Want to learn more about recognizing symptoms and diagnosing patients? In a MedPage article, authors encourage readers to follow along on a case study to understand why a 58-year-old patient is experiencing progressively worse lightheadedness. Can a diagnosis of a rare heart disease, undifferentiated pleomorphic sarcoma, really present in feeling faint? Read the full study published in the American Journal of Case Reports.
The Case Study
The Patient
The patient is a Texan man who has felt lightheaded for months. Previously, he has also experienced seizures. However, he has not been epileptic for years. He has previously spoken to his doctor about his dizziness, but the results from all tests were normal.
Testing
An echocardiogram, an ultrasound test to determine heart function, finds that the patient’s left atrium is inflamed and enlarged. Additionally, they find various masses and a preserved ejection fraction. According to the American Heart Association, preserved ejection fraction is considered diastolic heart failure in which the ventricles of the heart do not relax, even when the heart is contracting normally. When doctors run a transesophageal echocardiogram, they believe that the patient has a primary cardiac tumor.
Next, during surgery, doctors remove some of the masses from the left atrium and also repair some of the aortic valves. Based on their initial findings, as well as cellular evidence, the patient is diagnosed with unclassified pleomorphic sarcoma of the left atrium.
Unclassified Pleomorphic Sarcoma
According to the Mayo Clinic, unclassified (undifferentiated) pleomorphic sarcoma is a rare cancer that forms in soft tissue. It is most often found in the arms or legs but can occur elsewhere. Symptoms vary but include fever, unintended weight loss, and swollen limbs. About 12% of primary cardiac tumors, or tumors that first appear in cardiac tissue, are unclassified.
Undifferentiated pleomorphic sarcoma was once referred to as malignant fibrous histiocytoma. But now, the name of the cancer reflects what researchers know about it. “Undifferentiated” or “unclassified” refers to the difference in appearance between the cancerous cells and the soft tissue cells. “Pleomorphic” refers to the way that the cancerous cells are all different shapes and sizes. Read more about unclassified or undetermined pleomorphic sarcoma here.
Patient Treatment for Pleomorphic Sarcoma
December 2015 – November 2017
For the first 4 months, the patient is given six cycles of chemotherapy with 75 mg/m2 of doxorubicin and 10 g/m2 of ifosfamide. A CT scan finds that the size of the left atrial mass is now smaller. In May of 2016, doctors resection part of the sarcoma and reconstruct the left atrium. They also perform a:
total cardiopulmonary bypass, and cold del Nido cardioplegia for myocardial protection.
Two months later, the patient receives two additional cycles chemotherapy treatment including 675 mg/m2 of gemcitabine and 75 mg/m2 of docetaxel. By June of 2017, the CT scans are showing no continuance of the sarcoma or any additional diseases.
The rare heart disease shows up again in September of 2017 with both a nodule and a lesion. Doctors believe this may mean that the cancer is metastasizing. The patient is put back on two cycles of chemotherapy with doxorubicin and ifosfamide, with the same doses as before. Though imaging shows that the heart disease is not progressing, the patient is experiencing some loss of kidney function.
December 2017 – August 2019
The lesion in the left superior pulmonary vein is growing in size. Doctors change the chemotherapy treatment to six cycles of 75 mg/m2 of doxorubicin and 15 mg/kg of olaratumab. But patient pelvic pain suggests metastatic sarcoma. Over the coming months, the pulmonary tumor continues to grow and does not seem to respond to new treatment of a daily 800 mg of pazopanib or tri-weekly pembrolizumab. By August of 2019, the patient goes into hospice care.
Overall Findings
Imaging played a large role in patient diagnosis in this case study. Researchers suggest transthoracic echocardiography be used for any patients presenting with cardiac-related symptoms, such as shortness of breath, chest pain, or irregular heartbeats. More invasive measures can be used if the researcher needs more information or additional identifying features.
With cardiac pleomorphic sarcomas, symptoms may appear differently based on which side the tumor is present on. On the left, there may be irregular heartbeats, obstructed blood flow, hypertension, and stroke. On the right, symptoms may include pulmonary embolism or chest pressure.
The location makes it difficult to treat cardiac sarcomas. Many cannot be removed; if they are, the chance of reoccurrence or metastasis is high. Most patients only survive an average of 6 to 12 months after diagnosis, even with treatment. In this case, the patient survived for about four years. Understanding what aspects of his treatment or condition may have resulted in this could have implications for future care.
