into the possible mass etiology [9]. more specifically in the left atrium, and cause different clini- Most patients with malignant neoplasms present with dis- cal presentations depending on the location of the tumor seminated disease at the time of diagnosis, with the lungs inside the heart, more than its histological subtype. They being the most frequent site of metastases; however, other can cause constitutional and systemic symptoms, obstructive metastasis sites include the liver, lymph nodes, bone, adrenal and restrictive cardiac symptoms, arrhythmias, and systemic glands, and spleen. Thus, for the staging of this type of malig- embolization [1, 6]. The most prevalent symptoms that nancies, other required studies include CT scan, magnetic patients with primary cardiac malignant tumors complain resonance imaging (MRI), echocardiography, and in some about are exertional dyspnea, chest pain, and palpitations cases, positron electron tomography (PET) scan [2]. In the [7]. Though these symptoms are not specific of cardiac cases of malignant tumors, imaging would show a mass infil- malignancies, in the context of systemic symptoms imaging trating into more than one cardiac chamber and into the studies of the thorax are recommended to assess the cardiac aorta in most cases; this causes a restriction of heart move- and thoracic anatomy. ment and is highly suggestive of malignancy. Case Reports in Oncological Medicine 3 (a) (b) (c) Figure 3: (a) Microphotography H&E (20x). In the center, we can observe a blood vessel surrounded by neoplastic cells in two different patterns: cells forming nests and spindle cells fusing with each other. (b) Microphotography H&E (40x). The tumoral cells are shown in detail, with a medium to large size, scant cytoplasm, oval nuclei of vesicular chromatin, prominent nucleolus, and several mitotic figures. (c) Immunochemistry of the CD31 marker: the neoplastic cells’ cytoplasm and membrane are positive, while the bronchial epithelium is negative. In the case of malignant cardiac tumors, the differen- When the diagnosis is uncertain through imaging stud- tial diagnosis includes primarily benign cardiac tumors, ies, biopsy determines the histological type and subtype of which can be suspected clinically with the classic triad of the tumor. Microscopic analysis of epithelioid angiosarcomas cardiac obstruction, embolism, and constitutional symp- includes complex interanastomosing vessels lined with epi- thelioid endothelial cells to solid sheets of atypical epithelioid toms. Other diagnoses include intracardiac thrombi, vege- tations and other infective masses, and calcified lesions. endothelial cells. Tumor cells are polygonal with prominent This can be differentiated by imaging studies previously nuclear atypia, intracytoplasmic vacuoles, and abundant described. Cardiac masses with intramural growth or infil- amphophilic cytoplasm with tumor necrosis often seen [3]. tration often produce conduction defects or arrhythmias All pathology analyses should include IHC, and a large that can sometimes be fatal; myocardial infiltration can panel of antibodies must be performed to avoid misdiagnosis also produce congestive heart failure secondary to systolic because a number of cell markers are shared by many types of and/or diastolic dysfunction. Other manifestations include tumors, such as pancytokeratins. Epithelioid angiosarcoma is positive for vascular markers such as CD31, CD34, and ERG myocardial ischemia or infarction secondary to infiltration and compression of coronary arteries. These are the signs transcription factor. It is also positive for cytokeratin (indic- that suggest malignancy. Pericardial effusion is reported in ative of epithelial cells and angiosarcoma), FLI-1, high Ki-67, both benign and malignant cardiac tumors, but hemor- p53 (positive in 20% of cases), and vimentin (a marker of rhagic effusion or tamponade is suggestive of malignancies mesenchymal cells) [3, 11]. as well [2]. IHC is also useful to determine