Osteosarcoma (Bone Cancer): Comprehensive Cancer Information

Osteosarcoma (Bone Cancer): A Comprehensive Guide

This information is intended to provide a general overview of Osteosarcoma (Bone Cancer). It is not a substitute for professional medical advice, diagnosis, or treatment. Always consult with a qualified healthcare provider for any health concerns or before making any decisions related to your health.

General Information

Osteosarcoma is a type of bone cancer where malignant (cancerous) cells produce immature bone. It is considered a rare cancer, with an estimated 800 to 900 cases diagnosed annually across the country, approximately half of which occur in children and teenagers.

Common Locations and Timing

Nearly 80% of these tumours develop in the thighbone (femur) or the lower leg bones (tibia or fibula). They can also form in the upper arm bone (humerus).

The tumours typically develop during periods of rapid growth, often referred to as "growth spurts," in the teenage years.

Even if the osteosarcoma appears to be confined to a single area in the lower leg or forearm, almost all patients have microscopic tumour cells, known as micrometastases, present in the lungs.

Understanding Bone Structure

Bones provide support and structure to the body and generally consist of three main components:

Matrix

This is the outer layer of the bone, composed of fibre-like tissue and covered by a layer of tissue called the periosteum.

Bone Marrow

The soft tissue found within the hollow space (medullary cavity) of the bones. Cells in the bone marrow include:

  • Fat cells
  • Red blood cells, white blood cells, and platelets
  • Fibroblasts (cells that help build connective tissue)
  • Plasma (the liquid component where blood cells are suspended)

Cartilage

This material is located at the ends of most bones. It is softer than bone but firmer than soft tissue. Cartilage, along with ligaments and other tissues, forms joints, which connect bones.

Bone is continually being remodelled as new bone is formed and old bone dissolves. When new bone is made, the body deposits calcium into the cartilage.

It is important to note that many cancers that start in other organs can spread to the bones. These are called metastatic bone cancers but are not considered true bone cancers like osteosarcoma.

Osteosarcoma Risk Factors

While the precise cause of osteosarcoma is not known and it does not typically run in families, certain factors can increase a child's risk:

  • Age: It is most common in individuals between 10 and 30 years old, particularly adolescents undergoing rapid growth.
  • Height: Most children who develop osteosarcoma are tall for their age.
  • Gender: Males have a slightly higher risk than females.
  • Previous Radiation Therapy
  • Non-Cancerous Bone Conditions: Including Paget's disease and osteochondromas.
  • Rare Inherited Cancer Syndromes: Such as Li-Fraumeni syndrome, Rothmund-Thompson syndrome, and the RB1 gene mutation (which causes retinoblastoma).
  • Other Rare Genetic Conditions: Including Bloom syndrome and Werner syndrome.

Since osteosarcoma is rare, having these risk factors does not guarantee the disease will develop. If you are concerned about your child's risk, you should consult with their doctor.

Diagnosis

Accurate diagnosis is vital for successful osteosarcoma treatment, as a misdiagnosis can be harmful. The specialised surgeons and pathologists at SSCHRC are highly skilled in diagnosing paediatric osteosarcoma and use the latest methods and technology.

It is strongly advised that the initial biopsy, if possible, be performed at the cancer centre where your child is planned to receive treatment. We recommend seeking a centre that manages a high volume of paediatric osteosarcoma patients and has a dedicated sarcoma team, including specialised pathologists.

Diagnostic Tests

If a child presents with symptoms that suggest osteosarcoma, the doctor will conduct a careful examination and inquire about the child's and family's medical history. One or more of the following diagnostic tests may be used to confirm the presence of osteosarcoma, determine if it has spread, and monitor the effectiveness of treatment:

Blood Tests

Imaging Tests

These may include:

  • CT (Computed Tomography) scans
  • MRI (Magnetic Resonance Imaging) scans
  • X-rays
  • PET (Positive Emission Tomography) scans
  • Bone scans

Biopsy

Two main types of biopsies are used for diagnosis, and the doctor will select the best method for the individual child:

  • Core Biopsy: A needle is used to remove small, cylinder-shaped samples (cores) of tissue.
  • Surgical (Open) Biopsy: An incision is made in the skin, and the surgeon removes a small piece of the tumour. If the tissue removed during a surgical biopsy is confirmed to be osteosarcoma, the surgery to remove the main tumour may be performed during the same procedure.

