Osteosarcoma Cancer Treatment in Delhi, India

Osteosarcoma is a type of bone cancer that develops in the cells that form bones. It primarily affects the long bones, such as those in the arms and legs, but it can also occur in other bones. Osteosarcoma often develops in children, adolescents, and young adults, although it can occur at any age. This cancer typically arises in areas of rapid bone growth, such as bone near the growth plates, and it tends to be aggressive, potentially spreading to other parts of the body if not treated promptly.

Osteosarcoma, a type of bone cancer, manifests in two primary types: central (medullary) and surface (peripheral). Each type presents distinct subtypes, categorised based on microscopic examination of tumour cells.

Central/Medullary Osteosarcoma Subtypes

• Conventional Central Osteosarcoma: This encompasses various subtypes, including osteoblastic, chondroblastic, fibroblastic, and mixed types. These subtypes differ based on the type of cells seen under a microscope.
• Telangiectatic Osteosarcoma: Characterised by large cystic or blood-filled spaces within the tumour, this subtype is relatively rare.
• Intraosseous Well-Differentiated or Low-Grade Central Osteosarcoma: This subtype represents a less aggressive form of osteosarcoma.
• Small Cell Osteosarcoma: A rare subtype characterised by small, uniform tumour cells, which may exhibit a more aggressive clinical course.

Surface/Peripheral Osteosarcoma Subtypes

• Parosteal Well-Differentiated or Low-Grade Osteosarcoma (Juxtacortical Osteosarcoma): This subtype originates from the outer layer of the bone, often near the bone surface, and tends to have a better prognosis compared to other types.
• Periosteal Osteosarcoma: Typically low to intermediate grade, this subtype arises beneath the periosteum, the fibrous membrane covering the outer surface of bones.
• High-Grade Surface Osteosarcoma: This subtype presents as aggressive tumours arising on the surface of bones, often in soft tissue adjacent to bone.

The most prevalent subtype of osteosarcoma is conventional central osteosarcoma, which includes osteoblastic, chondroblastic, fibroblastic, and mixed types. In contrast, the other subtypes are less common, each accounting for less than 5% of all osteosarcomas. Understanding the specific subtype of osteosarcoma is crucial for determining appropriate treatment strategies and predicting prognosis.

While the exact cause of osteosarcoma is not fully understood, several factors may contribute to its development:

• Genetic Factors: In some cases, genetic mutations or inherited conditions may predispose individuals to osteosarcoma. For example, individuals with hereditary retinoblastoma, Li- Fraumeni syndrome or Rothmund-Thomson syndrome have an increased risk of developing osteosarcoma.
• Radiation Exposure: Previous exposure to high doses of radiation, such as radiation therapy for other cancers, may increase the risk of developing osteosarcoma later in life. Radiation-induced osteosarcoma typically develops in the irradiated area several years after treatment.
• Bone Conditions: Certain bone conditions or abnormalities may increase the risk of osteosarcoma. For example, individuals with Paget's disease of bone, fibrous dysplasia, or bone infarcts may have an elevated risk of developing osteosarcoma in affected bones.
• Environmental Factors: While specific environmental factors have not been definitively linked to osteosarcoma, some studies suggest a potential association between exposure to certain chemicals or toxins and an increased risk of bone cancer. However, more research is needed to understand the role of environmental factors in osteosarcoma development.
• Age and Gender: Osteosarcoma most commonly occurs in children, adolescents, and young adults during periods of rapid bone growth. Additionally, males are slightly more likely to develop osteosarcoma than females.

It's important to note that most cases of osteosarcoma occur sporadically, without a clear cause or identifiable risk factors. While these factors may increase the risk of developing osteosarcoma, not everyone with these risk factors will develop the disease. Additionally, many individuals diagnosed with osteosarcoma do not have any known risk factors, highlighting the complexity of the disease and the need for further research into its causes.