the potential use of tar- The lung is a common site of metastasis from soft tis- geted therapy in oncology, and in these particular cases it sue sarcomas, both from the thorax and the extremities. would be useful to search for the presence of important gene Leiomyosarcoma (21%), high-grade pleomorphic sarcomas markers for current targeted therapy—KDR and FLT- (18%), synovial sarcomas (14%), and liposarcomas (12%) 4—which encode for VEGFR-2 and VEGFR-3, respectively. These markers are receptor tyrosine kinases present in angio- are the most common sarcomas that metastasize to the lung. Furthermore, all these tumors should be in the dif- sarcomas, and their presence has shown tumors to be sensi- ferential diagnosis of lesions like these in order to deter- tive or responsive to sorafenib, a tyrosine kinase inhibitor mine their origin and should be studied by pathologic used in a wide range of cancers [12]. analysis with different techniques such as optic micros- Cardiac angiosarcomas usually have a poor prognosis; they most frequently affect men aged 40 to 50 years, and copy, immunohistochemistry (IHC), or cytogenetics [10]. 4 Case Reports in Oncological Medicine Deutsches Äerzteblatt International, vol. 111, no. 12, pp. 205– the prognosis is worse in younger patients, such as the patient 211, 2014. in this case report. The mean survival rate of cardiac angio- sarcomas is 9 3±4 2 months without surgical treatment; in [7] L. Yu, T. Gu, E. Shi et al., “Primary malignant cardiac tumors,” Journal of Cancer Research and Clinical Oncology, vol. 140, some cases, where the extent of the disease and the anatom- no. 6, pp. 1047–1055, 2014. ical location allow a complete resection, surgery might be considered a treatment option [13]. On the other hand, che- [8] C. Liu, Y. Zhao, Z. Yin et al., “Right atrial epithelioid angiosar- coma with multiple pulmonary metastasis confirmed by multi- motherapy with doxorubicin and a combination of antineo- modality imaging-guided pulmonary biopsy,” Medicine, plastic drugs has been shown to increase mean survival vol. 97, no. 30, article e11588, 2018. rate, and radiation therapy is also an option that has been [9] S. D. Patel, A. Peterson, A. Bartczak et al., “Primary cardiac associated with a better survival rate. Pegylated-liposomal angiosarcoma—a review,” Medical Science Monitor, vol. 20, doxorubicin has shown promising results in other sarcomas, pp. 103–109, 2014. such as Kaposi’s sarcoma, but no randomized controlled [10] S. Suster and C. Moran, Diagnostic Pathology: Thoracic, Else- clinical trials have been conducted to assess efficacy and vier, 2012. safety [14]. Other chemotherapeutic drugs such as cisplatin, cyclophosphamide, dacarbazine, ifosfamide, mitomycin-C, [11] A. Saqi, R. B. Ramdall, and D. Hamele-Bena, “Metastatic epi- thelioid angiosarcoma of the heart in peritoneal fluid,” Diag- paclitaxel, and vincristine are commonly prescribed, but no nostic Cytopathology, vol. 30, no. 5, pp. 350–352, 2004. standardized treatment algorithms exist as of this moment, although good algorithm proposals have been made [15]. In [12] T. Guo, L. Zhang, N. E. Chang, S. Singer, R. G. Maki, and C. R. Antonescu, “Consistent MYC and FLT4 gene amplification in this patient, palliative care was decided in combination with radiation-induced angiosarcoma but not in other radiation- the medical team, the patient, and his family members. associated atypical vascular lesions,” Genes, Chromosomes & Though information related to the best therapeutic options Cancer, vol. 50, no. 1, pp. 25–33, 2011. and outcomes of this type of pathology is insufficient, as just [13] C. Lestuzzi, A. De Paoli, T. Baresic, G. Miolo, and a handful of patients with the diagnosis of primary cardiac A. Buonadonna, “Malignant cardiac tumors: diagnosis and epithelioid angiosarcoma has been reported, we hope that treatment,” Future Cardiology, vol. 11, no. 4, pp. 485–500, this article provides further insight into this pathology. [14] D. W. Northfelt, B. J. Dezube, J. A. Thommes