Soft tissue sarcoma

From WikiMD

Soft tissue sarcoma is a broad term for cancers that start in soft tissues (muscle, tendons, fat, lymph and blood vessels, and nerves). These cancers can develop anywhere in the body but are found mostly in the arms, legs, chest, and abdomen. Soft tissue sarcoma is a form of cancer that occurs due to abnormal and uncontrolled cell growth of the "soft tissues" of the body. These tissues connect, support and surround other body parts and may include muscle, fat, blood vessels, lymph vessels, nerves, tendons and the lining of joints.

Soft tissue sarcoma.jpg
Undifferentiated soft tissue sarcoma in left lung of young child.jpg

Types

Table 1: Major Types of Soft-Tissue Sarcomas in Adults
Tissue of Origin Type of Cancer Usual Location in the Body
Fibrous tissue Fibrosarcoma Arms, legs, trunk
Malignant fibrous
hystiocytoma
Legs
Dermatofibrosarcoma Trunk
Fat Liposarcoma Arms, legs, trunk
Muscle
Striated muscle
Smooth muscle

Rhabdomyosarcoma
Leiomyosarcoma

Arms, legs
Uterus, digestive tract
Blood vessels Hemangiosarcoma Arms, legs, trunk
Kaposi's sarcoma Legs, trunk
Lymph vessels Lymphangiosarcoma Arms
Synovial tissue
(linings of joint cavities, tendon sheaths)
Synovial sarcoma Legs
Peripheral nerves Malignant peripheral nerve sheath tumour/Neurofibrosarcoma Arms, legs, trunk
Cartilage and bone-forming tissue Extraskeletal chondrosarcoma Legs
Extraskeletal osteosarcoma Legs, trunk (not involving the bone)
Table 2: Major Types of Soft-Tissue Sarcomas in Children
Tissue of Origin Type of Cancer Usual Location in the Body Most common ages
Muscle
Striated muscle Rhabdomyosarcoma
Embryonal Head and neck, genitourinary tract Infant–4
Alveolar soft part sarcoma Arms, legs, head, and neck Infant–19
Smooth muscle Leiomyosarcoma Trunk 15–19
Fibrous tissue Fibrosarcoma Arms and legs 15–19
Malignant fibrous
histiocytoma
Legs 15–19
Dermatofibrosarcoma Trunk 15–19
Fat Liposarcoma Arms and Legs 15–19
Blood vessels Infantile hemangio-pericytoma Arms, legs, trunk, head, and neck Infant–4
Synovial tissue
(linings of joint cavities, tendon sheaths)
Synovial sarcoma Legs, arms, and trunk 15–19
Peripheral nerves Malignant peripheral nerve sheath tumors (also called neurofibrosarcomas, malignant schwannomas, and neurogenic sarcomas) Arms, legs, and trunk 15–19
Muscular nerves Alveolar soft part sarcoma Arms and legs Infant–19
Cartilage and bone-forming tissue Extraskeletal myxoid chondrosarcoma Legs 10–14
Extraskeletal mesenchymal Legs 10–14


Cause

No one knows exactly what causes these cancers.

Riskfactors

People who have previously received radiation therapy and those with certain inherited disorders (such as Gorlin syndrome, Gardner syndrome, Li-Fraumeni syndrome, Tuberous sclerosis, neurofibromatosis type 1, and Werner syndrome) have an increased risk of developing a soft tissue sarcoma.

Signs and symptoms

A sarcoma may appear as a painless lump under the skin, often on an arm or a leg. Sarcomas that begin in the abdomen may not cause signs or symptoms until they get very big. As the sarcoma grows bigger and presses on nearby organs, nerves, muscles, or blood vessels, signs and symptoms may include:

  • Pain.
  • Trouble breathing.

Diagnosis

The only reliable way to determine whether a soft-tissue tumour is benign or malignant is through a biopsy. The two methods for acquisition of tumour tissue for cytopathological analysis are:

  • Needle aspiration biopsy, via needle
  • Surgically, via an incision made into the tumour

A pathologist examines the tissue under a microscope. If cancer is present, the pathologist can usually determine the type of cancer 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. For soft-tissue sarcoma, the two histological grading systems are the National Cancer Institute system and the French Federation of Cancer Centers Sarcoma Group system.

Treatment

The best treatment options depend on many factors but may include surgery, chemotherapy, and radiation therapy. The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

  • Olaratumab (Brand name: Lartruvo)In combination with doxorubicin, for the treatment of adult patients with soft tissue sarcoma (STS) with a histologic subtype for which an anthracycline-containing regimen is appropriate and which is not amenable to curative treatment with radiotherapy or surgery.
  • Pazopanib (Brand name: Votrient)Advanced soft tissue sarcoma (STS) who have received prior chemotherapy.

Epidemiology

Soft-tissue sarcomas are relatively uncommon cancers. They account for less than 1% of all new cancer cases each year. This may be because cells in soft tissue, in contrast to tissues that more commonly give rise to malignancies, are not continuously dividing cells.

In 2006, about 9,500 new cases were diagnosed in the United States.Soft-tissue sarcomas are more commonly found in older patients (>50 years old), although in children and adolescents under age 20, certain histologies are common (rhabdomyosarcoma, synovial sarcoma).

Around 3,300 people were diagnosed with soft-tissue sarcoma in the UK in 2011.

it:Sarcoma#Sarcoma epitelioide

NIH genetic and rare disease info

Soft tissue sarcoma is a rare disease.

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WikiMD Resources - Soft tissue sarcoma


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