Primary Cardiac Sarcoma Research Articles

Combined treatment for a large primary cardiac sarcoma: a case report

BackgroundUndifferentiated pleomorphic sarcoma (UPS) as a primary cardiac tumor is rare, with extremely poor prognosis owing to high recurrence and invasion. We encountered a patient who presented with a primary cardiac tumor incarcerating the mitral valve and who was in a shock state.Case presentationA 41-year-old man was transported emergently to our hospital owing to acute respiratory distress and hemoptysis. He was diagnosed with acute left heart failure caused by a large mass in the left atrium (LA) that obstructed cardiac blood flow, as revealed by imaging study findings, and he underwent an emergency open-heart surgery for tumor resection. He was pathologically diagnosed with UPS invading the muscle layer of the LA. However, after receiving combined therapy for local recurrence and distant metastasis, including proton beam radiotherapy and chemotherapy with molecularly targeted drugs, he could return to work for 2 more years after surgery.ConclusionIn this study, we reported the case of a patient who was in a state of shock state owing to the presence of UPS in the LA. The patient underwent an emergency surgery and received combined therapy. He survived for 2 more years after an initial diagnosis, without active local recurrence and distant metastasis.

The Heart of the Matter: A Unique Convergence of Cardiac Neoplasm, Hereditary Nonpolyposis Colorectal Cancer, and Spindle Cell Sarcoma

Primary cardiac tumours are exceedingly unusual and aggressive; they often develop in younger patients and present with advanced disease. The rarity and heterogeneity of primary cardiac tumours challenge the standardisation of therapeutic guidelines. Undifferentiated primary cardiac spindle cell sarcomas, a distinct subset of primary cardiac sarcomas, are especially unique with <20 cases reported worldwide, the majority of which are of left atrial origin. This article presents a review of the aetiology, pathophysiology, and therapy of undifferentiated primary cardiac spindle cell sarcomas. In conjunction, the authors present a unique case of a woman with hereditary nonpolyposis colorectal cancer (Lynch syndrome) who presented with a primary cardiac spindle cell sarcoma of left ventricular origin; this is the first case of this type and location of cardiac tumour reported in a patient with Lynch syndrome.

Multidisciplinary approach to rare primary cardiac sarcoma: a case report and review

BackgroundUndifferentiated pleomorphic sarcoma is a very rare and aggressive type of primary cardiac tumors. Most cardiac sarcomas result in rapid growth and quick death. According to different sources the median survival is typically 6 to 12 months. We are presenting a case of primary cardiac sarcoma with 26 months disease free survival following cytoreductive surgery and chemotherapy.Case presentationA 48-year-old woman with progressing symptoms of dyspnea and palpitations for over 2 months was referred to a cardiologist. With the help of echocardiography and cardiovascular magnetic resonance cardiac sarcoma was suspected. Open biopsy and cytoreductive surgery were performed, complete resection of the tumor was not possible. Histology revealed undifferentiated pleomorphic sarcoma. Seven cycles of chemotherapy with Doxorubicine and Ifosfamide were completed. Cardiovascular magnetic resonance revealed a complete response – only signs of fibrosis without any signs of tumor were visible. Follow ups with echocardiography, cardiovascular magnetic resonance and chest, abdomen and pelvic computed tomography is performed every 3 months. Twenty-six months from initial diagnosis the patient is still free of recurrence of tumor with no compromises of the quality of life.ConclusionStandard chemotherapy together with cytoreductive surgery can have a complete response effect in undifferentiated pleomorphic sarcoma with unusual long-term survival.

Primary Cardiac Sarcoma: A Rare, Aggressive Malignancy with a High Propensity for Brain Metastases

