A 5-year-old boy comes into the clinic with a swollen right forearm. His parents only villagers. We investigate and treat for an infection of the bone (osteomyelitis). Months go by with no improvement. The swelling has increased and x-rays are now highly suggestive of a bone cancer. Unable to do a biopsy as the parents are reluctant to consent for any procedure and results will take months to come back, the option for amputation is discussed. Scared and confused the parents decide not to have their sons arm amputated and request to wait and take him back to the village. More months go by and the young man returns with his parents to the clinic. The swelling has engulfed his elbow now. His parents see no other choice but to amputate. A difficult and hard decision. The chances of success are lower now but we have to remove the cancerous arm if he has any chance of survival. He is taken into the operating theatre with two arms and comes out with only one. He is nursed in the ward until discharge a few days later. Chemotherapy now the next part of his treatment. He returns to clinic two weeks later. The wound healed but has developed right sided shoulder and facial swelling. It was too late. The cancer has spread and chemotherapy drugs are not available. His parents take him home where he passes away.
Three other cases seen in our clinics. All teenagers, one female and two males with lower limb cancerous bone growths confirmed from x-rays. All wait late to make the decision to amputate. Two go through with the amputation but soon develop metastasis to the lungs. The other who refused also develops metastasis and is taken home for palliative care. Sad but true stories. We have seen many more others including adults with the same condition but present late or either continually refuse surgical intervention.
So, what is bone cancer?
Bone cancer can refer to primary bone cancer or secondary bone cancers. Primary bone cancer is cancer that begins in the bones. Secondary (metastatic) bone cancer refers to a cancer that started elsewhere in the body and has spread to the bones.
Each year about 1000 new cases of bone cancer are diagnosed in the UK and USA. Bone cancers represent less than 1% of all cancers diagnosed worldwide each year.
What are the common types of bone cancer?
There are more than 30 different types of primary bone cancer. Also called bone sarcoma, the most common types include:
- Osteosarcoma, which starts in cells that grow bone tissue
- Chondrosarcoma, which starts in cells that grow cartilage
- Ewing’s sarcoma, which affects cells in the bone or soft tissue that multiply rapidly.
Who can get bone cancer?
- Osteosarcoma: the most common type, which mostly affects children and young adults under 20.
- Ewing sarcoma: which most commonly affects people aged between 10 and 20.
- Chondrosarcoma: which tends to affect adults aged over 40.
Young people can be affected because the rapid growth spurts that occur during puberty may make bone tumours develop.
What causes bone cancer?
In most cases, it is not known why a person develops bone cancer.
Some factors that can increase your risk of bone cancer include:
- Previous radiotherapy, particularly for people who received high doses at a young age
- Other bone conditions, such as Paget’s disease of the bone
- Genetic factors, such as inherited conditions like Li-Fraumeni syndrome, and a strong family history of certain cancers.
What are the signs and symptoms?
- swelling over the affected part of the bone
- stiffness or tenderness in the bone
- problems with movement
- unexplained weight loss
- a fractured bone
- loss of feeling in the affected limb
- tiredness
How do you diagnose bone cancer?
Radiological studies such as:
X-rays, CT scan, MRI and Bone scans can be used to effectively diagnose cancer of the bone.
X-rays are reliable and easily accessible. X-rays of a typical bone cancer would show the cancer eroding the normal bone and surrounding structures. Other findings may include a “Sun burst” appearance or an “onion skin” appearance.
CT scans and other studies are not available in most provincial hospitals in PNG.
Biopsy: can confirm a diagnosis. Histopathology services in PNG are slow and unreliable due to the sheer number of samples that are sent to pathology labs and the lack of pathologists. The option to wait for a diagnosis or amputate is a common dilemma for doctors and patients in resource limited countries such as PNG.
What are the most common sites for bone cancer?
Is there a cure for bone cancer?
