Sarcoma

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WikiDoc Resources for Sarcoma

Articles

Most recent articles on Sarcoma

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Review articles on Sarcoma

Articles on Sarcoma in N Eng J Med, Lancet, BMJ

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Powerpoint slides on Sarcoma

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Clinical Trials

Ongoing Trials on Sarcoma at Clinical Trials.gov

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Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Sarcoma

NICE Guidance on Sarcoma

NHS PRODIGY Guidance

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Books

Books on Sarcoma

News

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Commentary

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Definitions

Definitions of Sarcoma

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Risk calculators and risk factors for Sarcoma

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Symptoms of Sarcoma

Causes & Risk Factors for Sarcoma

Diagnostic studies for Sarcoma

Treatment of Sarcoma

Continuing Medical Education (CME)

CME Programs on Sarcoma

International

Sarcoma en Espanol

Sarcoma en Francais

Business

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Experimental / Informatics

List of terms related to Sarcoma

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). It can be classified based on the tissue involved and the histology of the lesion. Soft tissue sarcoma needs to differentiated from soft tissue benign tumors such as Adenoma, lipoma, and fibroma. The estimated incidence of soft tissue sarcoma worldwide is 1.8 to 5 per 100,000 per year. Soft tissue sarcomas are more commonly found in older patients (>50 years old). Risk factors include radiation exposure, damaged lymphatic system, and inherited conditions. There is insufficient evidence to recommend routine screening for sarcoma. If left untreated, sarcoma can lead to complications of local tissue erosion, compression and invasion. Complications can also include side effects of chemotherapy and radiation therapy. Prognosis depends upon the size and stage of the tumor, age and general health of the patient, and benign/malignant nature of the tumor. Sarcoma can be diagnosed by combination of imaging and biopsy. Symptoms include painless swelling or lump, menstrual irregularities, constipation, and indigestion. Chemotherapy may be used with radiation therapy either before or after surgery to try to shrink the tumor or kill any remaining cancer cells. Surgery is the most common treatment for soft tissue sarcomas. 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 (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. There are no established measures for the primary and secondary prevention of sarcoma.

Classification

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 tumor/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 tumor (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.)

Differentiating Sarcoma from Other Diseases

Soft tissue sarcoma needs to differentiated from soft tissue benign tumors such as:

Epidemiology and Demographics

Incidence

  • The estimated number of new cases of soft tissue sarcoma in the United States is approximately 12,000.[4]

Age

Percent Distribution of Soft Tissue Sarcoma by Histology

Risk Factors

Screening

  • There is insufficient evidence to recommend routine screening for sarcoma.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

  • There are no ECG findings associated with sarcoma.

X-ray

Echocardiography or Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

  • There are no other diagnostic studies associated with sarcoma.

Treatment

Medical Therapy

Surgery

  • Surgery is the most common treatment for soft tissue sarcomas.
  • 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).
  • 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

  • In some cases, it can be used to treat tumors 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.

Primary Prevention

Secondary Prevention

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. 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
  4. American Cancer Society: Cancer Facts and Figures 2014. Atlanta, Ga: American Cancer Society, 2014. Available online . Last accessed May 21, 2014.
  5. 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. doi:10.1093/annonc/mdp415. PMID 19858086.
  6. 6.0 6.1 6.2 6.3 6.4 6.5 "Sarcoma".

External Links

ar:غرن da:Sarkom de:Sarkom fa:سارکوما it:Sarcoma he:סרקומה nl:Sarcoom no:Benvevskreft sv:Sarkom ur:لحمومہ


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