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About Sarcoma

What are Sarcomas?

 

Sarcomas

Soft Tissue Sarcomas

Bone Sarcomas

 

Sarcomas

A sarcoma (from the Greek sarx (σάρκα) meaning "flesh") is a cancer that arises from transformed cells of mesenchymal origin. Thus, malignant tumors made of cancerous bone, cartilage or soft tissues such as fat, muscle, connective tissue or blood vessels. This is in contrast to a malignant tumor originating from epithelial cells, which are termed carcinoma. Sarcomas are named more specifically by the type of tissue that they have arisen from, for example, sarcomas of fat are called lipo-sarcomas, and sarcomas from bone are called osteo-sarcomas.  Some sarcomas are so bizarre it is impossible to tell what they developed from and these are often given descriptive names, for example, pleomorphic sarcoma. Furthermore, there are many different types of sarcoma, but it is useful to think of them as either soft tissue sarcomas or bone sarcomas. Sarcomas are also assigned a grade (low, intermediate, or high) based on the presence and frequency of certain cellular and subcellular characteristics associated with malignant biological behaviour.

Sarcomas are often diagnosed late as there are many benign diseases that may have similar symptoms. A multidisciplinary team needs to manage a sarcoma because even a biopsy need to be handled in specific ways. A biopsy should be done after consultation with a sarcoma surgeon if a sarcoma is suspected.

Treatment usually involves a combination of chemotherapy, radiation therapy and surgery and may extend over many months. Low grade sarcomas are usually treated surgically, although sometimes radiation therapy or chemotherapy is used. Intermediate and high grade sarcomas are more frequently treated with a combination of surgery, chemotherapy and/or radiation therapy. Since higher grade tumors are more likely to undergo metastasis (invasion and spread to loco regional and distant sites), they are treated more aggressively. It will often require major surgery that involves removal of muscles and bones and sometimes amputation.  Despite this aggressive treatment the cure rate is in the order of 50%.

Sarcomas occur in approximately 1% of the Australian population. Sarcomas affect people of all ages. Approximately 50% of bone sarcomas and 20% of soft tissue sarcomas are diagnosed in people under the age of 35.Some sarcomas, such as leiomyosarcoma, chondrosarcoma and gastrointestinal stromal tumor(GIST) are more common in adults than in children. Most high-grade bone sarcomas, including Ewing's sarcoma and osteosarcoma, are much more common in children and young adults.

Soft Tissue Sarcomas

A soft-tissue sarcoma is a form of sarcoma that develops in connective tissue such as fat, muscle, nerves, deep skin tissue and blood vessels, though the term is sometimes applied to elements of the soft tissue that are not currently considered connective tissue. Nearly 60% of soft tissue sarcomas arise in the arms or legs, 20% in the trunk, 5% in the head and neck, and 15 - 20% in the abdomen or internal organs. There is almost an equal risk for males and females.

Types

There are over 70 types of soft tissue sarcomas. These are named after the abnormal tissue cells that they have arisen from. Malignant fibrous histiocytoma is the most common type of soft tissue sarcomas and the next most common type is liposarcoma.

• Malignant fibrous histiocytoma, from a cell of unknown origin

• Liposarcoma, from fat tissue

• Leiomyosarcoma and Rhabdomyosarcoma, from muscle tissues

• Angisarcoma, from blood vessels

• Kaposi’s sarcoma of the skin

• Synovial sarcoma, from the tissue that surrounds joints

• Stromal sarcoma, from supporting tissues

• Gastrointestinal stromal sarcoma(GIS), this is treated differently from others types of soft tissue sarcoma

 

 Risk Factors

 The causes of most sarcomas are not known, but there are several risk factors;

• Chlorophenols (in wood preservatives) and phenoxy (herbicides) may have an increased risk of developing soft-tissue sarcomas, angiosarcoma of the liver may associate to vinyl chloride(PVC) in their work.

• High doses of radiation caused soft-tissue sarcomas in some patients.

• Kaposi's sarcoma, a rare cancer of the cells that line blood vessels in the skin and mucus membranes, is caused by Human herpes virus 8.

• Sarcoma may be related to a rare inherited genetic alteration of the p53 gene and is known as Li-Fraumeni syndrome, Werner syndrome, Rothmund-Thomson syndrome, Bloom syndrome and hereditary retinoblastoma.

 

Symptoms

The signs and symptoms of sarcomas vary according to where they develop. Soft tissue sarcomas usually do not cause symptoms in their early stages. Because soft tissue is relatively elastic, tumors can grow rather large, pushing aside normal tissue, before they are felt or cause any problems. The first noticeable symptom is usually a painless lump or swelling. As the tumor grows, it may cause other symptoms, such as pain or soreness, as it presses against nearby nerves and muscles.

 

Diagnosis

The only reliable way to determine whether a soft-tissue tumour is benign or malignant is through a biopsy. There are two methods such as needle aspiration and surgically of tumour tissue for cytopathological analysis.