Osteosarcoma may manifest with various symptoms, although some individuals with osteosarcoma may not experience any symptoms initially. Common signs and symptoms of osteosarcoma may include:

• Bone Pain: Persistent bone pain, which may worsen at night or with activity, is a hallmark symptom of osteosarcoma. The pain often occurs at the site of the tumour and may be localised or diffuse.
• Swelling or Lump: A noticeable lump or swelling may develop over the affected bone, particularly if the tumour is located near the surface of the body. The lump may feel firm or tender to the touch.
• Limited Range of Motion: Osteosarcoma tumours in or near joints can cause stiffness and limited range of motion in the affected joint. This may lead to difficulty with everyday activities such as walking or bending.
• Bone Fractures: Osteosarcoma weakens the affected bone, increasing the risk of fractures, even with minimal trauma or stress. Fractures may occur spontaneously or with minor injuries.
• Limping or Favouring One Limb: Children with osteosarcoma in the leg bones may develop a limp or start favouring one limb due to pain or discomfort.
• Unexplained Weight Loss: In some cases, individuals with osteosarcoma may experience unexplained weight loss, fatigue, or general malaise. These symptoms may indicate advanced disease or systemic effects of the cancer.
• Fatigue: Ongoing pain and discomfort associated with osteosarcoma can lead to fatigue and decreased energy levels. Fatigue may also result from the body's efforts to fight cancer.

It's essential to note that these symptoms are not exclusive to osteosarcoma and may also occur due to other conditions or injuries. However, if any of these symptoms persist or worsen over time, it's important to consult an orthopaedic oncologist for further evaluation and diagnosis.

Diagnosing osteosarcoma typically involves a series of tests. Here's a breakdown of the usual steps:

Initial Examination and Medical History

A doctor will first ask about the patient's medical history and symptoms. This may include questions about:

• Bone pain, especially at night or with activity
• Swelling or tenderness around a bone
• Limited movement in an affected area
• Unexplained bone fractures

Imaging Tests

Several imaging tests can help visualise the bone and surrounding tissues to identify abnormalities suggestive of osteosarcoma. These may include:

• X-ray: This is often the first imaging test used. It can reveal abnormalities in the bone, such as destruction or new bone formation.
• MRI (magnetic resonance imaging): This creates detailed images of bones and soft tissues, which can help determine the size, location, and extent of a tumour.
• CT scan (computed tomography): This provides detailed cross-sectional images of bones and surrounding tissues. It may be used to assess the spread of cancer to other parts of the body, such as the lungs.
• Bone scan: A small amount of radioactive material is injected into the bloodstream, which is taken up by bones. Areas with increased bone activity, such as tumours, will appear brighter on the scan.
• PET scan (positron emission tomography): This scan uses a radioactive tracer to measure the metabolic activity of cells. Cancer cells are typically more metabolically active than healthy cells, so they will appear brighter on the scan. A PET scan may be used to detect osteosarcoma that has spread to other parts of the body.

Biopsy

A biopsy is the only definitive way to diagnose osteosarcoma. During a biopsy, a small sample of tissue is removed from the tumour and examined under a microscope by a pathologist to determine if it is cancerous. There are different types of biopsies that can be performed, such as:

• Core needle biopsy: A thin needle is inserted into the tumour to collect a sample of tissue.
• Surgical biopsy: An incision is made in the skin to remove a larger sample of tissue.
• Fine needle aspiration (FNAC) : A thin needle is inserted into the tumour to withdraw a small amount of fluid or cells. It is important to note that FNAC is not recommended for a primary diagnosis of osteosarcoma, as it can be misleading. It can, however, be used to prove a suspected recurrence in a known case of osteosarcoma.

If osteosarcoma is diagnosed, the doctor will determine the stage of the cancer. The stage is based on the size and location of the tumour, as well as whether it has spread to other parts of the body. The stage of cancer helps determine the prognosis (outlook) and treatment plan.

Treatment for osteosarcoma typically involves a multidisciplinary approach, incorporating surgery, chemotherapy, and sometimes radiation therapy. The specific treatment plan is determined based on factors such as the location and extent of the tumour, the patient's age and overall health, and whether the cancer has spread to other parts of the body. Here's an overview of the main treatment modalities for osteosarcoma:

Surgery is the primary treatment for localised osteosarcoma, aimed at removing the tumour while preserving as much healthy bone and tissue as possible. The surgical approach may involve either limb-sparing surgery or amputation, depending on factors such as tumour size, location, and response to preoperative chemotherapy.

