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Right ventricular undifferentiated pleomorphic sarcoma: A case report
Journal of Cardiology Cases 13 (2016) 60–62
Contents lists available at ScienceDirect
Journal of Cardiology Cases journal homepage: www.elsevier.com/locate/jccase
Case Report
Right ventricular undifferentiated pleomorphic sarcoma: A case report Jared P. Beller (MD)a, Scott Maddalo (MD)a, Ronaldo Zamuco (MD)b, Leon Axel (MD, PhD)c, Abe DeAnda (MD)a, Leora B. Balsam (MD)a,* a
Department of Cardiothoracic Surgery, New York University School of Medicine, New York, NY, USA Department of Pathology, New York University School of Medicine, New York, NY, USA c Department of Radiology, New York University School of Medicine, New York, NY, USA b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 12 June 2015 Received in revised form 16 September 2015 Accepted 20 October 2015
Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, is a neoplasm that occurs most often in the extremities, trunk, and retroperitoneum. Rarely, UPS can occur in the cardiac chambers and great vessels. The diagnosis of UPS is difficult to establish with noninvasive imaging techniques, and these tumors may be mistaken for benign neoplasms preoperatively. Surgical excision is the standard therapy, although the extent and location of the tumor may limit the ability to perform a complete resection. Adjuvant chemotherapy and/or radiation are often used for incomplete resections. We report the case of a 57-year-old woman with a large right ventricular UPS who presented with signs of right-sided heart failure. Preoperative imaging was suggestive of a myxoma; however, histopathologic evaluation of the specimen confirmed a diagnosis of UPS. Microscopic margins of the specimen were positive, and adjuvant chemotherapy was given. We discuss diagnostic and treatment considerations for this unusual cardiac tumor. ß 2015 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
Keywords: Cardiac tumor Sarcoma Right ventricle
Introduction Primary cardiac tumors are rare, with an autopsy incidence of 0.001–0.030% [1]. Of these, approximately 25% are malignant, with sarcomas comprising the majority of this group [2]. Distribution among the cardiac chambers varies, but those arising from the atria are more common. We report the case of a 57-year-old woman with a large right ventricular undifferentiated pleomorphic sarcoma that was thought to be a myxoma on preoperative imaging. Case report A 57-year-old woman presented with several weeks of dyspnea, lower extremity edema, fatigue, and influenza-like symptoms. Transaminases were mildly elevated (aspartate transaminase
* Corresponding author at: NYU-Langone Medical Center, Suite 9-V, 530 First Avenue, New York, NY 10016, USA. Tel.: +1 212 263 7835; fax: +1 212 263 2042. E-mail address: [email protected] (L.B. Balsam).
50 U/L, alanine transaminase 46 U/L) and B-type natriuretic peptide level was 219.2 pg/mL (normal range 0–100 pg/mL). Transthoracic echocardiography demonstrated a mass filling the right ventricular cavity (Supplementary Fig. 1). Cardiac magnetic resonance imaging (MRI) showed a 4.9 cm 3.2 cm 5.5 cm lobulated, somewhat mobile, heterogenous and moderately enhancing mass that obstructed tricuspid inflow (Fig. 1). There was a broad attachment to the basal inferior right ventricular wall without evidence of invasion. The mass appeared to be most consistent with myxoma. Chest and abdominal computed tomography failed to show evidence of malignancy elsewhere. Coronary angiography showed nonobstructive coronary disease and a tumor blush from distal branches of the right coronary artery. Surgical excision was performed via median sternotomy with cardiopulmonary bypass and cardioplegic arrest. The right ventricular mass was excised through a right atriotomy; surgical margins of excision included part of the tricuspid subvalvular apparatus and partial-thickness resection of the right ventricular wall (Fig. 2). The tricuspid valve was replaced with a 31 mm bioprosthesis.
http://dx.doi.org/10.1016/j.jccase.2015.10.008 1878-5409/ß 2015 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
J.P. Beller et al. / Journal of Cardiology Cases 13 (2016) 60–62
Fig. 1.
Cardiac magnetic resonance imaging demonstrates right ventricular mass (labeled * in images). (A and B) Two-chamber view at end-diastole and end-systole, respectively. The right ventricular mass fills the cavity. It has a broad attachment to the inferior wall of the ventricle without gross invasion. There is no attachment to the proximal right ventricular outflow tract. The mass pushes against the tricuspid valve in systole. (C) Two-chamber first-pass perfusion image demonstrates an enhancing mass adjacent to inferior wall. (D) Four-chamber late gadolinium enhancement image shows enhancing mass. There is lack of involvement of septum and lateral wall.
