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. More research is being done on possibility on females. 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. 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. ... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... 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 Disease Bold textDatabase 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. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... X-Ray of a child with Ewings sarcoma of the tibia Ewings sarcoma is the common name for primitive neuroectodermal tumor. ... Cancer is a class of diseases or disorders characterized by uncontrolled division of cells and the ability of these to spread, either by direct growth into adjacent tissue through invasion, or by implantation into distant sites by metastasis (where cancer cells are transported through the bloodstream or lymphatic system). ... This article is about the skeletal organs. ... Biological tissue is a collection of interconnected cells that perform a similar function within an organism. ... The pelvis (pl. ... The femur or thigh bone is the longest, most voluminous, and strongest bone of the mammalian bodies. ... 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. ... Year 1943 (MCMXLIII) was a common year starting on Friday (the link will display full 1943 calendar) of the Gregorian calendar. ... This article is about lymphoma in humans. ... Teenagers is the fourth single and eleventh track from My Chemical Romances third studio album, The Black Parade. ... Chromosome 22 is one of the 23 pairs of chromosomes in humans. ... Chromosome 11 is one of the 23 pairs of chromosomes in humans. ...

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 can occur anywhere in the body, but most commonly in the pelvis and proximal long tubular bones. The diaphyses of the femur are the most common sites, followed by the tibia and the humerus. Thirty percent are overtly metastatic at presentation. The pelvis (pl. ... The femur or thigh bone is the longest, most voluminous, and strongest bone of the mammalian bodies. ... This article is about the vertebrate bone. ... The humerus is a long bone in the arm or fore-legs (animals) that runs from the shoulder to the elbow. ... For the musical composition, see Metastasis (Xenakis composition). ...

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. A radiograph of a right elbow-joint Radiography is the use of certain types of electromagnetic radiation—usually ionizing—to view objects. ... 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. MRI redirects here. ... General Name, Symbol, Number gadolinium, Gd, 64 Chemical series lanthanides Group, Period, Block n/a, 6, f Appearance silvery white Standard atomic weight 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... Chemotherapy, in its most general sense, refers to treatment of disease by chemicals that kill cells, specifically those of micro-organisms or cancer. ...

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 clinical 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. Osteomyelitis is an infection of bone, usually caused by pyogenic bacteria or mycobacteria. ... Osteosarcoma is the most common type of malignant bone cancer, accounting for 35% of primary bone malignancies. ...

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. Due to the prevalence of diagnosis during teenage years, there may possibly be a link between the onset of puberty and the early stages of this disease, although no research is currently being conducted to confirm this theory.

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 neo-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. Radical chemotherapy may be as short as 6 treatments at 3 week cycles, however most patients will undergo chemotherapy for 6-12 months and radiation therapy for 5-8 weeks. Chemotherapy, in its most general sense, refers to treatment of disease by chemicals that kill cells, specifically those of micro-organisms or cancer. ... Chemotherapy, in its most general sense, refers to treatment of disease by chemicals that kill cells, specifically those of micro-organisms or cancer. ... 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. For the musical composition, see Metastasis (Xenakis composition). ... For the musical composition, see Metastasis (Xenakis composition). ... A diagram showing the CNS: 1. ... Lymph nodes are components of the lymphatic system. ...

Survival for localized disease is 65-70% when treated with chemotherapy. Long term survival for metastatic disease can be less than 10% but some sources state it is 25-30%.^{[citation needed]}... Chemotherapy, in its most general sense, refers to treatment of disease by chemicals that kill cells, specifically those of micro-organisms or cancer. ... For the musical composition, see Metastasis (Xenakis composition). ...

References

External links

• National Cancer Institute listing for Ewing's sarcoma
• Ewing's Research Foundation
• Ewings Sarcoma Family of Tumors (ESFT) by R. Lor Randall, MD
• Adult Bone Cancer Survivors - Ewing's sarcoma survivors' stories, resources, and support forum
• Liddy Shriver Sarcoma Initiative
• Natural History of Ewings Sarcoma