Most cardiac tumors are metastatic tumors, which are 20–40 times more common than primary tumors of the heart. Most primary tumors of the heart are benign, with atrial myxomas being the most common. Primary cardiac tumors are extremely rare with an incidence of less than 0.1 percent. Virtually all types of sarcomas have been reported in the heart as isolated case reports. We present a rare case of biatrial high-grade osteosarcoma. A fifty-four-year-old Hispanic female presented with shortness of breath and was cyanotic on the exam while visiting Mexico. Due to abnormal chest x-ray, echocardiogram concerning for bilateral atrial myoma she was referred to a cardiothoracic surgeon. She underwent bi atrial intracardiac tumor resection in Mexico. Several months prior to her resection she noted numbness on the side of the face that was evaluated by her physicians in the United States with a Brain MRI and carotid Ultrasound/Doppler that was unrevealing. She also remembered an episode of uncontrolled hypertension two years prior to surgery requiring admission to a local hospital in East Tennessee with cardiology evaluation that did not include an echocardiogram. Surgical pathology showed extensive undifferentiated spindle cell proliferation with multifocal osteoid production and foci of osseocartilaginous differentiation. There was prominent necrosis and moderately high mitotic rate (10-19/HPF). Tumor cells were positive for SatB2 and negative for vascular, muscular, or neural markers. This is consistent with a primary cardiac high-grade osteosarcoma. These occur very rarely, usually in the atria, and behave aggressively. Post resection staging PET-CT showed hypermetabolic mixed lytic and sclerotic lesion of T10 concerning for metastasis disease. She received approximately 6 cycles of adriamycin and ifosfamide chemotherapy. Adriamycin was discontinued due to left ventricular dysfunction with an ejection fraction of 30-35%, multiple segmental abnormalities, diffuse left ventricular hypokinesis, and moderate to severe mitral valve regurgitation. Despite intracardiac tumor, resection, concern for metastatic disease, chemotherapy, and systolic dysfunction patient is asymptomatic and does have robust performance status. A follow-up PET-CT five months after cessation of treatment reveals no significant evidence of uptake other than abnormalities in the T10 vertebra. A repeat echocardiogram continues to reveal a depressed ejection fraction of 35%. The patient is completely asymptomatic, seemingly fit with an ECOG performance status of 0-1. Osteosarcomas are aggressive with a high incidence of recurrence and metastasis. Fewer than 50 cases of primary cardiac osteosarcomas have been reported in the literature. Currently, it is postulated that they arise from undifferentiated mesenchymal stem cells in the endocardium that transform into active osteoblasts. Even though complete resection can be achieved in some cases, long-term results are usually poor. No standard therapy has been established due to the tumor's low incidence rate and lack of clinical trial data. Our case highlights the importance of evaluating common symptoms thoroughly since it may be a harbinger of rare and serious disorders. This case reflects the heterogeneous nature of sarcoma histology, the consequent tumor biology and hence varied clinical course and prognosis.
| Identifer | oai:union.ndltd.org:ETSU/oai:dc.etsu.edu:asrf-1546 |
| Date | 21 April 2020 |
| Creators | Manthri, Sukesh, Youssef, Bahaaeldin, Chakraborty, Kanishka, Jaishankar, Devapiran |
| Publisher | Digital Commons @ East Tennessee State University |
| Source Sets | East Tennessee State University |
| Detected Language | English |
| Type | text |
| Format | application/pdf |
| Source | Appalachian Student Research Forum |
Page generated in 0.0028 seconds