Histologic examination of the tumor showed spindle cells arranged haphazardly in a variable collagenous stroma (Fig. 3) with negative stains for cytokeratin AE1/AE3, epithelial membrane antigen (EMA), myogenin, S100, and desmin, CD31, and smooth muscle actin. There were pleomorphic bizarre cells and no areas of myxoid change; a vague storiform area was noted. The proliferation index (Ki-67) was 60%. A diagnosis of high-grade undifferentiated pleomorphic sarcoma was made. The microscopic margins of the specimen were positive. The patient’s postoperative hospital course was uneventful and she was discharged home on postoperative day six. She was treated with adjuvant chemotherapy (doxorubicin, ifosfamide, and mesna) as an outpatient. Doxorubicin 75 mg/m2 was given in divided doses over 3 days and then repeated every three weeks. Ifosfamide was given 10 g/m2 in divided doses over four days and then repeated every three weeks. Mesna 500 mg/m2 was given prior to each ifosfamide dose and then 4 and 8 h after each dose. The patient completed 2 cycles of chemotherapy but due to intolerance of side effects, she declined further cycles. The patient is alive at 14 months without evidence of disease on surveillance imaging. Discussion The term undifferentiated pleomorphic sarcoma (UPS) is used interchangeably with malignant fibrous histiocytoma (MFH) to describe tumors with no definable line of cellular differentiation and microscopic features of fibroblasts and/or myofibroblasts
Fig. 2.
61
[3]. The existence of MFH as a distinct entity has been called into question, and UPS has largely supplanted MFH in the classification of these high-grade tumors. Pathologically there is a lack of consistent immunoreactivity, so the role of immunohistochemistry is in the exclusion of other subtypes of pleomorphic tumors. Similarly, cytogenetic and molecular genetic aberrations are generally nonspecific. UPS most commonly affects the extremities, followed by the trunk and retroperitoneum. Although transthoracic and transesophageal echocardiography are often employed in screening for cardiac masses, their limited ability to characterize tissue type and distinguish tumors restricts their diagnostic application. Cardiac MRI can provide more information regarding the nature of cardiac masses, although no single imaging feature is pathognomic for malignancy [4,5]. Dynamic gadolinium enhancement suggests a highly vascularized malignancy such as angiosarcoma. Other non-invasive imaging features that are suggestive of malignancy include ill-defined margins, size > 5 cm, tissue heterogeneity, broad attachment site, and right-sided tumors. In conjunction with cross-sectional imaging, 2-deoxy-2[F18]fluoro-D-glucose positron emission tomography (18FDG-PET) can be utilized to improve preoperative diagnosis of primary cardiac tumors [6]. In 24 consecutive patients, maximum standardized uptake values (SUV) were significantly higher in patients with primary malignant lesions versus benign lesions (8.0 2.1 vs. 2.8 0.6). Further, with selection of a high maximum SUV threshold of 3.5 (due to increased background myocardial uptake) a sensitivity of 100% and specificity of 86% was achieved.
Operative images of right ventricular mass. (A) The mass is approached through the right atrium and delivered through the tricuspid valve. (B) The broad attachment site to the inferior wall of the right ventricle is labeled with black arrows. (C) Excised right ventricular mass M, mass.
62
Fig. 3.
J.P. Beller et al. / Journal of Cardiology Cases 13 (2016) 60–62
Histologic characteristics of right ventricular high grade undifferentiated pleomorphic sarcoma. (A) Spindle cells are arranged haphazardly in a variably collagenized stroma (hematoxylin and eosin stain, 100). (B) Hyperchromatic, multinucleated, and bizarre cells with background patchy inflammation (hematoxylin and eosin stain, 400).