et al., “Efficacy of 4. Conclusion pegylated-liposomal doxorubicin in the treatment of AIDS- related Kaposi’s sarcoma after failure of standard chemother- Cardiac epithelioid angiosarcomas are rare. Although echo- apy,” Journal of Clinical Oncology, vol. 15, no. 2, pp. 653– cardiography and CT scan might suggest the pretense of a 659, 1997. malignant cardiac neoplasm, immunohistochemical diagno- [15] I. A. Paraskevaidis, C. A. Michalakeas, C. H. Papadopoulos, sis is required to confirm the diagnosis. When cardiac epithe- and M. Anastasiou-Nana, “Cardiac tumors,” ISRN Oncology, lioid angiosarcomas are diagnosed, prognosis is poor. vol. 2011, 5 pages, 2011. Conflicts of Interest The authors declare that there are no conflicts of interest regarding the publication of this article. References [1] B. Hudzik, K. Miszalski-Jamka, J. Glowacki et al., “Malignant tumors of the heart,” Cancer Epidemiology, vol. 39, no. 5, pp. 665–672, 2015. [2] C. Basso, S. Rizzo, M. Valente, and G. Thiene, “Cardiac masses and tumours,” Heart, vol. 102, no. 15, pp. 1230–1245, 2016. [3] J. S. Ko and S. D. Billings, “Diagnostically challenging epitheli- oid vascular tumors,” Surgical Pathology Clinics, vol. 8, no. 3, pp. 331–351, 2015. [4] L. A. M. Matzke, M. A. Knowling, D. Grant et al., “A rare car- diac neoplasm: case report of cardiac epithelioid angiosar- coma,” Cardiovascular Pathoogy, vol. 20, no. 5, pp. e197– e201, 2011. [5] P. Nirmalanantham, R. M. Elliott, B. Fitzsimons et al., “Pri- mary cardiac epithelioid angiosarcoma with frond-like fea- tures: a rare and ominous radiological mimicker of benign cardiac tumors,” Cardiovascular Pathology, vol. 41, pp. 18– 20, 2019. [6] A. Hoffmeier, J. R. Sindermann, H. H. Scheld, and S. Martens, “Cardiac tumors—Diagnosis and Surgical Treatment,” MEDIATORS of INFLAMMATION The Scientific Gastroenterology Journal of World Journal Research and Practice Diabetes Research Disease Markers Hindawi Hindawi Publishing Corporation Hindawi Hindawi Hindawi Hindawi www.hindawi.com Volume 2018 http://www www.hindawi.com .hindawi.com V Volume 2018 olume 2013 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 International Journal of Journal of Immunology Research Endocrinology Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 Submit your manuscripts at www.hindawi.com BioMed PPAR Research Research International Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 Journal of Obesity Evidence-Based Journal of Journal of Stem Cells Complementary and Ophthalmology International Alternative Medicine Oncology Hindawi Hindawi Hindawi Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2013 Parkinson’s Disease Computational and Behavioural Mathematical Methods AIDS Oxidative Medicine and in Medicine Neurology Research and Treatment Cellular Longevity Hindawi Hindawi Hindawi Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Case Reports in Oncological Medicine Hindawi Publishing Corporation

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Abstract

Hindawi Case Reports in Oncological Medicine Volume 2019, Article ID 2641976, 4 pages https://doi.org/10.1155/2019/2641976 Case Report Primary Cardiac Epithelioid Angiosarcoma in a Latin American Patient: Case Report and Literature Review 1 2 1 Dan Morgenstern-Kaplan , Carlos Manuel Aboitiz-Rivera, Ruben Blachman-Braun , 3 2 María Eugenia Vázquez-Manríquez, Benito Sarabia-Ortega, and Mario Enrique Baltazares-Lipp Centro de Investigación en Ciencias de la Salud (CICSA), Facultad de Ciencias de la Salud, Universidad Anáhuac Mexico Campus Norte, Huixquilucan, Edo. de Mexico, Mexico Echocardiography and Hemodynamics Service, Instituto Nacional de Enfermedades Respiratorias, Tlalpan, Mexico City, Mexico Department of Pathology, Instituto Nacional de Enfermedades Respiratorias, Tlalpan, Mexico City, Mexico Correspondence should be addressed to Ruben Blachman-Braun; rubenblachman@gmail.com Received 17 April 2019; Accepted 29 June 2019; Published 30 July 2019 Academic Editor: Jose