A patient once nearly cost Dr Chye Ping Ching her life.
The Chicago-trained senior consultant orthopaedic oncology surgeon was in the middle of operating on the young patient when her appendix burst – a medical emergency.
However, there was no one else near enough that was qualified to continue with the surgery, which was to remove a rare bone tumour and replace the diseased bone.
So, she soldiered on through the hours-long surgery, exiting the operating theatre once she was done, only to promptly enter another one as a patient to remove her ruptured appendix.
It was months before she recovered enough from this life-threatening condition.
And with only a handful of orthopaedic oncology surgeons in the country, her services are certainly needed.
Says Dr Chye: “In the early days, patients with musculoskeletal tumours were treated by general surgeons and general orthopaedic surgeons.
"Virtually everyone with sarcoma succumbed to the disease and surgery was almost always an amputation.
“It is not until the 1980s that orthopaedic oncology emerged as a subspecialty in orthopaedic surgery across Europe and America. In Malaysia, its development has been a slow one since the 1990s.”
It is estimated that there are currently about 250 orthopaedic oncology surgeons in the world.
“The small number of these sub-specialists is not surprising, considering that this discipline is notoriously demanding in terms of commitment, knowledge, surgical skills, attention to the finest of details, concentration, stamina, and mental and physical fitness of the surgeons.
"This has made orthopaedic oncology a challenging subspecialty to master and practice,” says the Malaysian Orthopaedic Association president-elect.
Types of tumours
Dr Chye, one of the pioneering orthopaedic oncology surgeons in the country and one of the very few female ones in the world, explains that there are more than 200 types of tumours in the musculoskeletal system alone.
“Orthopaedic oncology surgeons treat tumours of soft tissue, bone and cartilage origins, and also tumour-like lesions that affect the musculoskeletal system,” she says.
“Muscle, fat, skin, ligaments, tendons, fascia, blood vessels, nerves are all soft tissue. And there are hundreds of these soft tissue tumours.
“Bone tumours are mainly from bone cells or cartilage.”
She adds that these musculoskeletal tumours can either be benign (non-cancerous) or malignant (cancerous).
“And there are also some benign tumours that behave like they are malignant,” she says.
Examples of common malignant bone tumours are osteosarcoma; Ewing’s sarcoma, which affects mostly children and young adults; and chondrosarcoma and multiple myeloma, which are both more common in older people.
Common malignant soft tissue tumours include liposarcoma, synovial sarcoma, malignant peripheral nerve sheath tumour and squamous cell carcinoma.
Common benign bone tumours are osteochondroma, osteoid osteoma, giant cell tumour and fibrous dysplasia, while common benign soft tissue tumours are lipoma, haemangioma, neurofibroma, fibromatosis and schwannoma.
However, there are many more metastatic bone diseases from cancers of the breast, prostate, thyroid and kidney (secondary tumours) than primary tumours that originate in the bone itself.
“Bone is a common place for cancer to spread to, especially in advanced stages,” says Dr Chye.
Fortunately, having bone cancer, even a metastatic one, does not spell immediate doom nowadays.
As Dr Chye explains: “Oncology treatment has advanced tremendously in the past decade with amazing improvement in various diagnostic and imaging methods, drugs, chemotherapy, radiotherapy, treatment protocols, surgical techniques, designs and precision of surgical instruments, biomaterials, implants and prostheses.
“All these have led to better clinical outcome, survival and quality of life for the patients.”
Surgeons now target limb salvage, rather than amputation, and preserving the function of the affected limb, without compromising overall survival, is now the norm.
For example, the five-year survival rate for non-metastatic osteosarcoma and Ewing’s sarcoma can be as good as 70%, if all treatment is performed successfully without delays and complications.
“People have the wrong impression that when the disease spreads to the bone, death is imminent.Instead, many patients are able to survive for a good period of time, even with disease in the bone.
“And for the lucky ones – those with a single metastasis to the bone – it can often be excised and (the affected area) reconstructed.