Finally, whole body PET imaging has the ability to detect metastatic disease, which may alter initial management decisions. UPS of the right ventricle is a particularly rare entity, with only a dozen cases reported in the literature. As with other cardiac sarcomas, prognosis is worse than extra-cardiac tumors with similar features. The treatment of choice, whenever possible, is complete resection, and prognosis is significantly affected by completeness of resection. Li et al. recently reported outcomes following surgical resection of primary cardiac sarcomas [7]. The median survival was 58 months following complete resection versus 11 months following incomplete resection. Moreover, the median local recurrence-free survival in this cohort was 36 months versus 6 months for complete and incomplete resections, respectively. Complete resection is difficult to achieve for cardiac sarcomas, highlighted by a 13% complete resection rate in the largest contemporary series [2]. Poor rates of complete resection have been partially attributed to preoperative misdiagnoses. The role of adjuvant radiation and/or chemotherapy for primary cardiac sarcomas is not well established, and there is currently no consensus on the ideal timing and duration of therapy. A large meta-analysis of adjuvant chemotherapy in resectable extremity soft tissue sarcomas demonstrated improved overall survival and freedom from local and distant recurrences (Sarcoma Metaanalysis Collaboration), yet these findings were contradicted by a recent randomized controlled study (EORTC 62931) of high-dose chemotherapy in a similar cohort [8,9]. A recent retrospective series spanning 25 years at the Cleveland Clinic found a survival advantage for patients with primary cardiac sarcoma that received multimodality treatment (any combination of surgery, radiation therapy, and chemotherapy) versus those treated with surgery, radiation therapy, or chemotherapy alone [10]. In cases where complete resection is not possible, cardiac transplantation has been utilized with variable results. In one series of 124 patients, five underwent heart transplantation when a conservative surgical approach yielded marginal resection, with a median survival of 27.5 months [2]. Overall, enthusiasm for heart transplantation is tempered by the scarcity of donor organs and the unknown interplay between tumor growth and post-transplant immunomodulatory therapy. More importantly, many patients are
not candidates for heart transplantation due to medical and psychosocial exclusions, and therefore standard surgical excision with or without adjuvant chemotherapy remains the standard treatment for this disease. Conflict of interests The authors have no relevant conflict of interest to declare. Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.jccase.2015.10.008. References [1] Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: diagnosis and management. Lancet Oncol 2005;6:219–28. [2] Isambert N, Ray-Coquard I, Italiano A, Rios M, Kerbrat P, Gauthier M, Blouet A, Chaigneau L, Duffaud F, Piperno-Neumann S, Kurtz JE, Girard N, Collard O, Bompas E, Penel N, et al. Primary cardiac sarcomas: a retrospective study of the French Sarcoma Group. Eur J Cancer 2014;50:128–36. [3] Goldblum JR. An approach to pleomorphic sarcomas: can we subclassify, and does it matter. Mod Pathol 2014;27:S39–46. [4] Sparrow PJ, Kurian JB, Jones TR, Sivananthan MU. MR imaging of cardiac tumors. Radiographics 2005;25:1255–76. [5] Hoey ET, Mankad K, Puppala S, Gopalan D, Sivananthan MU. MRI and CT appearances of cardiac tumours in adults. Clin Radiol 2009;64:1214–30. [6] Rahbar K, Seifarth H, Schafers M, Stegger L, Hoffmeier A, Spieker T, Tiemann K, Maintz D, Scheld HH, Schober O, Weckesser M. Differentiation of malignant and benign cardiac tumors using 18F-FDG PET/CT. J Nucl Med 2012;53:856–63. [7] Li H, Xu D, Chen Z, Ding W, Hong T, Chen H, Shao M, Lai H, Hou Y, Wang C. Prognostic analysis for survival after resections of localized primary cardiac sarcomas: a single-institution experience. Ann Thorac Surg 2014;97:1379–85. [8] Sarcoma Meta-analysis Collaboration. Adjuvant chemotherapy for localized resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Lancet 1997;350:1647–54. [9] Woll PJ, Reichardt P, Le Cesne A, Bonyalot S, Azzarelli A, Hoekstra HJ, Leahy M, Van Coevorden F, Verweij J, Hogendoorn PC, Ouali M, Marreaud S, Branwell, Hohenberger P. Adjuvant chemotherapy with doxorubicin, ifosfamide and lenograstim for resected soft tissue sarcoma (EORTC 62931): a multicenter randomized controlled trial. Lancet Oncol 2012;12:1045–54. [10] Randhawa JS, Budd GT, Randhawa M, Ahluwalia M, Jia X, Daw H, Spiro T, Haddad A. Primary cardiac sarcoma: 25-year Cleveland clinic experience. Am J Clin Oncol 2014. July 17 [Epub ahead of print].