Primary bone cancer is usually treated with surgery, chemotherapy and radiotherapy or a combination of these treatments.
Many cases of bone cancer are successfully treated. In these instances, cancer never returns. Sometimes people need multiple surgeries to accomplish this outcome. Other people with bone cancer might need to continue treatments including radiation therapy and chemotherapy to keep cancer from spreading.
Before 1970, amputation was the sole treatment for a high-grade osteosarcoma and 80% of patients would die due to lung metastasis even after amputation. These tumours are very aggressive in their nature; hence, most of the surgeon’s considered amputation as the answer to halt its aggressiveness. Surgical options include:
- Limb-salvage (limb-sparing) surgery: removing the cancer and some surrounding normal tissue but leaving the limb basically intact. This is reserved for small cancers that are localised to the bone.
- Amputation: Removing the cancer and all or part of an arm or leg. This is indicated for large cancers that will involve other structures. An amputation is often the only mode of treatment for many as chemotherapy and radiotherapy may not be available. In such cases an amputation is considered a life saving procedure.
“The earlier the diagnosis the better the chances of a successful treatment”
Can we prevent bone cancer?
There are no known lifestyle changes that can prevent bone cancers.
What is palliative care?
In some cases of bone cancer, your medical team may talk to you about palliative care. Palliative care aims to improve your quality of life by alleviating symptoms of cancer and not treatment to cure. As well as slowing the spread of bone cancer, palliative treatment can relieve pain and help manage other symptoms. Treatment may include radiotherapy, chemotherapy or other drug therapies.
Where does bone cancer metastasize to?
Primary bone cancer commonly spreads to the lungs (secondary lung cancer). Once this happens the cancer is of an advanced stage. This is eventually what kills the patient. Chest X-rays may show “cannon ball” lesions with fluid in one or both lungs.
What is the prognosis of bone cancer?
According to USA and UK statistics:
- Around 85 out of 100 people (around 85%) survive their cancer for 1 year or more.
- More than 65 out of 100 people (more than 65%) survive their cancer for 5 years or more.
These figures are largely due to the fact that diagnosis and treatment options in developed countries have significantly improved since 1970. This all has occurred because of improvements mainly in multimodal chemotherapy improved imaging investigations, improved histopathological techniques such as various tumour markers, molecular studies of tumour, surgical technique improvements (better skeletal reconstruction after tumour resection), advances in bioengineering, and endoprosthesis. Implant designs, osteoarticular allografts, allograft prosthetic composites, expandable prosthesis, vascularized grafts, and arthrodesis and rotation plasty. Treatment teams are also important to successfully provide a multimodal approach to manage any cancer cases including bone cancer. These teams, depending on the treatment may include:
- GP (General Practitioner): looks after your general health and works with your specialists to coordinate treatment.
- Surgeon: surgically removes tumours and performs some biopsies.
- Radiation oncologist: prescribes and coordinates radiation therapy treatment.
- Medical oncologist: prescribes and coordinates the course of chemotherapy.
- Pain management team: treats pain, particularly if it is difficult to control or severe.
- Cancer nurse: assists with treatment and provides information and support throughout your treatment.
- Physiotherapists: for rehabilitation or prosthesis after surgery.
- Other allied health professionals: such as social workers, pharmacists, and counsellors.
As the rest of the developed world make waves in managing and fighting cancer, developing countries such as PNG are still struggling to provide the basic of services. As a result, cancer is often neglected, health workers are powerless and patients are left to the mercy of GOD.
References
- https://cancer.org.au/cancer-information/types-of-cancer/bone-cancer
- https://www.cancerresearchuk.org/health-professional/cancer-statistics/statistics-by-cancer-type/bone-sarcoma/incidence
- Dhammi, I. K., & Kumar, S. (2014). Osteosarcoma: A journey from amputation to limb salvage. Indian journal of orthopaedics, 48(3), 233–234. https://doi.org/10.4103/0019-5413.132486
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