Introduction Primary cardiac sarcoma (PCS) has a poor prognosis compared to other sarcomas due to late presentation, challenging resection, incidence of metastases, and limited efficacy of systemic therapies. Methods A medical record search engine was queried to identify patients diagnosed with PCS from 1992 to 2017 at the University of Michigan. Results Thirty-nine patients with PCS had a median age of 41 years (range 2–77). Common histologies were angiosarcoma (AS, 14), high-grade undifferentiated pleomorphic sarcoma (UPS, 10), and leiomyosarcoma (LMS, 5). Sites of origin were left atrium (18), right atrium (16), and pericardium (5). AS was the most common right-sided tumor; UPS was more common on the left. Eighteen patients presented with metastases involving lung (10), bone (7), liver (5), and brain (4). Twenty-five patients underwent resection, achieving 3 R0 resections. Patients received a median of 2 (1–6) systemic therapies. Median overall survival (OS) was 12.1 months (range 0–79). Median OS was 14.0 months and 8.2 months in patients who did or did not undergo resection, respectively (p=0.018). Brain metastases occurred in 12 (31%) patients, 9 (75%) of whom had left heart tumors, at a median of 8.5 months (range 0–75) from diagnosis. Median OS was 5.6 months (range 0–30) after the diagnosis of brain metastases. Conclusions PCS portends a poor prognosis, because of difficulty in obtaining complete resection of sarcoma, advanced stage at diagnosis, and high risk of brain metastases. Providers should be aware of the increased risk of brain metastases and consider brain imaging at diagnosis and follow-up.

THE ‘BENIGN' PERICARDIAL EFFUSION: NOT ALWAYS WHAT IT SEEMS

Pericardial effusion is a clinical condition often due to pericarditis or malignancy. Primary cardiac sarcomas are extremely rare with a non-specific clinical presentation which may include effusion. A 45-year old woman presented to hospital with pleuritic chest pain after a viral illness.

Primary cardiac synovial sarcoma that was continuous with the mitral valve caused severe thrombocytopenia: a case report

BackgroundPrimary cardiac sarcomas are exceedingly rare, and they commonly result in nonspecific constitutional symptoms such as shortness of breath, weight loss, and anaemia-related fatigue and malaise. However, thrombocytopenia has very rarely been reported in association with cardiac tumours, either benign or malignant. We report one case of primary cardiac synovial sarcoma continuous with the mitral valve, which was accompanied by severe thrombocytopenia, and the platelet counts returned rapidly to a normal range early after tumour excision and without any special therapies.Case presentationA 52-year-old male diagnosed with atrial myxoma with severe thrombocytopenia was admitted to our hospital. Blood analysis showed severe thrombocytopenia, whereas erythrocyte and leucocyte counts were within the normal range. A 50 × 35 mm mobile mass continuous with the mitral valve was found to be present in the left atrium upon echocardiography. Bone marrow aspiration and related examinations excluded thrombocytopenia caused by haematologic malignancies. The patient received a platelet transfusion, but platelet counts decreased quickly. Glucocorticoid therapy and immunoglobulin transfusion were also used, but were ineffective. Although the operation risk was high, tumour resection was performed via a median sternotomy with a cardiopulmonary bypass system. The postoperative pathological diagnosis was biphasic cardiac synovial sarcoma. Surprisingly, the platelet counts returned rapidly to a normal range early after tumour excision without any special therapies. The disappearance of the tumour from the annular region was confirmed on transthoracic echocardiography 6 days after surgery, and an FDG-PET scan performed 8 days after surgery showed no abnormal accumulation. Unfortunately, the patient died suddenly 6 months later without unknown cause.ConclusionsWe report that a rare primary cardiac synovial sarcoma case continuous with the mitral valve caused severe thrombocytopenia; this provides further support for the awareness and diagnosis of primary cardiac synovial sarcoma. We also highlight that thrombocytopenia might be one rare symptom of a solid cardiac tumour but need more cases for support.

Primary cardiac sarcomas: A multi-national retrospective review.

BackgroundPrimary cardiac sarcoma (PCS) is a rare but often fatal disease. The current study aimed to analyze the impact of baseline demographics, local and systemic therapies in a contemporary cohort.MethodsClinical records of PCS across six institutions in three continents were reviewed. Kaplan‐Meier method was used to estimate survival. Cox proportional hazard model was used to determine variables impacting progression‐free survival (PFS) or overall survival (OS).ResultsSixty‐one patients with PCS (1996‐2016) were identified. The median age at diagnosis was 46 (range 18‐79); 36% (n = 22) presented with metastatic disease. The most common histology was angiosarcoma (n = 24, 39%). A total of 46 patients received surgery (75%) but only 5 (8%) patients achieved R0 resection. Multi‐modality treatment to the primary tumor was given to 28 patients (46%; localized disease 23/39 (59%); metastatic disease 5/22 (23%)). The median OS for the entire cohort was 17.5 months (95% CI 9.5‐20.6), with seven (11%) patients surviving longer than 36 months. On multi‐variate analysis, age <65 (P = 0.01) was the only significant favorable prognostic factor. For first‐line palliative chemotherapy, the median PFS was 4.4 months (95% CI 2.9‐7.7 months). The best response for first‐line chemotherapy was 32% (CR = 1, PR = 9). No significant improvement in OS was identified in patients presenting throughout the 20‐year period of this review.ConclusionYounger age at diagnosis was associated with improved outcome although the prognosis of PCS remains poor. Given the lack of improvement in survival, further dedicated research is required.