Contents lists available at ScienceDirect
Journal of Cardiology Cases journal homepage: www.elsevier.com/locate/jccase
Case Report
Right ventricular undifferentiated pleomorphic sarcoma: A case report Jared P. Beller (MD)a, Scott Maddalo (MD)a, Ronaldo Zamuco (MD)b, Leon Axel (MD, PhD)c, Abe DeAnda (MD)a, Leora B. Balsam (MD)a,* a
Department of Cardiothoracic Surgery, New York University School of Medicine, New York, NY, USA Department of Pathology, New York University School of Medicine, New York, NY, USA c Department of Radiology, New York University School of Medicine, New York, NY, USA b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 12 June 2015 Received in revised form 16 September 2015 Accepted 20 October 2015
Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, is a neoplasm that occurs most often in the extremities, trunk, and retroperitoneum. Rarely, UPS can occur in the cardiac chambers and great vessels. The diagnosis of UPS is difficult to establish with noninvasive imaging techniques, and these tumors may be mistaken for benign neoplasms preoperatively. Surgical excision is the standard therapy, although the extent and location of the tumor may limit the ability to perform a complete resection. Adjuvant chemotherapy and/or radiation are often used for incomplete resections. We report the case of a 57-year-old woman with a large right ventricular UPS who presented with signs of right-sided heart failure. Preoperative imaging was suggestive of a myxoma; however, histopathologic evaluation of the specimen confirmed a diagnosis of UPS. Microscopic margins of the specimen were positive, and adjuvant chemotherapy was given. We discuss diagnostic and treatment considerations for this unusual cardiac tumor.
Keywords: Cardiac tumor Sarcoma Right ventricle
Introduction Primary cardiac tumors are rare, with an autopsy incidence of 0.001–0.030% [1]. Of these, approximately 25% are malignant, with sarcomas comprising the majority of this group [2]. Distribution among the cardiac chambers varies, but those arising from the atria are more common. We report the case of a 57-year-old woman with a large right ventricular undifferentiated pleomorphic sarcoma that was thought to be a myxoma on preoperative imaging. Case report A 57-year-old woman presented with several weeks of dyspnea, lower extremity edema, fatigue, and influenza-like symptoms. Transaminases were mildly elevated (aspartate transaminase
* Corresponding author at: NYU-Langone Medical Center, Suite 9-V, 530 First Avenue, New York, NY 10016, USA. Tel.: +1 212 263 7835; fax: +1 212 263 2042. E-mail address: [email protected] (L.B. Balsam).
50 U/L, alanine transaminase 46 U/L) and B-type natriuretic peptide level was 219.2 pg/mL (normal range 0–100 pg/mL). Transthoracic echocardiography demonstrated a mass filling the right ventricular cavity (Supplementary Fig. 1). Cardiac magnetic resonance imaging (MRI) showed a 4.9 cm 3.2 cm 5.5 cm lobulated, somewhat mobile, heterogenous and moderately enhancing mass that obstructed tricuspid inflow (Fig. 1). There was a broad attachment to the basal inferior right ventricular wall without evidence of invasion. The mass appeared to be most consistent with myxoma. Chest and abdominal computed tomography failed to show evidence of malignancy elsewhere. Coronary angiography showed nonobstructive coronary disease and a tumor blush from distal branches of the right coronary artery. Surgical excision was performed via median sternotomy with cardiopulmonary bypass and cardioplegic arrest. The right ventricular mass was excised through a right atriotomy; surgical margins of excision included part of the tricuspid subvalvular apparatus and partial-thickness resection of the right ventricular wall (Fig. 2). The tricuspid valve was replaced with a 31 mm bioprosthesis.
http://dx.doi.org/10.1016/j.jccase.2015.10.008 1878-5409/ß 2015 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
J.P. Beller et al. / Journal of Cardiology Cases 13 (2016) 60–62
Fig. 1.
Cardiac magnetic resonance imaging demonstrates right ventricular mass (labeled * in images). (A and B) Two-chamber view at end-diastole and end-systole, respectively. The right ventricular mass fills the cavity. It has a broad attachment to the inferior wall of the ventricle without gross invasion. There is no attachment to the proximal right ventricular outflow tract. The mass pushes against the tricuspid valve in systole. (C) Two-chamber first-pass perfusion image demonstrates an enhancing mass adjacent to inferior wall. (D) Four-chamber late gadolinium enhancement image shows enhancing mass. There is lack of involvement of septum and lateral wall.
