The rare disease that claimed the life of top US designer
17 Jan 2022 11:38pm
virgil
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The artistic director of Louis Vuitton’s menswear collection succumbed to cardiac angiosarcoma, a rare and aggressive form of cancer, on Nov 28, 2021, at 41.
According to Sunway Medical Centre Velocity consultant cardiologist and internal medicine Dr Foo Yoke Loong, cardiac angiosarcoma is not only a rare disease but difficult to diagnose as well.
He said to date Malaysia does not have any accurate statistics on the number of people afflicted with this cancer.
Globally, the soft tissue sarcoma prevalence rate stood at 5.9 percent per 100,000 population between 1973 and 2006, he said.
Dr Foo said cardiac angiosarcoma belongs to a group of cancers identified as sarcomas that start in the bone and soft tissues such as muscle, fat, blood vessel, nerve and tendon.
Cardiac angiosarcoma consists of primary and secondary cancer. The primary tumour, which is rare, forms in the heart while the secondary tumour can start in other parts of the body, usually on the scalp and neck, and later spread to the heart.
"Secondary tumours are more common compared to primary tumours. Children face a higher risk of developing primary tumours and adults, secondary tumours,” he told Bernama recently.
SYMPTOMS
Dr Foo said cardiac angiosarcoma is hard to diagnose as it rarely occurs and also due to the absence of symptoms in the early stage of the disease. Most of the time, it is only identified after the patient’s symptoms grow severe and the disease has reached an advanced stage.
"Symptoms associated with cardiac angiosarcoma are breathing difficulty and tiredness when exercising or doing heavy work but then, these symptoms can be caused by other diseases as well,” he said.
Only when the tumour grows bigger and presses against the heart will the patient experience shortness of breath, swelling of the legs and chest pain that comes and goes, he added.
Dr Foo said cardiac angiosarcoma is not a hereditary disease and advised the public to go for regular health screenings.
As for the treatment of this cancer, he said it would depend on various factors such as the patient’s age, and the size and location of the tumour.
"If the tumour can be removed, then an open heart surgery will be required to get rid of the whole tumour and to prevent it from recurring. After that, the treatment will proceed with chemotherapy and radiotherapy,” he added.
He also shared that while he was undergoing training to be a cardiologist, he treated a patient who had cardiac angiosarcoma.
"She came to the hospital when she experienced shortness of breath and symptoms of heart failure after her pregnancy. Unfortunately, she died because of the aggressive nature of this cancer,” he said.
He added that reports have indicated that patients diagnosed with cardiac angiosarcoma can live for 12 to 30 months if they undergo a combination of treatments such as surgery, chemotherapy and radiotherapy.
"Without any surgery, the patient may only have an average of four to seven months to live,” he added.
SCREENING
Sunway Medical Centre Velocity consultant clinical oncologist Dr Hafizah Zaharah, meanwhile, shared her experience of treating a young patient who had angiosarcoma on the scalp.
"By the time it was diagnosed, the cancer was at a stage where surgery was not an option. The cancer earlier appeared as a wound on the scalp and later ulcerated. The patient then went through radiation and chemotherapy,” she said.
Being an aggressive form of cancer, cardiac angiosarcoma can spread fast and penetrate into the lining of the heart muscle, which disrupts blood flow to the heart and metastasises. This can lead to death,” she said.
Dr Hafizah said the cancer can be detected through various tests, including an echocardiogram, computed tomography (CT) scan and magnetic resonance imaging (MRI), followed by a heart biopsy.
She said the echocardiogram test is used widely and that it is an easy and non-invasive way to make an early assessment of this cancer.
"It can also detect the presence of a mass or tumour and provide information to identify any obstruction to the circulatory system,” she added. - BERNAMA