Treatment of cardiac synovial sarcoma: experience of two cases

BackgroundPrimary heart sarcomas are exceedingly rare tumors. Among primary cardiac sarcomas, synovial sarcoma is one of the rarest, involving cardiac cavities or pericardium.Case presentationTwo cases of synovial sarcoma are presented with the clinical course and therapy. Both cases were treated with surgery and chemo/radiotherapy. Interestingly, one of the patient, a 52-year-old male with an intracardiac synovial sarcoma, undergone a SynCardia total artificial heart implantation, but died for multiple pulmonary metastases waiting for transplantation.ConclusionComplete surgical resection of cardiac synovial sarcoma is the gold standard of therapy, though rarely possible. Although guidelines for the treatment are not well established, due to limited number of cases reported, chemotherapy and radiotherapy are frequently administered and seem to prolong mean patient’s survival. Cardiac transplantation could be considered in selected cases.

The impact of postoperative therapy on primary cardiac sarcoma

ObjectivePrimary cardiac sarcomas (PCS) are extremely rare, portend a very poor prognosis, and have limited outcomes data to direct management. This study evaluated the impact of postoperative chemotherapy and/or radiotherapy on survival for PCS. MethodsA retrospective chart review was conducted of 12 patients diagnosed with and who underwent resection for PCS at a single institution between 2000 and 2016. Data were collected on patient/tumor characteristics and analyzed with respect to treatment and outcome using Kaplan–Meier methods. ResultsMedian age was 43 (range 21-73 years) with a 50:50 male-to-female ratio. The most common subtype was angiosarcoma (42%), and 25% presented with distant metastases (DMs). The initial treatment modality for all patients was surgery, with 58% having macroscopically positive (R2) margins. In total, 75% received postoperative chemotherapy and/or radiotherapy. Median progression-free survival (PFS) was 5.9 months, and median overall survival (OS) was 12.0 months. Achieving negative or microscopically positive margins (R0/R1) as compared with R2 resection significantly improved PFS (12.6 vs 2.7 months, P = .008) and OS (21.8 vs 7.2 months, P = .006). DM at presentation demonstrated a significantly shorter OS (7.0 vs 16.9 months, P = .04) and PFS (0.7 vs 7.9 months, P = .003) compared with localized disease. Patients given postoperative therapy had longer OS compared with surgery only, but this difference was not statistically significant (15.5 vs 2.6 months, P = .12). ConclusionsGross total surgical resection can significantly improve PFS and OS in PCS, but DM at diagnosis is an extremely poor prognostic sign. Postoperative therapy should be considered, although this study was likely underpowered to demonstrate a statistically significant benefit.

Evaluation and Management of Cardiac Tumors.