Treatment

Upon a diagnosis of osteosarcoma, the medical team will discuss the best treatment options, which are tailored based on several factors, including the cancer's size and location, whether it has spread, and the child's age and general health.

Osteosarcoma is typically managed with a combination of therapies, which may include surgery, chemotherapy, and radiation. Surgery is nearly always a necessary part of the treatment plan.

The experts at SSCHRC will design a customised treatment course for your child. One or more of the following therapies may be recommended:

Surgery

Our specialised orthopaedic surgeons at SSCHRC are often able to perform complex limb-sparing surgery, which removes the tumour while preserving surrounding tendons, nerves, and blood vessels, thereby avoiding the need for amputation in an arm or leg. The removed bone is replaced with a bone graft or an internal prosthesis (similar to an artificial joint).

Osteosarcoma surgery is most successful when performed by a specialist with extensive experience in the specific procedure. The surgeons at SSCHRC are highly skilled and among the few in the world who specialise in paediatric sarcoma treatment, utilising minimally invasive and advanced techniques.

Wherever possible, the biopsy and definitive surgical treatment are planned together.

Chemotherapy

Chemotherapy treatment for osteosarcoma is typically administered both before surgery (neoadjuvant chemotherapy) and after surgery (adjuvant chemotherapy).

SSCHRC offers the most current and advanced chemotherapy options.

Radiation Therapy

Radiation therapy uses high-energy beams to destroy cancer cells. Since osteosarcoma cells do not respond well to radiation, it is generally not the primary treatment for most cases. It may be employed if the tumour cannot be completely removed by surgery or to help control symptoms.

If radiation is required, new techniques at SSCHRC allow doctors to target tumours more precisely, delivering the maximum necessary dose while minimising damage to healthy cells. This includes leading-edge treatments such as 3D-conformal radiation therapy and intensity-modulated radiotherapy (IMRT), which are shaped to the tumour's specific form.

Targeted Therapies

SSCHRC is at the forefront of cancer treatment, developing innovative targeted therapies. These agents are specifically designed to treat a cancer's unique genetic and molecular profile to help the body fight the disease.

Many of the doctors treating osteosarcoma at SSCHRC are dedicated researchers who pioneer and actively lead national and international clinical trials involving novel targeted agents.

Our Treatment Approach and Expertise

Osteosarcoma is a rare disease, meaning many oncologists may have limited experience treating it. The physicians at SSCHRC treat a high volume of osteosarcoma patients, which translates into a high level of skill and expertise, earning us a reputation as one of the best osteosarcoma treatment centres in the country.

Multidisciplinary Care

Since osteosarcoma often requires multiple treatments, a comprehensive, personalised team approach is essential. Your child is the focus of a multidisciplinary group of specialists, including surgeons, oncologists, radiation oncologists, and pathologists, who guide care throughout the treatment journey. They are supported by highly trained rehabilitation specialists, therapists, nurses, and other healthcare professionals.

Advanced Treatment Strategy

In most cases, very small, undetectable areas of cancer (micrometastases) have spread to other parts of the body. For this reason, chemotherapy—which travels throughout the body—is almost always the first line of treatment and is also routinely given after surgery.

Surgical Excellence

Osteosarcoma treatment almost always involves surgery. The surgeons at SSCHRC are highly skilled, utilising the most advanced techniques, including limb-sparing surgeries, to help most patients avoid the loss of a limb. If limb-sparing surgery is not an option, SSCHRC provides the most advanced prostheses, complemented by specialised paediatric rehabilitation services.

Groundbreaking Research

Many children with osteosarcoma receive innovative agents through participation in clinical trials. The SSCHRC participates in Children's Oncology Group protocols and other clinical trials that are available at only a select number of hospitals worldwide.

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