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Encyclopedia > Primitive neuroectodermal tumor
Ewing's sarcoma
ICD-9 170.9
ICD-O: 9260/3
DiseasesDB 4604
MedlinePlus 001302
eMedicine ped/2589
X-Ray of a child with Ewing's sarcoma of the tibia
X-Ray of a child with Ewing's sarcoma of the tibia

Ewing's sarcoma is the common name for primitive neuroectodermal tumor. It is a rare disease in which cancer cells are found in the bone or in soft tissue. The most common areas in which it occurs are the pelvis, the femur, the humerus, and the ribs. James Ewing (1866-1943) first described the tumor, establishing that the disease was separate from lymphoma and other types of cancer known at that time. Ewing's sarcoma occurs most frequently in male teenagers, such as Brandon Russell. Ewing's sarcoma is the result of a translocation between chromosomes 11 and 22, which fuses the EWS gene of chromosome 22 to the FLI1 gene of chromosome 11. The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The Diseases Database is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Download high resolution version (256x686, 8 KB)Child with Ewings sarcoma of the tibia image created by Michael Richardson, M.D. on Oct 25th, 2004 File history Legend: (cur) = this is the current file, (del) = delete this old version, (rev) = revert to this old version. ... Tumor (American English) or tumour (British English) originally means swelling, and is sometimes still used with that meaning. ... When normal cells are damaged beyond repair, they are eliminated by apoptosis. ... Grays illustration of a human femur, a typically recognized bone. ... Biological tissue is any substance made up of cells that perform a similar function within an organism. ... Human male pelvis, viewed from front Human female pelvis, viewed from front The pelvis is the bony structure located at the base of the spine (properly known as the caudal end). ... The femur or thigh bone is the longest, most voluminous. ... The humerus is a long bone in the arm or fore-legs (animals) that runs from the shoulder to the elbow. ... This article is about the bones called ribs. ... James Ewing (December 25, 1866, Pittsburgh — May 16, 1943, New York City) was an American pathologist. ... 1866 (MDCCCLXVI) is a common year starting on Monday of the Gregorian calendar or a common year starting on Wednesday of the 12-day-slower Julian calendar. ... 1943 (MCMXLIII) was a common year starting on Friday (the link is to a full 1943 calendar). ... Lymphoma is a variety of cancer that originates in the lymphatic system. ... A separate article is about the punk band called The Adolescents. ...

Contents


Clinical Findings

Ewing's sarcoma is more common in males and usually presents in childhood or early adulthood, with a peak between 10 and 20 years of age. It is found almost exclusively in caucasians. It can occur anywhere in the body, but most commonly in the pelvis and proximal long tubular bones. The diaphysis of the femur are the most common sites, followed by the tibia and the humerus. Thirty percent are overtly metastatic at presentation. Human male pelvis, viewed from front Human female pelvis, viewed from front The pelvis is the bony structure located at the base of the spine (properly known as the caudal end). ... The femur or thigh bone is the longest, most voluminous. ... Figure 1 : Upper surface of right tibia. ... The humerus is a long bone in the arm or fore-legs (animals) that runs from the shoulder to the elbow. ... Metastasis (Greek: change of the state) is the spread of cancer from its primary site to other places in the body. ...


The most common clinical findings are pain and swelling.


Imaging findings

On conventional radiographs, the most common osseous presentation is a permeative lytic lesion with periosteal reaction. The classic description of lamellated or "onion skin" type periosteal reaction is often associated with this lesion. Plain films add valuable information in the initial evaluation or screening. The wide zone of transition (e.g. permeative) is the most useful plain film characteristic in differention of benign versus aggressive or malignant lytic lesions. Radiography is the creation of images by exposing a photographic film or other image receptor to X-rays. ... The periosteum is an envelope of fibrous connective tissue that is wrapped around the bone in all places except at joints (which are protected by cartilage). ... The periosteum is an envelope of fibrous connective tissue that is wrapped around the bone in all places except at joints (which are protected by cartilage). ...


MRI should be routinely used in the work-up of malignant tumors. MRI will show the full bony and soft tissue extent and relate the tumor to other nearby anatomic structures (e.g. vessels). Gadolinium contrast is not necessary as it does not give additional information over noncontrast studies, though some current researchers argue that dynamic, contrast enhanced MRI may help determine the amount of necrosis within the tumor, thus help in determining response to treatment prior to surgery. The mri are a fictional alien species in the Faded Sun Trilogy of C.J. Cherryh. ... The mri are a fictional alien species in the Faded Sun Trilogy of C.J. Cherryh. ... General Name, Symbol, Number gadolinium, Gd, 64 Chemical series lanthanides Group, Period, Block n/a, 6, f Appearance silvery white Atomic mass 157. ...