Our purpose is to discuss the importance of multimodality imaging in the assessment of cardiac tumors and management. We have compiled a recent review of the scientific literature and embedded our clinical pathways and recommendations based on data and clinical experience. The use of contrast echocardiography in the assessment of cardiac masses has been shown to be helpful in distinguishing tumor from thrombus. Deformation imaging of cardiac tumors has been shown to differentiate better rhabdomyomas from fibromas in pediatric patients. Cardiac MRI (CMR) appears to be helpful in determining whether cardiac tumors are benign or malignant by identifying presence of infiltration, uptake of contrast in first pass perfusion and gadolinium enhancement. Patients with evidence of cardiac metastases by CMR show similar survival to stage IV cancer without cardiac metastases. In our institution, we use a standardized approach for the evaluation of cardiac masses, which includes multimodality imaging in the appropriate clinical context. The autotransplantation surgical technique has shown some promise in improving survival in patients with primary cardiac sarcomas. In our institution, we do not routinely recommend anticoagulation for "tumor-thrombus" in renal cell carcinoma due to risk of bleeding from primary tumor. Cardiac masses are often found incidentally, but sometimes can present with cardiovascular symptoms due to obstruction and valvular dysfunction, which may prompt imaging. It is important to determine whether the mass is a normal variant, imaging artifact, vegetation, thrombus, or tumor. Transthoracic echocardiography is ideally suited to be the initial imaging modality because of the portability, wide availability, lack of radiation, and relatively low cost. The gold standard cardiac imaging technique to distinguish tumor from thrombus is contrast enhanced CMR with prolonged inversion time. Advantages of CMR when compared to echocardiography regarding characterization of cardiac tumors are as follows: larger field of view, better spatial resolution, better tissue characterization, lack of attenuation, and ability to image at any prescribed plane. Primary and secondary cardiac tumors have particular characteristics in echocardiography and CMR. Imaging of cardiac tumors plays an important role in establishing a diagnosis and in planning management.

Clinicopathologic features and outcomes of primary cardiac tumors: a 16-year-experience with 212 patients at a Chinese medical center.

Primary cardiac tumors are uncommon, and the majority of them are benign which are curable but can cause significant morbidity if not diagnosed and treated in a timely fashion. The objective of this study was to review the clinicopathologic features and surgical outcomes of patients with primary cardiac tumors in a single medical center in China. We have retrospectively reviewed 212 consecutive adult patients who underwent surgical resection of primary cardiac tumors at our center from January of 2001 to June of 2017. All available clinicopathological features, imaging characteristics, and disease outcomes were summarized and presented. The present series enrolled 180 cardiac myxomas (84.9%) and 32 non-myxoma cases that included hemangioma, lipoma, papillary fibroelastoma, schwannoma, pericardial cyst, teratoma, paraganglioma, lymphoma, undifferentiated pleomorphic sarcoma, myxofibrosarcoma, angiosarcoma, and liposarcoma. All patients were diagnosed ante-mortem. The most frequent complaint was dyspnea. Benign tumors accounted for 93.9% of cases (199/212) and malignant tumors accounted for 5.7% (12/212), and the remaining one case was intermediate (paraganglioma). The outcome of benign tumors was favorable and only three recurrent cases were documented (1.5%) after surgical resection. All the seven patients with primary cardiac sarcomas (undifferentiated pleomorphic sarcoma, myxofibrosarcoma, angiosarcoma, and liposarcoma) suffered postoperative recurrence or disease related death. Cardiac myxoma represents the most frequent primary cardiac tumors. The clinical presentations, treatment strategies and outcomes of the primary cardiac tumors depend on the tumor location and histopathological type.

Primary epithelioid leiomyosarcoma of left atrium: Pathologist clinches it all

Primary cardiac neoplasms are rare with an estimated prevalence of 0.001%–0.2%. Cardiac tumors are mostly benign and myxomas are the commonest. Malignant tumors are rare in which metastasis and infiltration from the adjacent malignant tumor is more common than the primary ones. Amongst the primary malignant tumors sarcomas are the commonest. Primary epithelioid leiomyosarcoma of the heart is extremely rare. Here, we present a case of a 67-year-old female patient who was admitted with acute onset of pulmonary edema; on investigation, the patient was found to have left atrial mass. A provisional diagnosis of large left atrial myxoma with left ventricular inflow obstruction was made. Biopsy taken showed features of leiomyosarcoma which was confirmed by immunohistochemistry. Most of the patients with cardiac sarcoma have a high-grade tumor at the time of diagnosis with distant metastasis in the lungs (35.7%), lymph node (14.2%) followed by liver (7.14%) and they potent a poor prognosis. At present, only a few hundred primary cardiac sarcomas have been reported, most of which are based on autopsy series. Primary cardiac epithelioid leiomyosarcoma is a rare entity that can pose a diagnostic dilemma. However, atypical presentation of “atrial myxoma” being the common differential diagnosis. Histopathological evaluation is confirmatory to diagnose such a lesion. The pathologist should be aware of such an entity which can be attended by morphological evaluation including immunohistochemistry.

