Sarcoma CMT
TACKLING THE THREAT OF COMPLEX SARCOMAS
A Sarcoma is a rare type of cancer that affects the connective tissues of the body. About 1 out of 100 cases of adult cancer is sarcoma. There are more than 50 kinds of sarcoma, which are group into two major categories: soft-tissue sarcoma, and bone sarcoma. Below is a description of the same:
Bone Cancer
Bone cancer is a sarcoma that starts in the bone. To have an understanding of this type of cancer, it is important to know the structure of our bones. Bones are the support system of the body, together forming a skeleton which gives us shape and posture. The main parts of the bones are:
Matrix: The outer part of bone. The Matrix is lined with a thin but dense tissue layer called periosteum. Periosteum is made up of nerves and tissues that provide nourishment to the bone.
Bone marrow: This is the soft tissue deep inside of the bones, in the space in hollow bones called the medullary cavity. Bone marrow is made up of fat cells, red blood cells, white blood cells and platelets, connective tissue and Plasma (in which blood cells are suspended)..
Cartilage: The connective tissue at the end of most bones is known as the Cartilage. Cartilage and other tissues including ligaments make up joints which connect the bones.
Bone Cancer Types
There are several types of bone cancers, the most serious being primary bone cancers. Primary bone cancers develop directly in the bones or the surrounding tissues. The most commonly found types of primary bone cancer are:
Osteosarcoma: Also known as osteogenic sarcoma, this is the main type of bone cancer. Osteosarcoma usually originates at the tip of long bones like arms and legs, affecting the hard tissue that provides the outer layer of the bones. It can also originate in the pelvis, shoulders, or the area around the knees. It most commonly occurs in children and adolescents. In adults, it accounts for about one-fourth of bone cancer, and is more prevalent in males than females.
Chondrosarcoma: A cancer affecting the cartilage cells. Chondrosarcoma is the most prevalent bone cancer in adults, with 70% of the cases in patients aged over 40. Chondrosarcoma tends to be diagnosed at an early stage and often is low grade. Many chondrosarcoma tumours are benign, and have a tendency to originate on parts surrounding cartilage, especially the pelvis, leg or arm.
Ewing's sarcoma: A rare cancer that affects the soft tissues around the bone, or directly the bones. Ewing’s sarcoma is the second most prevalent type of bone cancer in children and adolescents, and the third most often found in adults. It accounts for about 8% of bone cancers in adults. Ewing's sarcoma can start in long bones, tissues or organs, especially the pelvis, chest wall, legs or arms.
Secondary (or metastatic) bone cancer: A cancer that spreads to the bone from another part of the body. This type of bone cancer is more prevalent than primary bone cancer.
Soft Tissue Sarcoma
Soft tissue sarcomas develop in the soft tissues of the body like fat, muscle, tendons, nerves, blood vessels, or deep skin tissue. These tissues surround, connect or support the body’s organs and structure.
Types
There are more than 30 types of soft tissue sarcoma making each extremely rare and not all of them being malignant (cancerous). Soft tissue sarcomas are extremely rare, and are grouped according to similar types of cancer cells and symptoms.
Sarcomas of uncertain tissue type: In this type of sarcoma, doctors are not able to determine the exact type of tissue where the cancer began.
Soft Tissue Sarcoma Risk Factors
Anything that increases your chance of getting cancer is a risk factor. For sarcoma, risk factors include:
Bone cancer Symptoms
In most cases, sarcoma does not have symptoms in the early stages. If symptoms are present, they may include:
Soft Tissue Sarcoma Symptoms
Signs of sarcoma vary from person to person. Many times sarcoma does not have symptoms in the early stages. Only about half of soft-tissue sarcomas are found in the early stages before they spread.
The location of the sarcoma makes a difference in the symptoms. For instance if they start:
If you have any of the following problems, talk to your doctor:
These symptoms do not always mean you have sarcoma. However, it is important to discuss any symptoms with your doctor, since they may also signal other health problems.
Accurate diagnosis is the essential first step for the successful treatment of bone cancer. A biopsy is critical to determine the presence of bone cancer and also to know sure if it is benign or malignant.
Imaging
This may include:
APCC is one of the most active sarcoma treatment centres in the world. APCC’s Bone and Soft Tissue CMT surgeons see only sarcoma patients.
This acute specialisation results in a high level of expertise and experience that translates to a higher chance for successful treatment.
The treatment modalities recommended for sarcoma are customized to the particular needs of the patient. One or more of the following therapies may be recommended to treat bone cancer or help relieve symptoms.
Surgery
Surgery is the main treatment for most sarcomas. The goal of surgery is to remove as much of the cancer as possible. If any cancer cells remain, they may grow and spread. To get as much of the cancer as possible, the surgeon performs a wide-excision surgery. This involves removing the cancer, as well as a margin of healthy tissue around it.
If the tumour is in an arm or leg, the surgeon almost always is able to perform limb-sparing surgery, which removes the cancer cells but allows you to keep full use of your leg or arm. To replace the bone that is removed during surgery, a bone graft may be done or an internal device called an endoprosthesis may be implanted.
If this is not possible, an amputation, or removal of the limb, may be performed. Reconstructive surgery and/or prosthesis will be needed. Rehabilitation is necessary after either procedure.
Radiation Therapy
New radiation therapy techniques and remarkable skill allow APCC doctors to target tumours more precisely, delivering the maximum amount of radiation with the least damage to healthy cells.
Proton Therapy
Proton therapy delivers high radiation doses directly into the tumour, sparing nearby healthy tissue and vital organs. Potentially higher doses of radiation and a low incidence of side effects are what make proton therapy an option worth considering for bone tumours, like osteosarcoma and Ewing sarcoma. In cases where complete removal of a bone tumour is not possible, proton therapy may be used to treat tumours without exposing surrounding tissues to high doses of radiation. It is particularly suitable for paediatric bone cancers where even low doses of radiation may result in significant late complications.
Targeted Therapy
These newer agents are used to help fight some types of sarcomas. Targeted therapies attack cancer cells by using small molecules to block pathways that cells use to survive and multiply.
Chemotherapy
Chemotherapy may be recommended to treat osteosarcoma or Ewing’s sarcoma. In osteosarcoma, it is often given before surgery to shrink the tumour and make it easier to remove and after surgery to destroy remaining cancer cells. Chemotherapy is also used for bone cancer that has metastasized (spread) to the lungs or other organs.
