Multidisciplinary team successfully treats rare cancer

A dedicated team of healthcare professionals recently treated a patient with a rare type of slow-growing, soft tissue cancer.

It all started in 2022 when the 26-year-old patient known as Neng had symptoms of itchy throat and frequent coughs which developed into breathing difficulties and hoarse voice. He suffered weeks of interrupted sleep and could only sleep sideways with his head propped up. Neng was eventually referred to a private medical centre where he was diagnosed with high-grade fibromyxoid sarcoma on his right neck.

Fibromyxoid sarcoma is a rare form of soft tissue cancer that can affect different parts of the body, such as the arms, legs, abdomen, and head and neck regions. Patients usually have solitary firm mass that can be difficult to detect, hence it is often misdiagnosed as other types of tumours. Treatment is challenging for both patients and doctors due to its rarity and complexity. The most effective treatment so far is surgery followed by radiotherapy. Despite the significant challenges and the inherent risks of treatment, Neng and his family were determined to fight this cancer.

According to Neng’s lead surgeon Dr Eng Chee Yean, a consultant ENT, head and neck, thyroid and parathyroid surgeon, “Advanced imaging showed that the tumour had encased and compressed the carotid artery, which is the main blood supply to the brain. The cancerous tumour in the right neck also displaced and compressed the airway. The tumour had extended down into the chest, reaching the level of the arch of the aorta close to the heart.”

The proximity of the tumour to Neng’s major nerves, blood vessels, airway, and heart posed significant risks including permanent disability or death. Also, the tumour was rapidly growing and compressing his airway causing difficulty in breathing. Therefore, surgery had to be performed urgently after meticulous planning and informed consent. “I had to make sure that the tumour was removed completely from the neck without damaging the trachea and important nerves. Despite the complex nature of the case, Neng displayed tremendous courage, and we were determined to provide him with the best possible care,” said Eng.

The multidisciplinary team of specialists which cared for Neng, comprised Eng, Dr Kenny Cheng, a consultant cardiothoracic surgeon; Dr Liew Ngoh Chin, a consultant general and vascular surgeon; Dr Navinsatku Nithianandhan, a consultant cardiac anaesthesiologist, and Dr Winnie Ng Nyek Ping, a consultant clinical oncologist. Along with a team of nurses and operating staff, the team performed the surgery and successfully removed the tumour without any complication. Neng made a smooth and fast recovery after the surgery.

As of 22 May, he was going through postoperative radiotherapy. He said, “I would not have made it without the love and support of my family and the skills of the doctors. They saved my life. I want to live my life to the fullest and to travel to Japan once I am fully recovered.” Neng hopes that his story can inspire others who might be battling a rare form of cancer. He is greatly relieved to be able to lie down flat again.

Insights from the surgical team and Neng’s family

Dr Navinsatku Nithianandhan recognized the high-risk nature of the surgery and the challenges that the team faced. There were a lot of factors to consider in supporting this complex procedure and monitoring the patient closely, especially the heart and the brain. “The moment I extubated him, we knew everything was going to be well. I was elated and very thankful to be a part of this life-changing surgery.”

Dr Kenny Cheng noted the intricate nature of Neng’s medical condition from the moment of examining his medical records and imaging results. The extent of the tumour right down to the chest invading the arch of the aorta adjacent to the heart presented a unique challenge that required careful consideration and expertise. It involved the challenging task of opening the chest cavity to operate on the arch of the aorta.

Dr Eng Chee Yean said that after conducting a thorough evaluation, the team had to carefully consider various potential risks associated with the procedure. The risks included the impact on Neng’s blood vessels, airway, and blood flow to his brain. To mitigate these risks, a team of multidisciplinary specialists worked collaboratively to develop a comprehensive and effective treatment plan.

Dr Liew Ngoh Chin shared that even though the major blood supply to the brain on the right had been cut off, three other blood vessels: one from the opposite carotid artery and two from the back of the neck (vertebral arteries) to the brain, maintained flow into the brain. This continuous flow had to be maintained to prevent a massive stroke. “The main supply of this vertebral artery was the subclavian artery, which was also encased by the tumour and a bypass had to be performed before the tumour surgery. Intraoperatively, the brain had to be closely monitored for any lack of blood flow.”

Dr Winnie Ng Nyek Ping said that the symptoms of fibromyxoid sarcoma are dependent on tumour location, and thus diagnosis can be particularly challenging. This form of cancer is typically low-grade and is diagnosed in older individuals. However, for Neng the fibromyxoid sarcoma is high-grade and he is only in his 20s. Early detection of the disease and appropriate treatment is crucial to improve the chances of successful treatment. She has been monitoring Neng’s radiotherapy treatment closely, and he is recovering remarkably well. “Despite everything, Neng never lost his determination. With his commitment to his recovery journey and positive attitude, I’m confident that Neng will soon be back to living life as actively as ever.”

SK Lim, Neng’s sister

“We were referred to a private medical centre by a doctor friend and initially, we saw Dr Alex Tang Ah Lak, a consultant vascular and interventional radiologist and Dr Mohd Haris Fadzillah, a consultant physician and haematologist, as we initially suspected it could be lymphoma. When the diagnosis was high-grade fibromyxoid sarcoma, it was life-shattering. I remember when the news was shared with him, I could see something in my brother broke. He was shocked, but we knew that he had a quiet strength in him to face the gruelling treatment process ahead.”

Lim, Neng’s father