Ewing sarcoma

(medicine) A primary malignant tumor of bone, usually arising as a central tumor in long bone.

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Key Terms: Biopsy, Chemotherapy, Metastasize, Oncologist, Pathologist, Radiation therapy.

Definition

Ewing's sarcoma is a cancer that affects children, teens, and less often, young adults. It begins in developing bone cells. Ewing's sarcoma cells grow uncontrollably and form masses or lumps called tumors. They can start in any bone in the body but about half of all cases involve flat bones such as the pelvic bones and the long bones in the leg—the tibia, fibula, and femur.

Description

Ewing's sarcoma is the second most common bone tumor among children and teens. It accounts for about 1% of all childhood cancers. This cancer is named for James Ewing, the researcher who first described the tumor in 1921. There are some rare cases of Ewing's sarcoma that do not begin in bones. These tumors are thought to start in nerve or other soft tissues.

Demographics

Ewing's sarcoma occurs most frequently in children ages 11 to 15 years old. Slightly more males than females develop Ewing's sarcomas, and like osteosarcoma, the most common bone cancer found in children, it is more often diagnosed in taller teens. The disease is rarely diagnosed in children younger than 5 or adults older than 30. It affects primarily Caucasians, and rarely occurs in African Americans and native Chinese.

Causes and Symptoms

The causes of Ewing's sarcoma are not known. It is possible that certain inherited conditions increase the risk of developing this cancer.

About two–thirds of patients with Ewing's sarcoma have a painful swelling or lump that can be felt in the affected bone. Along with tenderness, the area of swelling may be hot. The symptoms depend on the site of the tumor and whether it has spread. For example, a tumor on a rib may cause painful breathing. When Ewing's sarcoma has spread, patients may have other symptoms such as fatigue, weight loss, and fever.

Diagnosis

Most patients who have Ewing's sarcomas go to the doctor because they have discovered a lump or mass or swelling on or near a bone. Others have symptoms related to the part of the body that is affected by the tumor, such as pressure on the bladder from a tumor on a pelvic bone.

The patient's doctor will take a detailed medical history to find out about the symptoms. The history is followed by a complete physical examination with special attention to the suspicious symptom or body part.

Depending on the location of the tumor (mass or lump), the doctor will order imaging studies such as x ray, computed tomography (CT) scans and magnetic resonance imaging (MRI) to help determine the size, shape and exact location of the tumor. The doctor will also order a chest x ray to find out if the tumor has spread to the lungs, and bone scans to determine if the tumor has spread to bones. Blood tests will be done and an examination of the bone marrow will be performed to see if the marrow is involved.

A biopsy of the tumor is necessary to make a diagnosis of Ewing's sarcoma. During a biopsy, some tissue from the tumor is removed. The tissue sample is examined by a pathologist, a doctor who specializes in the study of diseased tissue.

Types of Biopsy

The type of biopsy done depends on the location of the tumor. For some small tumors, such as those on the arm or leg, the doctor may perform an excisional biopsy, removing the entire tumor and a margin of surrounding normal tissue. Most often, the doctor will perform an incisional biopsy, a procedure that involves cutting out only a piece of the tumor. This biopsy provides a core of tissue from the tumor that is used to confirm the diagnosis of Ewing's sarcoma.

Treatment Team

Like other cancer patients, teens and young adults with Ewing's sarcoma are usually cared for by a multi-disciplinary team of health professionals. The patient's pediatrician, family physician, or primary care doctor may refer the patient to other physician specialists, such as surgeons and oncologists (doctors who specialize in cancer medicine). Radiologic technicians perform x ray, CT and MRI scans and nurses and laboratory technicians may obtain samples of blood, urine and other laboratory tests.

Before and after any surgical procedures, including biopsies, specially trained nurses will explain the procedures and help to prepare patients and families. Depending on the tumor location and treatment plan, patients may also benefit from rehabilitation therapy with physical therapists and nutritional counseling from dieticians.

Clinical Staging, Treatments, and Prognosis

Staging

The purpose of staging a tumor is to determine how far it has advanced. This is important because treatment varies depending on the stage. Generally, stage is determined by the size of the tumor, whether the tumor has spread to nearby lymph nodes, whether the tumor has spread elsewhere in the body, and what the cells look like under the microscope.

There is no commonly accepted system for staging Ewing's sarcomas. As is the case with other cancers, patients with metastases (spread) tend to have worse outlooks than patients whose tumors have not spread.

Between one quarter to one third of patients with Ewing's sarcoma have metastases when they are first diagnosed. Patients with tumors closer to the trunk of the body or in the pelvic bones are more likely to have metastases than patients with tumors located on the lower leg or foot. The most common sites for spread of Ewing's sarcoma are to the lungs or bones.