Primary cardiac sarcoma complicated with cerebral infarction and brain metastasis: A case report and literature review

In large autopsy series, the estimated frequency of primary tumors of the heart ranges from 0.0017% to 0.33%. Approximately 25% of primary cardiac tumors are malignant, and nearly 20% of these are sarcomas. To date, a completely feasible surgical resection remains the major treatment measure of cardiac sarcoma, especially for recurrent focal cardiac sarcoma and the recurrence of a restrictive metastasis. Althoughcharacteristically medicaltreatments are recommended, there is no consistent opinion for adjuvant radiotherapy and chemotherapy following an operation. Since these tumors usually undergo extensive spread by the time that the diagnosis is established, the prognosis of cardiac sarcoma remains poor. In this report, we described a case who underwent initial cardiac tumor resection, and was confirmed to be a pleomorphic undifferentiated sarcoma based on pathological findings. However, the patient complicated with cerebral infarction and subsequent brain metastasis sarcoma after the initial surgery, which was confirmed by brain tissue pathology. During the course of therapy, the patient underwent three surgical operations and refused to accept any chemotherapy and radiotherapy intervention. To the best of our knowledge, this is the first case report describing a primary cardiac sarcoma complicated with cerebral infarction and brain metastasis. The management of primary cardiac sarcoma is also discussed.

Cardiac Angiosarcoma: A Rare Cause of Recurrent Pericardial Effusion

SESSION TITLE: Cardiovascular Disease SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Cardiac angiosarcoma is a rare entity. Early diagnosis is delayed due to generalized symptoms. Diagnosis is made when specific symptoms related to tumor growth are investigated. CASE PRESENTATION: A 20 y/o female presented with pleuritic chest pain, treated as pericarditis. After one month, she developed fatigue and dyspnea. CT chest was remarkable for bilateral pleural effusions and pericardial effusion. She underwent pericardiocentesis. Pericardial bacterial and fungal evaluation was unrevealing. ANA, anti dsDNA, antihistone, complement levels, RF, HIV, acute hepatitis panel, RPR, coccidiomycosis and CMV titers, toxoplasma Ab, ANCA levels, coxsackie B, and IgM EBV were negative. She had a positive IgG EBV and her symptoms were attributed to viral perimyocarditis. She continued to have right-sided chest pain and was admitted to the hospital after three months. Her vital signs and physical examination were unremarkable. Cardiac MRI revealed a large mass, 4.1 x 6.3 x 4.8 cm, anterior to the right atrium and extending to the AV groove and proximal SVC, along with a moderate pericardial effusion. On perfusion images, there was gadolinium uptake at the anterior most part of the tumor with three hypoperfused areas portraying vascularity. Left heart catheterization showed extensive vascularization in the angiographic location of the right atrium where the tumor was located. She underwent tumor resection. The mass was extensive, beyond the right atrial free wall, on the right ventricle surface, pericardial metastasis and underneath an innominate vein on top of the aorta. Pathology displayed an aggressive high-grade angiosarcoma with a sheet-like growth pattern. Tumor cells had CD31 membranous and ERG nuclear staining. She was stable after surgery and further managed with paclitaxel. DISCUSSION: Over the years, the diagnosis of cardiac sarcomas has improved due to a combination of physical examination, ekg, cardiac catheterization, echocardiography, CT scan and MRI. It mostly involves the right atrium, presenting as pericardial effusion and/or cardiac tamponade in about 56% of cases. Complete or partial surgical resection is done for palliation or complete cure. Angiosarcomas are mostly resistant to chemotherapy and radiotherapy. However, recently paclitaxel and pegylated-liposomal doxorubicin have shown efficacy in treating angiosarcoma. CONCLUSIONS: Overall, even with the new imaging techniques for diagnosis and management, cardiac sarcomas still carry a poor prognosis with a 5-year survival rate of 14%. Reference #1: Blackmon, Shandra H., and Michael J. Reardon. “Surgical Treatment of Primary Cardiac Sarcomas.” Texas Heart Institute Journal 36.5 (2009): 451-452. Reference #2: Loukas, Marios. “Primary Cardiac Angiosarcoma - A Review”. Medical Science Monitor 20 (2014): 103-109. Reference #3: Skubitz, Keith M., and Philip A. Haddad. “Paclitaxel And Pegylated-Liposomal Doxorubicin Are Both Active In Angiosarcoma”. Cancer 104.2 (2005): 361-366. DISCLOSURE: The following authors have nothing to disclose: Sidra Khalid, Richard Meredith, Allan Klein No Product/Research Disclosure Information

