Sarcoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Sabawoon Mirwais, M.B.B.S, M.D.[2]

## Overview[edit | edit source]

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[edit | edit source]

* 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]
* 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, it has risen to 60 - 70%.[3]

### Tables[edit | edit source]

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[edit | edit source]

(ICD-O codes are provided where available.)

* Askin's tumor (8803/3)
* Chondrosarcoma (9220/3-9240/3)
* Ewing's (9260/3) - PNET (9473/3)
* Malignant Hemangioendothelioma (9130/3)
* Malignant Schwannoma (9560/3-9561/3)
* Osteosarcoma (9180/3-9190/3)
* Soft tissue sarcomas, including:
* Alveolar soft part sarcoma (9581/3)
* Angiosarcoma (9120/3)
* Cystosarcoma Phylloides[3]
* Dermatofibrosarcoma (8832/3-8833/3)
* Desmoid Tumor (8821/1-8822/1)
* Desmoplastic small round cell tumor (8806/3)
* Epithelioid Sarcoma (8804/3)
* Extraskeletal chondrosarcoma (9220/3)
* Extraskeletal osteosarcoma (9180/3)
* Fibrosarcoma (8810/3)
* Hemangiopericytoma (9150)
* Hemangiosarcoma (9120/3)
* Kaposi's sarcoma (9140/3)
* Leiomyosarcoma (8890/3-8896/3)
* Liposarcoma (8850/3-8858/3)
* Lymphangiosarcoma (9170-9175)
* Lymphosarcoma
* Malignant fibrous histiocytoma (8830/3)
* Neurofibrosarcoma (9540/3)
* Rhabdomyosarcoma (8900-8920)
* Synovial sarcoma (9040/3-9043/3)

## Differentiating Sarcoma from Other Diseases[edit | edit source]

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

* Adenoma

* Lipoma

* Fibroma

## Epidemiology and Demographics[edit | edit source]

### Incidence[edit | edit source]

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

* The estimated incidence of soft tissue sarcoma worldwide is 1.8 to 5 per 100,000 per year.[5]

### Age[edit | edit source]

* Soft tissue sarcomas are more commonly found in older patients (>50 years old).
* Certain histological sub-types are more common in children and adolescents under age 20 (rhabdomyosarcoma).

### Percent Distribution of Soft Tissue Sarcoma by Histology[edit | edit source]

* 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's sarcoma: 0.8%
* Malignant peripheral nerve sheath tumor: 1.6%
* Malignant neurilemmoma: 0.2%
* Neuroblastoma: 0.6%
* Synovial sarcoma: 4.8%

## Risk Factors[edit | edit source]

* Radiation exposure: Clinical studies suggest that patients with other kind of cancers such as lymphoma and breast cancer may develop sarcomas from radiation therapy. The sarcoma often develops in the area of the body that had been treated with radiation.

* Damaged lymphatic system: Clinical observations demonstrate that lymphangiosarcoma is a very rare complication of chronic lymphedema that is the result of damaged lymphatic system.

* Inherited conditions: Some inherited conditions may increase the risk of developing soft tissue sarcomas, such as neurofibromatosis, Gardner syndrome, Li-Fraumeni syndrome, Retinoblastoma, and Werner syndrome.

## Screening[edit | edit source]

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

## Natural History, Complications, and Prognosis[edit | edit source]

* If left untreated, sarcoma can lead to complications of local tissue erosion, compression and invasion.
* It can also lead to metastasis to distant sites.
* Complications can also include side effects of chemotherapy and radiation therapy.
* Surgical wound after resection can also complicate a sarcoma.
* 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 nature.
* The patient’s general health.
* Whether the sarcoma has just been diagnosed or has recurred.

## Diagnosis[edit | edit source]

### Diagnostic Study of Choice[edit | edit source]

* Sarcoma can be diagnosed by combination of imaging and biopsy.

### History and Symptoms[edit | edit source]

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

### Physical Examination[edit | edit source]

* Patients with sarcoma usually appear normal.
* Common physical examination findings include painless lump or swelling.

### Laboratory Findings[edit | edit source]

* There are no diagnostic laboratory findings associated with sarcoma.

### Electrocardiogram[edit | edit source]

* There are no ECG findings associated with sarcoma.