Limb-sparing surgery (also known as limb-salvage surgery) involves removing the tumour and a margin of surrounding healthy tissue while preserving the affected limb. Reconstruction techniques, such as bone grafts, prosthetic implants, or biological substitutes, may be used to restore function and appearance. At Max Hospitals, more than 90 percent of patients with osteosarcoma are treated with a limb sparing surgery.

In cases where limb-sparing surgery is not feasible or if the tumour involves critical structures, such as major blood vessels or nerves, amputation may be necessary to achieve complete tumour removal and prevent recurrence.

Chemotherapy is an essential component of treatment for osteosarcoma, both before (neoadjuvant) and after (adjuvant) surgery.

Neoadjuvant chemotherapy is administered before surgery to shrink the tumour, reduce the risk of metastasis, and make surgical resection more feasible.

Adjuvant chemotherapy is given after surgery to eradicate any remaining cancer cells, reduce the risk of recurrence, and improve long-term survival.

Chemotherapy regimens typically consist of a combination of drugs, such as doxorubicin, cisplatin, methotrexate, and ifosfamide, administered in cycles over several months.

Radiation therapy may occasionally be used in combination with surgery and chemotherapy in certain cases of osteosarcoma, particularly when the tumour is located in areas where achieving adequate surgical margins is challenging.

External beam radiation therapy delivers high-energy radiation beams to the tumour site, targeting cancer cells and reducing the risk of local recurrence.

Radiation therapy may also be used palliatively to relieve pain and improve quality of life in individuals with advanced or metastatic osteosarcoma.

Treatment for osteosarcoma varies from patient to patient, and decisions regarding the most appropriate treatment approach should be made in consultation with a multidisciplinary team of healthcare professionals, including orthopaedic oncologists, medical oncologists, radiation oncologists, and other specialists.

Osteosarcoma and its treatment can lead to both short-term and long-term complications. Here's a breakdown of some potential complications:

• Bone fractures: Osteosarcoma weakens the bone, making it more prone to fractures, especially around the tumour site.
• Wound infection and slow healing: Surgery can introduce a risk of infection at the incision site, and healing might be slow depending on the extent of surgery.
• Problems with implants or prosthetics: If surgery involves using bone grafts or prosthetics to replace a resected bone, there's a possibility of infection, loosening, non-union or other issues with the reconstruction, over time.
• Chemotherapy side effects: Chemotherapy drugs can cause various side effects, including fatigue, nausea, vomiting, hair loss, anaemia, increased risk of infection, nerve damage, and hearing loss. In some cases, there might be long-term effects on heart, kidney, or liver function.

Osteosarcoma prevention primarily focuses on reducing known risk factors and promoting healthy lifestyle habits. While osteosarcoma is not entirely preventable, some measures may help lower the risk:

• Avoid Radiation Exposure: Minimise exposure to ionising radiation, as it is a known risk factor for osteosarcoma development. This includes limiting unnecessary medical imaging procedures involving radiation.
• Limit Chemical Exposure: Limit exposure to chemicals known to increase the risk of osteosarcoma, such as certain herbicides and pesticides. Protective measures should be taken when handling potentially carcinogenic substances.
• Healthy Lifestyle Choices: Encourage maintaining a healthy lifestyle, including a balanced diet rich in fruits and vegetables, regular physical activity, and avoidance of tobacco and excessive alcohol consumption.
• Genetic Counselling: Individuals with certain genetic conditions, such as Li-Fraumeni syndrome or hereditary retinoblastoma, have an increased risk of developing osteosarcoma. Genetic counselling and testing may be recommended for individuals with a family history of these conditions.

While these preventive measures can reduce the risk of osteosarcoma, it's essential to remember that not all cases can be prevented. Regular medical check-ups and prompt evaluation of any concerning symptoms are crucial for early detection and treatment if osteosarcoma does occur.

How common is osteosarcoma?