I. Mayordomo Copyright © 2019 Dan Morgenstern-Kaplan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Cardiac angiosarcoma is a rare and clinically challenging pathology. It is a high-grade primary malignant tumor of the heart tissue that has many variants, of which the epithelioid variant is rarely present in the heart or the great vessels. As with many other cardiac tumors, it is mainly a diagnosis of exclusion and the initial diagnostic test is an echocardiogram followed by a biopsy with immunohistochemistry analysis to ascertain the type of tumor. The differential diagnosis of cardiac tumors is challenging due to the overlapping clinical manifestations with different cardiac tumors and systemic diseases. Cardiac angiosarcomas are often aggressive with a poor prognosis even with treatment. Herein, we present a case of the epithelioid variant of a cardiac angiosarcoma in addition to a thorough review of the recent literature on the clinical manifestation, diagnosis, and treatment of this type of tumors. 1. Introduction mented reports of this type of tumor [4, 5]. Herein, we pres- ent a case of a primary cardiac epithelioid angiosarcoma and a review of the literature. Cardiac tumors are rare, and, of these, metastatic tumors to the heart are nearly 30 times more common than primary cardiac 2. Case Report tumors. The estimated prevalence for primary cardiac tumors ranges from 0.0017% to 0.33% [1, 2]; of these, 90% are benign, A 20-year-old Hispanic male was referred for cardiologic eval- with myxomas being the most frequent. Of the 10% that are uation with the presence of thoracic oppressive pain and pro- malignant tumors, the most common are sarcomas, with gressively worsening dyspnea, orthopnea, dysphagia, and 8 kg undifferentiated pleomorphic sarcoma and angiosarcoma of weight loss in the last month. A chest computerized tomog- being the most prevalent histological subtypes, accounting for raphy (CT) scan was performed, reporting the presence of a 40% of all the malignant primary cardiac neoplasms [2]. The hyperdense mass within the pericardial space, with infiltration epithelioid variant is rarely present in cardiac tissue (less than into the myocardium, displacing and compressing adjacent 3% of all angiosarcomas are in the heart and/or great vessels), structures, with lymphadenopathy adjacent to the mass. In and it has been reported in many other tissues and organs addition to the mediastinal mass, several hyperdense masses (i.e., soft tissues and skin, especially in the extremities) [3]. were observed in the right lung (Figure 1). Afterward, trans- Primary cardiac epithelioid angiosarcoma is extremely thoracic echocardiography was performed, reporting pericar- rare; to our knowledge, there are only another seven docu- dial effusion with the presence of a mediastinal mass that 2 Case Reports in Oncological Medicine Figure 1: Chest CT scan with IV contrast showing a hyperdense mass within the pericardial space, with infiltration into the (a) myocardium, displacing and compressing adjacent structures. compromised all four cardiac cavities, infiltrating both atria and partially obstructing the right atrium (Figure 2). A biopsy was taken (Figure 3), and, macroscopically, the specimen was a 7×4 × 2cm lung parenchyma, with multiple, well-circumscribed solid brown nodular lesions surrounded by collapsed lung tissue. Histological analysis was consistent with a malignant neoplasia of mesenchymal origin with mostly polygonal cells and some spindle-shaped cells arranged around the blood vessels. The cells were pleomor- phic with vesiculated nuclei, prominent nucleoli, and a high atypical mitotic rate. The cells were surrounded by a net of capillaries with pleomorphic endothelial cells. In immuno- (b) histochemical analysis, neoplastic cells were intensely posi- tive for CD34 and CD31, positive for vimentin, and weakly Figure 2: Transthoracic echocardiography. (a) Parasternal short positive for angiotensin-converting enzyme (ACE), cytokera- axis