Sarcoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

A sarcoma (from the Greek 'sarx' meaning "flesh") is a cancer of the connective or supportive tissue (bone, cartilage, fat, muscle, blood vessels) and soft tissue. This is in contrast to carcinomas, which are of epithelial origin (breast, colon, pancreas, and others).

Classification

Sarcomas are given a number of different names, based on the type of tissue from which they arise. For example, osteosarcoma arises from bone, chondrosarcoma arises from cartilage, and leiomyosarcoma arises from smooth muscle. Sarcomas strike people in all age ranges, but they are very rare, accounting for only 1% of all cases of cancer.[1] Soft tissue sarcomas, such as leiomyosarcoma, chondrosarcoma, and gastrointestinal stromal tumor (GIST), are more common in adults than in children. GIST is the most common form of sarcoma, with approximately 3000-3500 cases per year in the United States.[2] This should be compared with breast cancer, with approximately 200,000 cases per year in North America.[3] Bone sarcomas, such as osteosarcoma and Ewing's sarcoma, are more common in children than in adults. These tumors most commonly strike adolescents and young adults between the ages of 12 and 25. In addition to being named based on the tissue of origin, sarcomas are also assigned a grade, such as low grade or high grade. Low grade sarcomas are usually treated surgically, although sometimes radiation therapy or chemotherapy are used. High grade sarcomas are more frequently treated with chemotherapy. Since these tumors are more likely to undergo metastasis (spreading to distant sites), these tumors are treated more aggressively. Childhood sarcomas are almost always treated with a combination of surgery and chemotherapy, and radiation is frequently used as well. The recognition that childhood sarcomas are sensitive to chemotherapy has dramatically improved the survival of patients. For example, in the era before chemotherapy, long term survival for patients with localized osteosarcoma was only approximately 20%, but now has risen to 60-70%.[4]

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

Types of sarcoma

(ICD-O codes are provided where available.)

Causes

Differential Diagnosis

Soft tissue sarcoma needs to differentiate from soft tissue benige tumors such as:

Drug Side Effect

Epidemiology

Incidence

  • The estimated number of new cases of soft tissue sarcoma in the United States is approximately 12,000.[5]
  • The estimated incidence of soft tissue sarcoma worldwide is 1.8 to 5 per 100,000 per year.[6]

Age

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).

Percent Distribution of Soft Tissue Sarcoma by Histology

  • Fibrosarcoma: 6.9%
  • Infantile fibrosarcoma: 0.2%
  • Fibrous histiocytoma, malignant: 9.2%
  • Dermatofibrosarcoma: 3.6%
  • Liposarcoma: 17.1%
  • Leiomyosarcoma: 13.2%
  • Rhabdomyosarcoma: 3.1%
  • Embryonal rhabdomyosarcoma: 1.3%
  • Hemangiosarcoma: 3.7%
  • Hemangiopericytoma, malignant: 0.5%
  • Kaposi sarcoma: 0.8%
  • Malignant peripheral nerve sheath tumor: 1.6%
  • Malignant neurilemmoma: 0.2%
  • Neuroblastoma: 0.6%
  • Synovial sarcoma: 4.8%

Risk Factor

  • Radiation exposure: Clinical stidies suggest that patients with other kind of cancers such as lymphoma and breast cancer may develop sarcomas from radiation therapy. The sarcoma often happens in the area of the body that had been treated with radiation.
  • Damaged lymph system: Clinical observations demonstrate lymphangiosarcoma is a very rare complication of chronic lymphedema that is the result of damaged lymph system.

Prognosis

The prognosis of soft tissue sarcoma is poor and it depends on the following:

  • Whether or not the tumor can be removed by surgery
  • The stage of the sarcoma:
    • The size of the tumor.
    • Benign or malignant.
  • The patient’s general health.
  • Whether the sarcoma has just been diagnosed or has recurred.

Symptoms

  • Painless lump or swelling
  • Pain or soreness
  • Menstrual cramps
  • Indigestion
  • Constipation

Medical Therapy

Chemotherapy may be used with radiation therapy either before or after surgery to try to shrink the tumor or kill any remaining cancer cells. In general, chemotherapy effects for soft tissue sarcoma have had little impact as opposed to other cancers. If the cancer has spread to other areas of the body, chemotherapy may be used to shrink tumors and reduce the pain and discomfort they cause, but is unlikely to eradicate the disease. The use of chemotherapy to prevent the spread of soft tissue sarcomas has not been proven to be effective. Patients with soft tissue sarcomas usually receive chemotherapy intravenously (injected into a blood vessel).

Surgery

Surgery is the most common treatment for soft tissue sarcomas. If possible, the doctor will remove the cancer and a safe margin of the healthy tissue around it. Depending on the size and location of the sarcoma, it may occasionally be necessary to remove all or part of an arm or leg (amputation). However, the need for amputation rarely arises; no more than 10 % to 15 % of individuals with sarcoma undergo amputation. In most cases, limb-sparing surgery is an option to avoid amputating the arm or leg. It is important to obtain a margin free of tumor to decrease the likelihood of local recurrence and give the best chance for eradication of the tumor.

Radiation Therapy

Radiation therapy (treatment with x-rays or radioactive implants) may be used either before surgery to shrink tumors or after surgery to kill any cancer cells that may have been left behind. In some cases, it can be used to treat tumours that cannot be surgically removed. In multiple studies, radiation therapy has been found to improve the rate of local control, but has not had any influence on overall survival.

References

  1. Borden EC, Baker LH, Bell RS, Bramwell V, Demetri GD, Eisenberg BL, Fletcher CD, Fletcher JA, Ladanyi M, Meltzer P, O'Sullivan B, Parkinson DR, Pisters PW, Saxman S, Singer S, Sundaram M, van Oosterom AT, Verweij J, Waalen J, Weiss SW, Brennan MF. Soft tissue sarcomas of adults: state of the translational science.Clin Cancer Res. 2003 Jun;9(6):1941-56. Review. PMID 12796356
  2. Tran T, Davila JA, El-Serag HB. The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1,458 cases from 1992 to 2000. Am J Gastroenterol 2005;100:162-8. PMID 15654796
  3. Smigal C, Jemal A, Ward E, Cokkinides V, Smith R, Howe HL, Thun M. Trends in breast cancer by race and ethnicity: update 2006. CA Cancer J Clin 2006;56:168-83. PMID 16737949
  4. Longhi A, Errani C, De Paolis M, Mercuri M, Bacci G. Primary bone osteosarcoma in the pediatric age: state of the art. Cancer Treat Rev. 2006;32:423-36. PMID 16860938
  5. American Cancer Society: Cancer Facts and Figures 2014. Atlanta, Ga: American Cancer Society, 2014. Available online . Last accessed May 21, 2014.
  6. Wibmer C, Leithner A, Zielonke N, Sperl M, Windhager R (2010). "Increasing incidence rates of soft tissue sarcomas? A population-based epidemiologic study and literature review.". Ann Oncol. 21 (5): 1106–11. PMID 19858086. doi:10.1093/annonc/mdp415. 

External Links

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