Osteosarcoma is a relatively rare type of cancer, accounting for approximately 3% of all childhood cancers and about 2 percent of all cancers diagnosed in adolescents and young adults. It most commonly occurs in children and young adults between the ages of 10 and 30, although it can occur at any age.

Does osteosarcoma cause pain?

Yes, osteosarcoma can cause pain, especially at the site of the tumour. The pain may be persistent and worsen over time as the tumour grows. Other symptoms may include swelling, tenderness, and limitations in joint movement near the tumour site.

Why does osteosarcoma hurt more at night?

Osteosarcoma pain may be more noticeable at night due to various factors, including changes in activity level, positioning during sleep, and decreased distraction from daily activities. Additionally, lying down may increase pressure on the affected area, exacerbating pain associated with the tumour.

Can I feel an osteosarcoma tumour?

In some cases, individuals may be able to feel an osteosarcoma tumour, especially if it is located near the surface of the body or in a limb. However, tumours located deep within the body, such as those in the pelvis or spine, may be more challenging to detect through touch alone.

Who treats osteosarcoma?

Osteosarcoma is typically treated by a multidisciplinary team of healthcare professionals, including orthopaedic oncologists (orthopedic surgeons who specialise in musculoskeletal cancers), medical oncologists, radiation oncologists, paediatric oncologists (for paediatric cases), pathologists, radiologists, and nurses specialising in oncology care. Each member of the team plays a crucial role in diagnosing, staging, and treating the disease, as well as providing supportive care throughout the treatment process.

What foods should I eat or avoid to reduce my risk of osteosarcoma?

While there are no specific foods that directly cause or prevent osteosarcoma, maintaining a balanced diet rich in fruits, vegetables, whole grains, and lean proteins can support overall health and may help reduce the risk of cancer. Avoiding excessive consumption of processed meats, sugary foods, and high-fat foods may also contribute to a healthy lifestyle.

Is osteosarcoma fatal?

Osteosarcoma can be a serious and potentially life-threatening condition, particularly if not diagnosed and treated promptly. However, with advances in medical treatment and multidisciplinary care, an increasing number of individuals with osteosarcoma are achieving long-term survival and cure.

What is the likelihood of osteosarcoma recurring after treatment?

The likelihood of osteosarcoma recurring after treatment depends on various factors, including the stage of the cancer, the effectiveness of the initial treatment, and individual patient characteristics. Recurrence rates vary, but close monitoring during follow-up appointments is essential to detect and address any signs of recurrence early.

Is there a cure for osteosarcoma?

Yes. Detected early and treated with multiagent chemotherapy and complete surgical removal, the reported cure rates vary between 60 – 80 %, depending on various factors. Early detection, prompt treatment, and multidisciplinary care are critical factors in improving outcomes.

Can anyone live a long life after osteosarcoma?

Yes, an increasing number of individuals with osteosarcoma are now living long and fulfilling lives after treatment. With advancements in medical care, including surgical techniques, chemotherapy protocols, and supportive care measures, the prognosis for osteosarcoma patients has improved significantly in recent years. However, long-term follow-up and monitoring are essential to detect and manage any potential late effects or recurrence of the disease.

What are the different stages of osteosarcoma, and how do they impact treatment and prognosis?

Osteosarcoma staging helps determine the extent of the cancer and guides treatment decisions. Stages range from I to IV, with higher stages indicating more advanced disease. Treatment options and prognosis vary depending on the stage. For example, low grade osteosarcoma may be treated with surgery alone, while higher grade disease often requires a combination of surgery, chemotherapy, and rarely radiation therapy. Prognosis tends to be better for patients with lower-stage disease, as they have a higher chance of successful treatment and long-term survival.

Are there any known genetic factors or predispositions that increase the risk of developing osteosarcoma?

While most cases of osteosarcoma occur sporadically, some genetic conditions, such as Li-Fraumeni syndrome and hereditary retinoblastoma, are associated with an increased risk of developing osteosarcoma. Additionally, certain inherited genetic mutations may predispose individuals to the disease. However, these genetic factors only account for a small percentage of osteosarcoma cases, and most cases occur without a known genetic predisposition.

How does age affect the prognosis and treatment options for osteosarcoma patients?