view showing tumoral infiltration around the aortic root tin AE1/AE3 (CKAE1/AE3), and epithelial cell adhesion without obstruction of the outflow tract. (b) Apical 4-chamber molecule (BerEP4), with a Ki-67 of 50%. This pathological view showing myocardial infiltration to the interatrial septum and the free wall of the right atrium and ventricle. analysis was consistent with the diagnosis of metastatic mul- tifocal epithelioid angiosarcoma. After the pathological diagnosis was made, the patient was readmitted with symptoms of superior vena cava syn- Cardiac tumors are generally diagnosed after excluding drome. It was then decided to start with the first cycle of che- other more common pathologies. Echocardiography is the motherapy with a combination of ifosfamide, epirubicin, and diagnostic tool of choice for the initial evaluation of cardiac cisplatin. He did not respond to treatment, and the patient neoplasms and the hemodynamic and functional effects of with his family decided to forgo treatment. The patient was the tumor. The location of the tumor is also of diagnostic discharged to palliative care at home. use; for example, the most common location for cardiac myx- omas is the left atrium, while the most common location for an epithelioid angiosarcoma is the right atrium [8]. Trans- 3. Discussion esophageal echocardiography has 97% sensitivity in detecting Cardiac angiosarcomas are rare entities, and the diagnosis cardiac masses, and it can help identify its location, size, shape, attachment, and mobility, characteristics that might and treatment of any cardiac tumor are a challenge for clini- cians. Most benign cardiac tumors originate in the atria, provide some insights into the possible mass etiology [9]. more specifically in the left atrium, and cause different clini- Most patients with malignant neoplasms present with dis- cal presentations depending on the location of the tumor seminated disease at the time of diagnosis, with the lungs inside the heart, more than its histological subtype. They being the most frequent site of metastases; however, other can cause constitutional and systemic symptoms, obstructive metastasis sites include the liver, lymph nodes, bone, adrenal and restrictive cardiac symptoms, arrhythmias, and systemic glands, and spleen. Thus, for the staging of this type of malig- embolization [1, 6]. The most prevalent symptoms that nancies, other required studies include CT scan, magnetic patients with primary cardiac malignant tumors complain resonance imaging (MRI), echocardiography, and in some about are exertional dyspnea, chest pain, and palpitations cases, positron electron tomography (PET) scan [2]. In the [7]. Though these symptoms are not specific of cardiac cases of malignant tumors, imaging would show a mass infil- malignancies, in the context of systemic symptoms imaging trating into more than one cardiac chamber and into the studies of the thorax are recommended to assess the cardiac aorta in most cases; this causes a restriction of heart move- and thoracic anatomy. ment and is highly suggestive of malignancy. Case Reports in Oncological Medicine 3 (a) (b) (c) Figure 3: (a) Microphotography H&E (20x). In the center, we can observe a blood vessel surrounded by neoplastic cells in two different patterns: cells forming nests and spindle cells fusing with each other. (b) Microphotography H&E (40x). The tumoral cells are shown in detail, with a medium to large size, scant cytoplasm, oval nuclei of vesicular chromatin, prominent nucleolus, and several mitotic figures. (c) Immunochemistry of the CD31 marker: the neoplastic cells’ cytoplasm and membrane are positive, while the bronchial epithelium is negative. In the case of malignant cardiac tumors, the differen- When the diagnosis is uncertain through imaging stud- tial diagnosis includes primarily benign cardiac tumors, ies, biopsy determines the histological type and subtype of which can be suspected clinically with the classic triad of the tumor. Microscopic