"With good chemoresponse, extended survival is possible while preserving the integrity and functions of the musculoskeletal system.”
However, according to Dr Chye, many orthopaedic surgeons still fix a pathological fracture in metastatic bone disease like a normal fracture.
“The moment a nail is inserted into the bone in such cases, the whole bone will be contaminated by the cancer cells – the limb becomes unsalvageable,” she says.
Sadly, she has seen too many cases where the fixation of the fracture has failed while the patient is still very much alive.
“The patient will become bed-ridden and nursing care difficult. This is a terrible way to die.” she says.
Dr Chye also notes that many pathological fractures, especially in the elderly, are from undiagnosed primary cancers, which can be challenging to locate.
“I’ve been teaching the post- graduates, don’t rush to fix a pathological fracture – investigate thoroughly and find out what is the underlying pathology, extent of the disease, quality of the bone and soft tissues, and surgical options before fixing it. And make sure the fixation will outlive the patient!
“Tumour surgeries must be done right the first time. The clock is ticking for the patients and there may not be a second chance,” she says.
There are usually two parts in orthopaedic oncology surgeries: excision and reconstruction.
Says Dr Chye: “Meticulous planning is vital to ensure there is no room for error. All instruments needed for surgery, possible difficulties and complications, and ways to overcome them must be thoroughly thought of prior to surgery.
“We always aim to achieve a wide surgical margin for thorough clearance of the tumour. A marginal or intralesional margin will have higher risks of tumour recurrence and poorer prognosis.
“It is important that surgery is done correctly by a trained surgeon the first time. A repeat surgery will definitely result in more extensive loss of tissue and function.”
She notes that often, soft tissue and bone need to be reconstructed after removing the tumour.
“The choice for soft tissue reconstruction depends on the size and tissue composition of the defect.
“For a large defect, a regional or free flap is required to cover it, and even for restoration of motor and sensory functions.
“As for bone defects, we can use an allograft, which is from deceased donors, or an autograft (the patient’s own bone).
“We frequently use megaprostheses (mostly made of titanium alloys), which are modular and readily available. Occasionally custom-made prostheses are needed (especially expandable ones for the growing child),” she says.
“We rely on x-rays, CT (computed tomography) scans, MRI (magnetic resonance imaging), PET (positron emission tomography) scans and radionuclide scans to gather information regarding the extent and effects of the tumour on the bone and soft tissues, for staging of the disease, and monitoring.
“It is important to know the exact diagnosis because different tumours have different clinical behaviours, aggressiveness, metastatic tendencies and responsiveness to treatment.
“Biopsy of the tumour is an important step in the management. Although seemingly easy, its execution requires thorough understanding of the local anatomy, tumour composition and extent, and future surgical approaches, and must be done by the surgeon who is going to perform the definitive surgery.
“A poorly-performed biopsy might not yield appropriate tumour tissue for histopathological examination and might cause the limb to become unsalvageable.”
“Before the surgery, we must study the MRI images in detail. We will convert the 2D images into 3D ones in our head in order to execute the surgery,” Dr Chye explains, adding that continuous assessment and decision-making are important during surgery.
“It is definitely not as simple as just cutting the tumour out. Surgeries are usually long and complicated,” she says.
“A four-hour surgery is a short one for us. Eight to 12 hours is very common. Some multidisciplinary cases last a whole day and night.
“My longest surgery so far lasted 36 hours.”
She adds: “Tumour surgery is team work, there is no way the surgeon can work alone. The team communicates with each other all the time.
"The tumour surgeon, pathologist, radiologist, anaesthetist, plastic surgeon, oncologist, physician, physiotherapist and nurses work hand in hand to achieve the best possible clinical outcome for the patients.”
In addition, facilities like blood banks and a good intensive care unit (ICU) are necessary.
Because of this, orthopaedic oncology surgeries can only be performed in large tertiary hospitals complete with the above-mentioned services.
“Patients often delay coming to hospital until the disease is advanced, please seek treatment early from the right doctors and hospitals.” Dr Chye advises.