CT can also be used to define the extraosseous extent of the tumor, especially in the skull, spine, ribs and pelvis. Both CT and MRI can be used to follow response to radiation and/or chemotherapy. CT apparatus in a hospital Computed axial tomography (CAT), computer-assisted tomography, computed tomography, CT, or body section roentgenography is the process of using digital processing to generate a three-dimensional image of the internals of an object from a large series of two-dimensional X-ray images taken around... CT apparatus in a hospital Computed axial tomography (CAT), computer-assisted tomography, computed tomography, CT, or body section roentgenography is the process of using digital processing to generate a three-dimensional image of the internals of an object from a large series of two-dimensional X-ray images taken around... The mri are a fictional alien species in the Faded Sun Trilogy of C.J. Cherryh. ...


Bone scintigraphy can also be used to follow tumor response to therapy. Nuclear medicine is the branch of medicine that uses unsealed radioactive substances in diagnosis and therapy. ...


Differential diagnosis

Other entities that may have a similar radiologic presentation include osteomyelitis, osteosarcoma (especially telangiectatic osteosarcoma) and eosinophilic granuloma. Soft tissue neoplasms such as malignant fibrous histiocytoma that erode into adjacent bone may also have a similar appearance. Osteosarcoma is a common primary bone cancer. ...


Epidemiology

The frequency in the United States depends on the patient's age, with a rate of 0.3 case per 1,000,000 children in those younger than 3 years of age to as high as 4.6 cases per 1,000,000 in adolescents aged 15-19 years. Internationally the annual incidence rate averages less than 2 cases per 1,000,000 children.[1] In the United Kingdom an average of six children per year are diagnosed, mainly males in early stages of puberty.


Treatment

Because almost all patients with apparently localized disease at diagnosis have occult metastatic disease, multidrug chemotherapy as well as local disease control with surgery and/or radiation is indicated in the treatment of all patients (2). Treatment often consists of adjuvant chemotherapy generally followed by wide or radical excision, and may also include radiotherapy. Complete excision at the time of biopsy may be performed if malignancy is confirmed at that time. Treatment lengths vary depending on location and stage of the disease at diagnosis, however most patients will undergo chemotherapy for 6-12 months and radiation therapy for 5-8 weeks. Chemotherapy is the use of chemical substances to treat disease. ... Chemotherapy is the use of chemical substances to treat disease. ... Radiation therapy (or radiotherapy) is the medical use of ionizing radiation as part of cancer treatment to control malignant cells (not to be confused with radiology, the use of radiation in medical imaging and diagnosis). ...


Prognosis

Staging attempts to distinguish patients with localized from those with metastatic disease. Most commonly, metastases occur in the chest, bone and/or bone marrow. Less common sites include the central nervous system and lymph nodes. Metastasis (Greek: change of the state) is the spread of cancer from its primary site to other places in the body. ... Metastasis (Greek: change of the state) is the spread of cancer from its primary site to other places in the body. ... A diagram showing the CNS: 1. ... Structure of the lymph node. ...


Survival for localized disease is 65-70% when treated with chemotherapy. Survival for metastatic disease is 25-30%. Chemotherapy is the use of chemical substances to treat disease. ... Metastasis (Greek: change of the state) is the spread of cancer from its primary site to other places in the body. ...


References

  1. ^ http://www.emedicine.com/radio/topic275.htm eMedicine article on Ewing's sarcoma accessed April 24, 2006

External links

National Cancer Institute listing for Ewing's sarcoma


Adult Bone Cancer Survivors - Ewing's sarcoma survivors' stories, resources, and support forum


Extraskeletal Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumour


  Results from FactBites:
 
Childhood Supratentorial PNET - National Cancer Institute (780 words)
Childhood supratentorial primitive neuroectodermal tumors and pineoblastoma are tumors in which malignant (cancer) cells form in the tissues of the brain.
The symptoms of childhood supratentorial primitive neuroectodermal tumors and pineoblastoma vary and often depend on the child’s age, where the tumor is located, and the size of the tumor.
Childhood supratentorial primitive neuroectodermal tumors and pineoblastoma are diagnosed and removed in surgery.
Types of Brain Tumors (technical) - Childrens Hospital San Diego (2075 words)
Primitive neuroectodermal tumors (PNETs) of the posterior fossa are traditionally referred to as medulloblastomas.
These tumors are classified based on their histology.  Although nuclear atypia and endothelial hyperplasia may occasionally be seen, these features do not herald a more ominous disease course; in fact, no prognostic information can be determined from any histological features of these tumors.
These tumors are most commonly found in children in the posterior fossa; however, approximately one-third of ependymomas occur in the supratentorial compartment.
  More results at FactBites »

 
 

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