1490P - Primary cardiac sarcoma (PCS): A multi-national retrospective review of clinical experience

1490P - Primary cardiac sarcoma (PCS): A multi-national retrospective review of clinical experience

1491P - Primary cardiac sarcoma: A retrospective study in two Korean tertiary centers

1491P - Primary cardiac sarcoma: A retrospective study in two Korean tertiary centers

Primary cardiac intimal sarcoma masquerading as mitral stenosis.

Key Clinical MessageIntimal sarcomas are a very rare medical entity and usually arise from the pulmonary artery or the thoracic aorta. Sarcomas should be considered in the differential diagnosis in patients with suspected mitral valve disease. Echocardiography should be performed as early as possible to establish a prompt diagnosis and management.

Primary cardiac leiomyosarcoma presenting as haemoptysis in a 22-year-old patient: an unusual presentation of a rare condition

Primary cardiac sarcomas are rare malignant tumours and among them, leiomyosarcoma is extremely rare. They are especially rare in a young age group. We present a case of a 22-year-old...

Postoperative Complications and Long-Term Results after Primary Cardiac Sarcoma Resection.

Primary malignant cardiac tumors rarely occur in clinical care. Little is known about the impact of a parameter on postoperative survival. From May 1991 to May 2014, a total of 24 patients underwent surgical treatment of a primary cardiac sarcoma in our center. We analyzed our clinical database retrospectively for information on patient characteristics and treatment data. The follow-up could be completed to 91.7%. Angiosarcoma and non-otherwise-specified sarcoma were the most common tumor entities. R0 resection was achieved in most cases. Postoperative mortality within the first 30 days was 20.8% (n = 5). In four of these five cases, postoperative low-output cardiac failure was the leading cause of death. The cumulative survival rate was 77.3% after 30 days, 68.2% after 3 months, 50.0% after 6 months, 45.0% after 12 months, and 18.0% after 24 months. The mean survival time in the whole group was 47.0 months. A low tumor differentiation was associated with low mean survival, but this was not statistically significant. Mean survival of sarcoma was higher after R0 resection. There was no significant rate of survival difference regarding the adjuvant therapy concept. Extended surgery alone or in combination with chemo- and/or radiotherapy may be successful in certain cases and may offer a satisfactory quality of life. The establishment of a multicenter heart tumor register in Germany is necessary to increase the number of cases in studies, get more remarkable study results, and standardize the diagnosis and therapy.

Improved Outcomes With the Evolution of a Neoadjuvant Chemotherapy Approach to Right Heart Sarcoma

Right-side heart sarcomas tend to be bulky, infiltrative, and difficult to treat. We have previously examined our outcomes with right heart sarcomas. Surgical resection with R0 margins showed better survival than positive margins but in only one third of cases could R0 status be achieved. The hypothesis for this study was that preoperative neoadjuvant chemotherapy would shrink the tumor margins and allow an increase in R0 resection, and hence, better survival. Review of our cardiac tumor database from 1990 to 2015 yielded 133 primary cardiac sarcoma cases. Of these, we identified 44 patients with primary right-side heart sarcomas. Prospective database and retrospective data collection and clinical outcomes were evaluated for all 44 patients. Primary outcomes included 30-day mortality and morbidity and long-term survival. We used univariate and multivariate analyses to identify independent predictors of overall survival. There were 27 male and 17 female patients with a mean age of 41 ± 12.7 years (range, 15 to 67). Seventy-three percent of the patients (32 of 44) received neoadjuvant chemotherapy. The most common tumor histology was angiosarcoma in 30 of 44 (68%). Thirty-day mortality was 4.5%, and statistically similar between the two groups. The median survival of patients who had R0 resection was 53.5 months compared with 9.5 months for R1. Neoadjuvant chemotherapy led to a doubling of survival (20 versus 9.5 months). Neoadjuvant chemotherapy followed by radical surgery is a safe and effective strategy in patients with primary right-side heart sarcoma. This multimodality treatment enhances resectability (R0 resection) that translates into improved patient survival.