Unusual Case of a Primary Cardiac Tumor Presenting as Superior Vena Cava Syndrome

Abstract

SESSION TITLE: Student/Resident Case Report Poster - Cardiac and Thoracic Surgery SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Primary cardiac tumors are extremely rare. Incidence is approximately 0.017% - 0.019% of all primary tumors. Spindle Cell Sarcoma is the rarest, with only 4 cases reported. Sarcomas, have a poor prognosis even after surgical intervention with an average mean survival of 6 months. CASE PRESENTATION: 67 year old female patient with PMH of Hypertension, Dyslipidemia and DM that presented to the emergency department with complaints of swelling and redness of the face for one year that was treated as an allergic reaction without improvement. Presentation was significant for shortness of breath, dry cough, bilateral jugular vein distention, low voltage on EKG and a opacity measuring 3.8 x 3.7cm at the right hilar region on CXR. Chest CT confirmed a well-defined mass measuring 7 x 5.7cm in the right atrium producing extrinsic compression of the right atrioventricular valve and proximal aspect of the right ventricle. The mass infiltrated to the right hilar region invading pericardial fat. Additionally, the tumor was obliterating the Superior Vena Cava. Biopsy of the cardiac mass was positive for Spindle Cell Sarcoma. The patient was not a candidate for surgical resection due to advanced stage malignancy. Unfortunately, patient perished before starting chemotherapy. DISCUSSION: Primary cardiac tumors are mostly benign, 50% to 80% are Myxomas [1]. The remaining 25% of cardiac tumors are malignant, 95% are Sarcomas [2]. The most common malignant tumor is Angiosarcoma. Spindle Cell Sarcoma is the rarest, with only 4 cases reported [2]. Benign tumors usually have a good prognosis with a normal life expectancy after surgical resection. Patients with malignant tumors have a poor prognosis even after surgical intervention, with an average mean survival of 6 months after diagnosis [3]. Diagnosis is made with imaging techniques such as: 2D echocardiogram, cardiac CT, MRI and confirmed with tissue biopsy [3]. Complete resection of the tumor remains the best treatment available with or without adjuvant chemotherapy [3]. CONCLUSIONS: Spindle Cell Sarcoma may present with few symptoms and may remain silent until an advanced stage, thus limiting the few therapeutic options available. The importance of this case relies in the early recognition of symptoms as in our patient who was presenting with Superior Vena Cava Syndrome and Heart Failure misdiagnosed for one year. By the time of diagnosis the patient was not candidate for tumor resection. Reference #1: Primary Cardiac Tumor: A Single-Center 41-Year Experience; Christina Maria Steger et al. International Scholarly Research Network; Vol 2012, Article ID 906109 Reference #2: Primary Intimal (Spindle Cell) Sarcoma of the Heart; A.Ibrahim et al. Hindawi Publishing Corporation; Vol 2013, Article ID 461815 Reference #3: Survival After Resection of Primary Cardiac Tumors. A 48-Year Experience; Andrew W. ElBardissi et al. CirculationAHA.107783126 DISCLOSURE: The following authors have nothing to disclose: Michael Cruz Caliz, Iván González Cancel, Ricardo Fernandez, Modesto Gonzalez-del Rosario, Kelvin Rivera Manzano, Gustavo Gonzalez Astacio, Joel Nieves Scharon, Hiram Jose Maldonado Quintana, Pedro Torrellas-Ruiz, Kimberly Padilla Rodriguez No Product/Research Disclosure Information