analysis of epithelioid angiosarcomas cardiac obstruction, embolism, and constitutional symp- includes complex interanastomosing vessels lined with epi- thelioid endothelial cells to solid sheets of atypical epithelioid toms. Other diagnoses include intracardiac thrombi, vege- tations and other infective masses, and calcified lesions. endothelial cells. Tumor cells are polygonal with prominent This can be differentiated by imaging studies previously nuclear atypia, intracytoplasmic vacuoles, and abundant described. Cardiac masses with intramural growth or infil- amphophilic cytoplasm with tumor necrosis often seen [3]. tration often produce conduction defects or arrhythmias All pathology analyses should include IHC, and a large that can sometimes be fatal; myocardial infiltration can panel of antibodies must be performed to avoid misdiagnosis also produce congestive heart failure secondary to systolic because a number of cell markers are shared by many types of and/or diastolic dysfunction. Other manifestations include tumors, such as pancytokeratins. Epithelioid angiosarcoma is positive for vascular markers such as CD31, CD34, and ERG myocardial ischemia or infarction secondary to infiltration and compression of coronary arteries. These are the signs transcription factor. It is also positive for cytokeratin (indic- that suggest malignancy. Pericardial effusion is reported in ative of epithelial cells and angiosarcoma), FLI-1, high Ki-67, both benign and malignant cardiac tumors, but hemor- p53 (positive in 20% of cases), and vimentin (a marker of rhagic effusion or tamponade is suggestive of malignancies mesenchymal cells) [3, 11]. as well [2]. IHC is also useful to determine the potential use of tar- The lung is a common site of metastasis from soft tis- geted therapy in oncology, and in these particular cases it sue sarcomas, both from the thorax and the extremities. would be useful to search for the presence of important gene Leiomyosarcoma (21%), high-grade pleomorphic sarcomas markers for current targeted therapy—KDR and FLT- (18%), synovial sarcomas (14%), and liposarcomas (12%) 4—which encode for VEGFR-2 and VEGFR-3, respectively. These markers are receptor tyrosine kinases present in angio- are the most common sarcomas that metastasize to the lung. Furthermore, all these tumors should be in the dif- sarcomas, and their presence has shown tumors to be sensi- ferential diagnosis of lesions like these in order to deter- tive or responsive to sorafenib, a tyrosine kinase inhibitor mine their origin and should be studied by pathologic used in a wide range of cancers [12]. analysis with different techniques such as optic micros- Cardiac angiosarcomas usually have a poor prognosis; they most frequently affect men aged 40 to 50 years, and copy, immunohistochemistry (IHC), or cytogenetics [10]. 4 Case Reports in Oncological Medicine Deutsches Äerzteblatt International, vol. 111, no. 12, pp. 205– the prognosis is worse in younger patients, such as the patient 211, 2014. in this case report. The mean survival rate of cardiac angio- sarcomas is 9 3±4 2 months without surgical treatment; in [7] L. Yu, T. Gu, E. Shi et al., “Primary malignant cardiac tumors,” Journal of Cancer Research and Clinical Oncology, vol. 140, some cases, where the extent of the disease and the anatom- no. 6, pp. 1047–1055, 2014. ical location allow a complete resection, surgery might be considered a treatment option [13]. On the other hand, che- [8] C. Liu, Y. Zhao, Z. Yin et al., “Right atrial epithelioid angiosar- coma with multiple pulmonary metastasis confirmed by multi- motherapy with doxorubicin and a combination of antineo- modality imaging-guided pulmonary biopsy,” Medicine, plastic drugs has been shown to increase mean survival vol. 97, no. 30, article e11588, 2018. rate, and radiation therapy is also an option that has been [9] S. D. Patel, A. Peterson, A. Bartczak et al., “Primary cardiac associated with a better survival rate. Pegylated-liposomal angiosarcoma—a