Histologic examination of the tumor showed spindle cells arranged haphazardly in a variable collagenous stroma (Fig. 3) with negative stains for cytokeratin AE1/AE3, epithelial membrane antigen (EMA), myogenin, S100, and desmin, CD31, and smooth muscle actin. There were pleomorphic bizarre cells and no areas of myxoid change; a vague storiform area was noted. The proliferation index (Ki-67) was 60%. A diagnosis of high-grade undifferentiated pleomorphic sarcoma was made. The microscopic margins of the specimen were positive. The patient’s postoperative hospital course was uneventful and she was discharged home on postoperative day six. She was treated with adjuvant chemotherapy (doxorubicin, ifosfamide, and mesna) as an outpatient. Doxorubicin 75 mg/m2 was given in divided doses over 3 days and then repeated every three weeks. Ifosfamide was given 10 g/m2 in divided doses over four days and then repeated every three weeks. Mesna 500 mg/m2 was given prior to each ifosfamide dose and then 4 and 8 h after each dose. The patient completed 2 cycles of chemotherapy but due to intolerance of side effects, she declined further cycles. The patient is alive at 14 months without evidence of disease on surveillance imaging. Discussion The term undifferentiated pleomorphic sarcoma (UPS) is used interchangeably with malignant fibrous histiocytoma (MFH) to describe tumors with no definable line of cellular differentiation and microscopic features of fibroblasts and/or myofibroblasts
Fig. 2.
61
[3]. The existence of MFH as a distinct entity has been called into question, and UPS has largely supplanted MFH in the classification of these high-grade tumors. Pathologically there is a lack of consistent immunoreactivity, so the role of immunohistochemistry is in the exclusion of other subtypes of pleomorphic tumors. Similarly, cytogenetic and molecular genetic aberrations are generally nonspecific. UPS most commonly affects the extremities, followed by the trunk and retroperitoneum. Although transthoracic and transesophageal echocardiography are often employed in screening for cardiac masses, their limited ability to characterize tissue type and distinguish tumors restricts their diagnostic application. Cardiac MRI can provide more information regarding the nature of cardiac masses, although no single imaging feature is pathognomic for malignancy [4,5]. Dynamic gadolinium enhancement suggests a highly vascularized malignancy such as angiosarcoma. Other non-invasive imaging features that are suggestive of malignancy include ill-defined margins, size > 5 cm, tissue heterogeneity, broad attachment site, and right-sided tumors. In conjunction with cross-sectional imaging, 2-deoxy-2[F18]fluoro-D-glucose positron emission tomography (18FDG-PET) can be utilized to improve preoperative diagnosis of primary cardiac tumors [6]. In 24 consecutive patients, maximum standardized uptake values (SUV) were significantly higher in patients with primary malignant lesions versus benign lesions (8.0 2.1 vs. 2.8 0.6). Further, with selection of a high maximum SUV threshold of 3.5 (due to increased background myocardial uptake) a sensitivity of 100% and specificity of 86% was achieved.
Operative images of right ventricular mass. (A) The mass is approached through the right atrium and delivered through the tricuspid valve. (B) The broad attachment site to the inferior wall of the right ventricle is labeled with black arrows. (C) Excised right ventricular mass M, mass.
62
Fig. 3.
J.P. Beller et al. / Journal of Cardiology Cases 13 (2016) 60–62
Histologic characteristics of right ventricular high grade undifferentiated pleomorphic sarcoma. (A) Spindle cells are arranged haphazardly in a variably collagenized stroma (hematoxylin and eosin stain, 100). (B) Hyperchromatic, multinucleated, and bizarre cells with background patchy inflammation (hematoxylin and eosin stain, 400).
