Soft-tissue sarcoma

Soft-tissue sarcoma
Classification and external resources
ICD-9-CM 171
DiseasesDB 31472
MeSH D018204
Undifferentiated soft tissue sarcoma in left lung of young child

A soft-tissue sarcoma is a form of sarcoma that develops in connective tissue,[1] though the term is sometimes applied to elements of the soft tissue that are not currently considered connective tissue.

Risk factors

Most soft-tissue sarcomas are not associated with any known risk factors or identifiable etiology. There are some exceptions:

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.[3] 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 2011.[4]

Symptoms

In their early stages, soft-tissue sarcomas usually do not cause symptoms. 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. If in the abdomen it can cause abdominal pains commonly mistaken for menstrual cramps, indigestion, or cause constipation.

Diagnosis

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

Soft tissue sarcomas commonly originate in the upper body, in the shoulder or upper chest. Some symptoms are uneven posture, pain in the trapezius muscle and cervical inflexibility [difficulty in turning the head].

The most common site to which soft tissue sarcoma spreads is the lungs.

Treatment

In general, treatment for soft-tissue sarcomas depends on the stage of the cancer. The stage of the sarcoma is based on the size and grade of the tumor, and whether the cancer has spread to the lymph nodes or other parts of the body (metastasized). Treatment options for soft-tissue sarcomas include surgery, radiation therapy, and chemotherapy.

A combination of Taxotere and Gemzar could be an effective chemotherapy regimen in patients with advanced soft-tissue sarcoma.[5]

Tables

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

An earlier version of this article was taken from the US National Cancer Center's Cancer Information Service.

Notable patients

References

  1. "soft tissue sarcoma" at Dorland's Medical Dictionary
  2. Dino Samartzis, University of Hong Kong, South China Morning Post, 1 June 2013
  3. Ries LAG, Harkins D, Krapcho M, et al. SEER Cancer Statistics Review, 1975–2003. Bethesda , MD: National Cancer Institute, 2006.
  4. "Soft tissue sarcoma statistics". Cancer Research UK. Retrieved 28 October 2014.
  5. http://professional.cancerconsultants.com/oncology_sarcoma_cancer_news.aspx?id=36670
  6. http://www.wwe.com/inside/the-scars-of-the-superstars/page-13
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