review,” Medical Science Monitor, vol. 20, doxorubicin has shown promising results in other sarcomas, pp. 103–109, 2014. such as Kaposi’s sarcoma, but no randomized controlled [10] S. Suster and C. Moran, Diagnostic Pathology: Thoracic, Else- clinical trials have been conducted to assess efficacy and vier, 2012. safety [14]. Other chemotherapeutic drugs such as cisplatin, cyclophosphamide, dacarbazine, ifosfamide, mitomycin-C, [11] A. Saqi, R. B. Ramdall, and D. Hamele-Bena, “Metastatic epi- thelioid angiosarcoma of the heart in peritoneal fluid,” Diag- paclitaxel, and vincristine are commonly prescribed, but no nostic Cytopathology, vol. 30, no. 5, pp. 350–352, 2004. standardized treatment algorithms exist as of this moment, although good algorithm proposals have been made [15]. In [12] T. Guo, L. Zhang, N. E. Chang, S. Singer, R. G. Maki, and C. R. Antonescu, “Consistent MYC and FLT4 gene amplification in this patient, palliative care was decided in combination with radiation-induced angiosarcoma but not in other radiation- the medical team, the patient, and his family members. associated atypical vascular lesions,” Genes, Chromosomes & Though information related to the best therapeutic options Cancer, vol. 50, no. 1, pp. 25–33, 2011. and outcomes of this type of pathology is insufficient, as just [13] C. Lestuzzi, A. De Paoli, T. Baresic, G. Miolo, and a handful of patients with the diagnosis of primary cardiac A. Buonadonna, “Malignant cardiac tumors: diagnosis and epithelioid angiosarcoma has been reported, we hope that treatment,” Future Cardiology, vol. 11, no. 4, pp. 485–500, this article provides further insight into this pathology. [14] D. W. Northfelt, B. J. Dezube, J. A. Thommes et al., “Efficacy of 4. Conclusion pegylated-liposomal doxorubicin in the treatment of AIDS- related Kaposi’s sarcoma after failure of standard chemother- Cardiac epithelioid angiosarcomas are rare. Although echo- apy,” Journal of Clinical Oncology, vol. 15, no. 2, pp. 653– cardiography and CT scan might suggest the pretense of a 659, 1997. malignant cardiac neoplasm, immunohistochemical diagno- [15] I. A. Paraskevaidis, C. A. Michalakeas, C. H. Papadopoulos, sis is required to confirm the diagnosis. When cardiac epithe- and M. Anastasiou-Nana, “Cardiac tumors,” ISRN Oncology, lioid angiosarcomas are diagnosed, prognosis is poor. vol. 2011, 5 pages, 2011. Conflicts of Interest The authors declare that there are no conflicts of interest regarding the publication of this article. References [1] B. Hudzik, K. Miszalski-Jamka, J. Glowacki et al., “Malignant tumors of the heart,” Cancer Epidemiology, vol. 39, no. 5, pp. 665–672, 2015. [2] C. Basso, S. Rizzo, M. Valente, and G. 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Martens, “Cardiac tumors—Diagnosis and Surgical Treatment,” MEDIATORS of INFLAMMATION The Scientific Gastroenterology Journal of World Journal Research and Practice Diabetes Research Disease Markers Hindawi Hindawi Publishing Corporation Hindawi Hindawi Hindawi Hindawi www.hindawi.com Volume 2018 http://www www.hindawi.com .hindawi.com V Volume 2018 olume 2013 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 International Journal of Journal of Immunology Research Endocrinology Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 Submit your manuscripts at www.hindawi.com BioMed PPAR Research Research International Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 Journal of Obesity Evidence-Based Journal of Journal of Stem Cells Complementary and Ophthalmology International Alternative Medicine Oncology Hindawi Hindawi Hindawi Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2013 Parkinson’s Disease Computational and Behavioural Mathematical Methods AIDS Oxidative Medicine and in Medicine Neurology Research and Treatment Cellular Longevity Hindawi Hindawi Hindawi Hindawi Hindawi www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018 www.hindawi.com Volume 2018

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