Age can impact the prognosis and treatment options for osteosarcoma patients. Younger patients, particularly adolescents and young adults, tend to have better outcomes compared to older adults. This may be due to factors such as overall health, response to treatment, and ability to tolerate aggressive therapies like chemotherapy. Additionally, treatment approaches may vary based on age, with younger patients often being more eligible for intensive treatments such as limb-salvage surgery.

How does osteosarcoma impact daily activities and quality of life for patients and their families?

Osteosarcoma and its treatment can significantly impact daily activities and quality of life for patients and their families. Physical limitations, pain, fatigue, emotional distress, and financial strain are common challenges faced by individuals undergoing treatment for osteosarcoma. Supportive care measures, including pain management, rehabilitation services, psychosocial support, and financial assistance programs, can help mitigate these challenges and improve overall well-being.

What supportive care options are available to manage symptoms and improve well-being during osteosarcoma treatment?

Several supportive care options are available to help manage symptoms and improve well-being during osteosarcoma treatment. These may include pain management techniques, physical therapy, occupational therapy, counselling or psychotherapy, nutritional support, and palliative care services. Additionally, healthcare providers may offer resources and referrals to community-based support programs and organisations that provide practical and emotional support to patients and their families. The sarcoma support group at Max Hospital is one such group of patients and survivors.

Are there any clinical trials or research studies available for individuals with osteosarcoma who are seeking new treatment options?

Clinical trials and research studies play a vital role in advancing the understanding and treatment of osteosarcoma. Many academic medical centres and research institutions conduct clinical trials to evaluate new therapies, treatment combinations, and supportive care interventions for osteosarcoma patients. Individuals interested in participating in clinical trials should discuss these options with their healthcare team to determine eligibility and explore available opportunities.

How does the location of the osteosarcoma tumour affect treatment decisions and outcomes?

The location of the osteosarcoma tumour can influence treatment decisions and outcomes. Tumours located in certain areas of the body, such as the limbs, may be amenable to limb-salvage surgery, where the affected limb is preserved. In contrast, tumours in critical locations, such as the spine or pelvis, may require more extensive surgery or alternative treatment approaches. Additionally, the proximity of the tumour to vital structures and organs may impact the feasibility of surgical resection and affect treatment outcomes.

What are the emotional and psychological effects of an osteosarcoma diagnosis, and how can patients and families cope?

An osteosarcoma diagnosis can have profound emotional and psychological effects on patients and their families. Feelings of fear, anxiety, sadness, anger, and uncertainty are common reactions. Coping strategies may include seeking support from loved ones, joining support groups, engaging in relaxation techniques, maintaining open communication with healthcare providers, and seeking professional counselling or therapy when needed. It's essential for patients and families to address their emotional well-being as part of their overall treatment plan.

Are there resources or support groups available for individuals with osteosarcoma and their caregivers?

Yes, there are resources and support groups available for individuals with osteosarcoma and their caregivers. These may include online forums, peer support programs, local and national nonprofit organisations, and hospital-based support services. These resources offer opportunities for individuals to connect with others facing similar challenges, share experiences, access

educational materials, and receive emotional support throughout their osteosarcoma journey. The sarcoma support group at Max Hospital is one such group of patients and survivors.

What is the outlook/prognosis for osteosarcoma?

The outlook/prognosis for osteosarcoma varies depending on factors such as the stage of the cancer, response to treatment, and individual patient characteristics. Generally, localised osteosarcoma has a more favourable prognosis with aggressive treatment (reported long term survival rates of 60-80% in early disease) while metastatic disease carries a poorer prognosis. Response to chemotherapy and successful surgical resection are crucial for improving outcomes. Regular surveillance is important for monitoring recurrence and late effects of treatment. Overall, advancements in treatment have led to improved survival rates, but each case is unique. Early diagnosis and comprehensive care are essential for optimising outcomes in osteosarcoma.

Reviewed By Dr. Akshay Tiwari, Senior Director - Musculoskeletal Oncology, Cancer Care / Oncology, Paediatric (Ped) Oncology, Surgical Oncology on 21 May 2024.