A pathologist examines the tissue under a microscope. If cancer is present, the pathologist can usually determine the type of sarcoma and its grade. Here, 'grade' refers to a scale used to represent concisely the predicted growth rate of the tumour and its tendency to spread, and this is determined by the degree to which the cancer cells appear abnormal when examined under a microscope. Low-grade sarcomas, although cancerous, are defined as those that are less likely to metastasise. High-grade sarcomas are defined as those more likely to spread to other parts of the body. The most common site to which soft tissue sarcoma spreads is the lungs.

 

Treatment

Your doctor will help you decide on the best treatment for your sarcoma depending on:

• The type of sarcoma that you have

• Where the sarcoma is in your body

• Whether or not the sarcoma has spread (its stage)

• Your age, fitness and general health

• What you want

Treatment may include surgery, chemotherapy and radiotherapy. They can be given alone or in combination.

 

 

Bone Sarcomas

Bone sarcoma is a type of cancer that forms as a painful lump (‘tumour’) in bone. It is also known as bone sarcoma. When a bone sarcoma begins to grow, the cancer cells multiply and start to destroy the bone. The affected bone becomes weak and starts to cause problems. The most common places where bone sarcoma develops are around the knee, the wrist, the shoulder and the pelvis. They are commonest in children, teenagers and young adults, accounting for 4% of all malignancy in children up to 14 years, although much less commonly they can occur in older age groups.

Types

There are over 30 types of bone sarcomas. The most common type is osteosarcoma, which is made up of millions of abnormal bone producing cells. Chondrosarcoma is a bone sarcomas that is made up of millions of abnormal cartilage cells. Ewing’s sarcoma is another type of bone sarcomas.

 

OSTEOSARCOMA

Osteosarcoma is an aggressive malignant neoplasm arising from primitive transformed cells of mesenchymal stem cell origin (and thus a sarcoma) that exhibit osteoblastic differentiation and produce malignant osteoid. It is the most common histological form of primary bone sarcoma.

Osteosarcoma is the eighth most common form of childhood cancer, comprising 2.4% of all malignancies in pediatric patients, and approximately 20% of all primary bone sarcomas. The tumor may be localized at the end of the long bone. Most often it affects the upper end of tibia or humerus, or lower end of femur. Osteosarcoma tends to affect regions around the knee in 60% of cases, 15% around the hip, 10% at the shoulder, and 8% in the jaw. Long-term survival probabilities for osteosarcoma have improved dramatically during the late 20th century and approximated 68% in 2009.

 

Risk Factors

The causes of most sarcomas are not known, but there are several risk factors;

• Bone sarcoma commonly affects teenagers, young adults and people over the age of 55.

• Some people who have had Paget’s disease of the bone, fibrous dysplasia or multiple enchondromas are at higher risk of bone sarcoma.

• Li-Fraumeni syndrome and Rothmund-Thomson syndrome are the predisposing factors for osteosarcoma development.

• High doses of radiation caused bone  sarcomas in some patients.

 

Symptoms

The most common symptom of bone sarcoma is a painful swelling. Pain may not be constant. It may be much worse at night or during activity. Taking pain-relieving tablets may not help. As the sarcoma grows over weeks or months the pain may get worse and become constant. The pain may cause problems with movement.

 

Diagnosis

The route to osteosarcoma diagnosis usually begins with an x-ray, continues with a combination of scans (CT scan, PET scan, bone scan, MRI) and ends with a surgical biopsy. Films are suggestive, but bone biopsy is the only definitive method to determine whether a tumor is malignant or benign.

A pathologist examines the tissue under a microscope. If cancer is present, the pathologist can usually determine the type of sarcoma and its grade. Here, 'grade' refers to a scale used to represent concisely the predicted growth rate of the tumour and its tendency to spread, and this is determined by the degree to which the cancer cells appear abnormal when examined under a microscope

 

Treatment

Your doctor will help you decide on the best treatment for your bone sarcoma, depending on:

• The type of bone sarcoma you have

• Whether or not the sarcoma has spread (its stage)

• Your age, fitness and general health

• What you want

Current standard treatment is to use neoadjuvant chemotherapy (chemotherapy given before surgery) followed by surgical resection. The percentage of tumor cell necrosis (cell death) seen in the tumor after surgery gives an idea of the prognosis and also lets the oncologist know if the chemotherapy regime should be altered after surgery.

 

References:

Darling, J 2007.,"A Different View of Sarcoma Statistics",ESUN 4 (6),Retrieved 2009-04-15

http://www.australiansarcomagroup.org/about-sarcoma.html

http://sarcomabonecancer.org/index.php?p=1_6_Information-on-sarcomas

http://www.londonsarcoma.org/what-is-sarcoma.asp

National Cancer Institute (NCI) U.S. Department of Health and Human Services

http://www.sarcomahelp.org/

http://cancerhelp.cancerresearchuk.org/type/sarcoma/about/risks-and-causes-of-soft-tissue-sarcomas

Ottaviani, G & Jaffe, N  2009, The epidemiology of osteosarcoma. In: Jaffe N. et al. "Pediatric and Adolescent Osteosarcoma", New York: Springer, doi:10.1007/978-1-4419-0284-9_1, ISBN 978-1-4419-0283-2, PMID 20213383

Cancer council; Victoria

http://cancerhelp.cancerresearchuk.org/type/sarcoma/about/about-sarcomas