Sarcomas are malignant tumors that arise out of transformed cells of connective tissue origin. Connective tissue is a group of tissues that helps in maintaining the form of the body and its organs and provide cohesion and anchorage. It is also a medium for exchange of nutrients, oxygen, and waste products between other tissues; it aids in defense and protection; and in certain sites, as in adipose tissue, it stores fat for cushioning and thermoregulation. Connective tissue, one of the four basic body tissues, is the most versatile, the types including connective tissue proper and the specialized blood, cartilage, and bone. It forms a vast and continuous compartment throughout the body, bounded by the basal laminae of the various epithelia and by the basal and external laminae of muscle cells and nerve-supporting cells. The incidence of bone tumors is very rare but the dismal survival of 50% and disfiguring surgery needed for the treatment make management of bone malignancies challenging. 

Soft tissues include the muscles, tendons, fat, fibrous tissue, synovial tissue, vessels and nerves. The target of soft tissue sarcomas is usually the extremities with incidence of about 60%. The incidence is more in the lower extremities than the upper extremities occurring almost three times more. The trunk comprises 30% with the retroperitoneum accounting for 40% of trunk lesions. The remaining 10% arise in the head and the neck. The age groups and anatomic sites are typical of specific tumor types. For example, osteosarcoma incidence peaks during adolescence and most frequently involves the knee. In contrast, chondrosarcoma affects the pelvis and proximal extremities of older adults. The factors that can lead to the formation of sarcomas can be the following:

Environmental factors

Sarcomas rarely occur due to a previous injury or trauma but they can arise in scar tissue that occur due to previous operation, burn, fracture or foreign body implantation. Exposure to some of the potent chemical carcinogens may also cause the formation of sarcomas. 

Iatrogenic (relating to illness caused by medical examination or treatment) factors

Sarcomas can occur in patients who have been treated with radiation therapy. The tumor mostly arises from the irradiated field.

Viruses

Kaposi sarcoma usually occurs in patients with AIDS

Immunologic factors

Congenital or acquired immunodeficiency, including therapeutic immunosuppression, increases the risk of sarcoma.

Genetic factors

Some mutations can lead to the formation of sarcomas in certain individuals.

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Frequently asked questions about Soft Tissue And Bone Sarcomas And Bone Metastases

What is Soft Tissue Sarcomas ?

Sarcomas are malignant tumors that arise out of transformed cells of connective tissue origin. Sarcomas also arise from soft tissues which include the muscles, tendons, fat, fibrous tissue, synovial tissue, vessels and nerves. The target of soft tissue sarcomas is usually the extremities with incidence of about 60%. The incidence is more in the lower extremities than the upper extremities occurring almost three times more. Sarcomas are usually rare tumors.

What Causes Soft Tissue Sarcomas?

The factors that can lead to the formation of sarcomas can be the following:

Environmental factors

Sarcomas rarely occur due to a previous injury or trauma but they can arise in scar tissue that occur due to previous operation, burn, fracture or foreign body implantation. Exposure to some of the potent chemical carcinogens may also cause the formation of sarcomas. 

Iatrogenic (relating to illness caused by medical examination or treatment) factors

Sarcomas can occur in patients who have been treated with radiation therapy. The tumor mostly arises from the irradiated field.

Viruses

Kapsosi sarcoma usually occurs in patients with AIDS

Immunologic factors

Congenital or acquired immunodeficiency, including therapeutic immunosuppression, increases the risk of sarcoma.

Genetic factors

Some mutations can lead to the formation of sarcomas in certain individuals.

How do you prevent soft tissue sarcoma?
The prevention measures that can be taken to avoid soft tissue sarcoma are:
  • Avoiding radiation exposure
  • Avoiding exposure to potent carcinogens like herbicides and dioxin
  • Genetic predisposition or family having patients suffering from neurofibromatosis, Li-Fraumeni syndrome, retinoblastoma or soft tissue sarcoma
How common is soft tissue sarcoma?

Soft tissue sarcomas are rare. They account for less than 1% of all cases of cancer. 

Who is at risk for sarcoma?

People having genetic predisposition are vulnerable to development of sarcomas. Otherwise, sarcomas are quite rare.

Is a sarcoma always malignant?

Sarcoma is a name that denotes malignancy.

What are the symptoms of Soft Tissue Sarcomas ?

A soft tissue sarcoma may not cause any signs and symptoms in its early stages. As the tumor grows, it may cause:

  • A noticeable lump or swelling
  • Pain, if a tumor presses on nerves or muscles
What is bone metastases?

Bone is a common site for many of the carcinomas of the prostate, breast, lung, kidney, bladder and thyroid and for lymphomas and sarcomas. Prostate, breast and lung primaries account for 80% of all bone metastases.

What is the best treatment for bone metastases?

Common treatments for bone metastasis include medications, radiation therapy and surgery.

Medications

  • Bone-building medications. 
  • Intravenous radiation.
  • Chemotherapy.
  • Hormone therapy.
  • Pain medications.
  • Steroids.
  • Targeted therapy.
What are the symptoms of bone metastases?

Bone metastases are common in many people with cancer. Bone metastases can sometimes result in severe pain and neurological impairment due to changes in the bone structure. Other symptoms of bone metastases can include:

  • Fragile bones
  • High levels of calcium in the blood, which may cause nausea and confusion
  • A loss of urinary or bowel control
  • Weakness in the legs
  • A low blood cell count and anemia due to the loss of bone marrow
How are bone metastases diagnosed?

Bone metastases are usually diagnosed by radiographic imaging techniques:

  • X-rays of the affected bone
  • bone scans to see if other bones are affected
  • CT scans
  • MRI scans
  • blood tests
What is the outlook for people with bone metastases?

The prognosis of bone metastases is usually poor. The treatment aims in improving the quality of life and managing the symptoms associated with the metastases.

Recent Articles

If the tumors of the hematopoietic system are excluded, the most common primary bone cancers are osteosarcoma, chondrosarcoma and Ewing sarcoma.
Osteosarcoma

It is the most common type of primary malignant tumor of the bone and accounts for approximately 20% of all the bone cancers. 75% of these occur in people less than 20 years old. Men are affected slightly more than women. The tumors mostly occur in the metaphyseal region of the long bones; almost 50% are near the knee in the distal femur or proximal tibia. These tumors often present with pain because of pathologic fractures. It can also lead to periosteal bone formation. The radiographic presence of Codman triangle indicates an aggressive tumor. Mostly all patients diagnosed with osteosarcoma have occult metastases at the time of diagnosis.Therefore, treatment usually involves neoadjuvant therapy, surgery and postoperative adjuvant chemotherapy. The usual sites of metastasis are lungs, bones, brain and other sites.

Chondrosarcoma

Chondrosarcomas are malignant cartilage-producing tumors. It is the second most common malignant matrix-producing tumor of bone. They usually arise in the axial skeleton especially the pelvis, shoulder and ribs but distal extremities are rarely involved. They present as painful and progressively enlarging masses. The primary treatment is surgical excision but some may require adjuvant chemotherapy.

Ewing sarcoma

Ewing sarcoma falls under the category of tumors of unknown origin and is a malignant bone tumor characterized by primitive round cells without obvious differentiation. Approximately 80% of the patients are younger than 20 years of age. Boys are usually more affected than girls and mostly arises in the diaphysis of long tubular bones, especially the femur and the flat bones of the pelvis and presents as painful, enlarging mass. The characteristic periosteal reaction produces layers of reactive bone deposited in an onion-skin fashion.

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