Finally, whole body PET imaging has the ability to detect metastatic disease, which may alter initial management decisions. UPS of the right ventricle is a particularly rare entity, with only a dozen cases reported in the literature. As with other cardiac sarcomas, prognosis is worse than extra-cardiac tumors with similar features. The treatment of choice, whenever possible, is complete resection, and prognosis is significantly affected by completeness of resection. Li et al. recently reported outcomes following surgical resection of primary cardiac sarcomas [7]. The median survival was 58 months following complete resection versus 11 months following incomplete resection. Moreover, the median local recurrence-free survival in this cohort was 36 months versus 6 months for complete and incomplete resections, respectively. Complete resection is difficult to achieve for cardiac sarcomas, highlighted by a 13% complete resection rate in the largest contemporary series [2]. Poor rates of complete resection have been partially attributed to preoperative misdiagnoses. The role of adjuvant radiation and/or chemotherapy for primary cardiac sarcomas is not well established, and there is currently no consensus on the ideal timing and duration of therapy. A large meta-analysis of adjuvant chemotherapy in resectable extremity soft tissue sarcomas demonstrated improved overall survival and freedom from local and distant recurrences (Sarcoma Metaanalysis Collaboration), yet these findings were contradicted by a recent randomized controlled study (EORTC 62931) of high-dose chemotherapy in a similar cohort [8,9]. A recent retrospective series spanning 25 years at the Cleveland Clinic found a survival advantage for patients with primary cardiac sarcoma that received multimodality treatment (any combination of surgery, radiation therapy, and chemotherapy) versus those treated with surgery, radiation therapy, or chemotherapy alone [10]. In cases where complete resection is not possible, cardiac transplantation has been utilized with variable results. In one series of 124 patients, five underwent heart transplantation when a conservative surgical approach yielded marginal resection, with a median survival of 27.5 months [2]. Overall, enthusiasm for heart transplantation is tempered by the scarcity of donor organs and the unknown interplay between tumor growth and post-transplant immunomodulatory therapy. More importantly, many patients are
not candidates for heart transplantation due to medical and psychosocial exclusions, and therefore standard surgical excision with or without adjuvant chemotherapy remains the standard treatment for this disease. Conflict of interests The authors have no relevant conflict of interest to declare. Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.jccase.2015.10.008. References [1] Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: diagnosis and management. Lancet Oncol 2005;6:219–28. [2] Isambert N, Ray-Coquard I, Italiano A, Rios M, Kerbrat P, Gauthier M, Blouet A, Chaigneau L, Duffaud F, Piperno-Neumann S, Kurtz JE, Girard N, Collard O, Bompas E, Penel N, et al. Primary cardiac sarcomas: a retrospective study of the French Sarcoma Group. Eur J Cancer 2014;50:128–36. [3] Goldblum JR. An approach to pleomorphic sarcomas: can we subclassify, and does it matter. Mod Pathol 2014;27:S39–46. [4] Sparrow PJ, Kurian JB, Jones TR, Sivananthan MU. MR imaging of cardiac tumors. Radiographics 2005;25:1255–76. [5] Hoey ET, Mankad K, Puppala S, Gopalan D, Sivananthan MU. MRI and CT appearances of cardiac tumours in adults. Clin Radiol 2009;64:1214–30. [6] Rahbar K, Seifarth H, Schafers M, Stegger L, Hoffmeier A, Spieker T, Tiemann K, Maintz D, Scheld HH, Schober O, Weckesser M. Differentiation of malignant and benign cardiac tumors using 18F-FDG PET/CT. J Nucl Med 2012;53:856–63. [7] Li H, Xu D, Chen Z, Ding W, Hong T, Chen H, Shao M, Lai H, Hou Y, Wang C. Prognostic analysis for survival after resections of localized primary cardiac sarcomas: a single-institution experience. Ann Thorac Surg 2014;97:1379–85. [8] Sarcoma Meta-analysis Collaboration. Adjuvant chemotherapy for localized resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Lancet 1997;350:1647–54. [9] Woll PJ, Reichardt P, Le Cesne A, Bonyalot S, Azzarelli A, Hoekstra HJ, Leahy M, Van Coevorden F, Verweij J, Hogendoorn PC, Ouali M, Marreaud S, Branwell, Hohenberger P. Adjuvant chemotherapy with doxorubicin, ifosfamide and lenograstim for resected soft tissue sarcoma (EORTC 62931): a multicenter randomized controlled trial. Lancet Oncol 2012;12:1045–54. [10] Randhawa JS, Budd GT, Randhawa M, Ahluwalia M, Jia X, Daw H, Spiro T, Haddad A. Primary cardiac sarcoma: 25-year Cleveland clinic experience. Am J Clin Oncol 2014. July 17 [Epub ahead of print].