### X-ray[edit | edit source]

* X-ray can be the first investigation ordered to evaluate a suspected sarcoma.[6]
* Chest x-ray can help rule in/out metastasis to the lungs.[6]

### Echocardiography or Ultrasound[edit | edit source]

* Ultrasound can help determine if a suspected sarcoma is fluid filled.[6]
* Ultrasound is usually performed before biopsy.

### CT scan[edit | edit source]

* CT scan can be used as a guiding tool in taking biopsy.[6]
* It can also help in making a diagnosis of the lesion itself.

### MRI[edit | edit source]

* MRI determines the extent of tumor invasion.[6]
* It provides a detailed picture of the lesion.[6]
* It can also help in determining the tissue of origin.

### Other Imaging Findings[edit | edit source]

* PET (positron emission tomography) scan can be used to determine the spread of sarcoma.

### Other Diagnostic Studies[edit | edit source]

* There are no other diagnostic studies associated with sarcoma.

## Treatment[edit | edit source]

### Medical Therapy[edit | edit source]

* 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, the effects of chemotherapy on 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.
* 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.

### Surgery[edit | edit source]

* 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[edit | edit source]

* 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 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[edit | edit source]

* There are no established measures for the primary prevention of sarcoma.

### Secondary Prevention[edit | edit source]

* There are no established measures for the secondary prevention of sarcoma.

## References[edit | edit source]

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.CS1 maint: Multiple names: authors list (link)
6. ↑ 6.0 6.1 6.2 6.3 6.4 6.5 "Sarcoma".

## External Links[edit | edit source]

* Template:DMOZ

* v
* t
* e

Soft tissue tumors and sarcomas (ICD-O 8800-9349)

Not otherwise specified (8800-8809)|

Soft tissue sarcoma \- Desmoplastic small round cell tumor

Fibromatous (8810-8839)|

Fibroma/fibrosarcoma \- Malignant fibrous histiocytoma \- Dermatofibroma/dermatofibrosarcoma \- Dermatofibrosarcoma protuberans

Myxomatous (8840-8849)|

Myxoma \- Ossifying fibromyxoid tumour

Lipomatous (8850-8889)|

Lipoma/liposarcoma (Angiomyolipoma)

Myomatous (8890-8929)|

Leiomyoma/leiomyosarcoma \- Myoma \- Rhabdomyoma/rhabdomyosarcoma \- Sarcoma botryoides

Complex Mixed And Stromal (8930-8999)|

Adenomyoma \- Pleomorphic adenoma \- Mixed Mullerian tumor \- Mesoblastic nephroma \- Wilms' tumor \- Rhabdoid tumour \- Clear cell sarcoma of the kidney \- Hepatoblastoma \- Carcinosarcoma

Fibroepithelial (9000-9039)|

Brenner tumour \- Fibroadenoma \- Phyllodes tumor

Synovial-like (9040-9049)|

Synovial sarcoma \- Clear cell sarcoma, NOS

Mesothelial (9050-9059)|

Mesothelioma \- Adenomatoid tumor

Germ cell tumors (9060-9119)|

germinomatous germ cell tumors: Dysgerminoma \- Germinoma \- Seminoma
nongerminomatous germ cell tumors: Embryonal carcinoma \- Endodermal sinus tumor / Yolk sac tumor \- Teratoma/Fetus in fetu / Dermoid cyst/Struma ovarii \- Gestational trophoblastic disease (Hydatidiform mole) - Choriocarcinoma \- Polyembryoma \- Gonadoblastoma

Vascular (9120-9179)|

blood vessels: Hemangioma/hemangiosarcoma \- Angioma/angiosarcoma \- Blue rubber bleb nevus syndrome \- Hemangioendothelioma \- Kaposi's sarcoma \- Hemangiopericytoma
lymphatic vessels: Lymphangioma/lymphangiosarcoma \- Lymphangioleiomyomatosis

Osseous and
chondromatous (9180-9349)|

Osteoma/osteosarcoma \- Osteochondroma \- Chondroma/enchondroma/chondrosarcoma \- Chondroblastoma \- Giant cell tumor of bone \- Ewing's sarcoma \- Chordoma

teeth/odontogenic: (Cementoblastoma, Cementoma, Odontoma, Adenomatoid odontogenic tumor, Ameloblastoma)

Adamantinoma

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

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