Getting To The Heart Of Angiosarcoma

Kuala Lumpur, Dec 5: Before this week, the term angiosarcoma was as foreign to most, given the rarity of its occurrence. It was only after cardiac angiosarcoma was made known as the primary cause of death of Virgil Abloh, a well-known celebrity designer that the rare form of cancer came to light.
While it is difficult to diagnose such rare cancers, it is important to first understand what they are, how they can occur and symptoms to look out for.
Known as a rare and aggressive form of cancer, angiosarcoma belongs to a group of cancers known as sarcoma that begin in the bones and soft tissues, which include muscle, fat, blood vessels, nerves, tendons and the lining of joints.
Sunway Medical Centre Velocity (SMCV)’s Consultant Clinical Oncologist, Dr Hafizah Zaharah shares that angiosarcoma can occur anywhere in the body, most often occurring in the skin on the head and neck region.
“Cardiac angiosarcoma is described as an aggressive form of cancer that grows rapidly and infiltrates into the muscle layer of the heart, thus disturbing the blood flow through the heart, metastases and can result in death.
“Sarcomas are so rare that it makes up less than one per cent of all adult malignancies and 12 percent of cancers among children.”
Dr Hafizah recalls that in all her years as an oncologist, she has treated one young patient with angiosarcoma of the scalp.
“Unfortunately, he presented at an unresectable stage.
“It appeared initially as a blue lesion of the scalp which subsequently progressed and ulcerated – he then had to receive radiation and chemotherapy treatments.”

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SMCV Consultant Cardiologist and Internal Medicine, Dr Foo Yoke Loong also relates his experience with a cardiac angiosarcoma patient who presented to the hospital he was attached to while doing his cardiology training years ago with shortness of breath and failure symptoms after pregnancy.
Unfortunately, the patient succumbed to the disease due to its aggressive nature.
“Cardiac angiosarcoma is very difficult to diagnose because it is often asymptomatic.
“Patients might present with mild difficulty in breathing or fatigue during exercise or exertion which can be due to various other reasons besides cardiac angiosarcoma.
“When the angiosarcoma is large and obstructs the heart then the patient might present with reduced effort tolerance, shortness of breath, leg swelling and occasional chest pain,” he said.

Symptoms and treatment for Cardiac Angiosarcoma

Dr Foo elaborates that many patients are often diagnosed when it is already at a late stage of the disease when symptoms are more significant.
However, there are several tell-tale signs that people should be aware of to seek out treatment immediately.
“As of this point and time, researchers have yet to find ways to prevent any type of angiosarcoma.
“If anyone is having any chest discomfort and shortness of breath which are prolonged and worsening in nature, it is recommended to seek for early medical attention as we will be able to diagnose them much earlier,” he adds.
When it comes to diagnosis and treatment, both consultants concur that specific screening tests are a good way to detect this rare cancer.
“Specific screening tests (Echocardiogram or Echo, CT Scan or MRI) followed by a cardiac biopsy can be done to diagnose the existence of this cancer in the patient’s body.
“Echo is widely available and provides a simple, non-invasive technique for the initial evaluation.
“It can identify the presence of a mass or tumour and may provide information about any obstruction to the circulation,” Dr Hafizah adds.

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Dr Foo said that cardiac angiosarcoma is not a hereditary disease, but it is still advisable for everyone to go for regular health check-ups and screening, and treatment varies from each patient.
“Finding the best treatment is difficult as the treatment plan is based on various factors such as the patient’s age, other comorbidity, and the size and location of the tumour.
“If the tumour is resectable then open heart surgery is required to conduct a complete resection in order to prevent the tumour from recurring.
“After that, the treatment will be followed up with chemotherapy and radiotherapy.”
Another rare form of cardiac disease that patients should know about, as pointed out by Dr Foo, is atrial myxoma, a benign growth in the heart.
“This disease has similar symptoms with cardiac myxoma, but these two types of cardiac diseases can be differentiated with an Echo and CT Scan.
“Ultimately, it is important for patients to consult with their trusted doctors or consultants once you recognise symptoms or feel that you require medical attention.
“Any little step can have a big impact on your overall health,” he concludes.

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