Treatment

Treatment for Ewing's sarcoma varies depending on the location of the tumor, its size and grade, and the extent of its spread. For most patients, the goals of treatment are to remove or control the tumor and combat the spread of the cancer.

'Generally, when removing the tumor will not sharply reduce function, Ewing's sarcoma tumors are surgically removed. The goal of removing as much tumor as possible is to reduce the amount of radiation needed after surgery. The part of the body where the tumor was removed is treated with radiation to destroy remaining tumor cells. Most patients also receive chemotherapy, powerful anti–cancer drugs to destroy remaining cancer cells.

When the disease has spread throughout the body, there may be no benefit from surgical removal of the tumor. These patients, who have widespread metastases, are treated with radiation and chemotherapy.

Side Effects

The surgical treatment of Ewing's sarcoma carries risks related to the surgical site, such as loss of function resulting from loss of a long bone in the leg. There also are the medical risks associated with any surgical procedure, such as reactions to general anesthesia or infection after surgery.

The side effects of radiation therapy depend on the site being radiated. Radiation therapy can produce side effects such as fatigue, skin rashes, nausea and diarrhea. Most of the side effects lessen or disappear completely after the radiation therapy has been completed.

The side effects of chemotherapy vary depending on the medication, or combination of anticancer drugs, used. Chemotherapy drugs often given to combat Ewing's sarcoma are cyclophosphamide, doxorubicin, vincristine, and dactinomycin.

For patients with widespread disease, chemotherapy may be given along with bone marrow transplant and radiation to the entire body. Nausea and vomiting, anemia, lower resistance to infection, and hair loss (alopecia) are common side effects of chemotherapy. Medication may be given to reduce the unpleasant side effects of chemotherapy.

Alternative and Complementary Therapies

Many patients find that alternative and complementary therapies help to reduce the stress associated with illness, improve immune function and feel better. While there is no evidence that these therapies specifically combat disease, activities such as biofeedback, relaxation, therapeutic touch, massage therapy and guided imagery have been reported to enhance well–being.

Prognosis

The outlook for patients with Ewing's sarcoma varies, depending on the size and volume of the tumor, its stage, and the extent of its spread. Patients with large tumors do not fare as well as those with smaller tumors. Patients with tumors in certain sites, such as the bones of the pelvis and spinal column also seem to have poor outlooks because by the time these tumors are discovered they have already spread.

Ewing's sarcoma may spread locally to areas near the tumor and it can spread to nearby lymph glands. To spread to distant parts of the body, the cancer cells travel in the blood, bone marrow or through the lymph glands. In general, tumors that have spread widely throughout the body are not associated with favorable survival rates.

Nearly three quarters of patients diagnosed before the cancer has spread are disease free for five years after treatment. Patients with tumors that do not respond to treatment and those who suffer recurrences have poor outlooks for long–term survival.

Coping With Cancer Treatment

Teens undergoing cancer treatment have special needs. The diagnosis of a life–threatening illness, surgery and radiation or chemotherapy may cause fear, anxiety, depression and loss of self–esteem. Disruption of normal routines and discomfort from diagnostic tests and treatment may also cause anxiety. There are additional social problems including making up missed school work, explaining the illness and treatment to friends, and coping with physical limitations or disability.

Teens with serious illnesses and disabilities face emotional conflicts and psychological challenges. One conflict is between the teen's growing desire for independence and the reality of dependence on others for the activities of daily living. It is important for teens to be fully informed about their disease and treatment plan and involved in treatment decision making. Many teens benefit from continuing contact with friends, classmates, teachers, and family during hospital stays and recovery at home.

Depression, emotional distress, and anxiety associated with the disease and its treatment may respond to counseling from a mental health professional. Individual and group therapy often help teens and young adults to reveal and express their feelings about illness and treatment. Many cancer patients and their families find participation in mutual aid and group support programs help to relieve feelings of isolation and loneliness. By sharing problems with others who have lived through similar difficulties patients and families can exchange ideas and coping strategies.

Clinical Trials

More than 25 clinical studies were underway during 2001. For example, at John Hopkins Oncology Center and other cancer centers across the United States, patients with recurring or widespread Ewing's sarcoma were being given chemotherapy to stop tumor cells from dividing as well as stem cells (bone marrow transplantation) to replace immune cells killed by chemotherapy.

Other clinical trials compare different combinations of drugs used for chemotherapy or combinations of chemotherapy and radiation to find out which combination is more effective. For example, in one study, patients with Ewing's sarcoma were randomly assigned to two different treatment groups. One group received chemotherapy followed by surgery and radiation therapy. The other group received radiation therapy during chemotherapy.

Other types of clinical research study individuals and families at high risk of cancer to help identify cancer genes. To learn more about clinical trials visit the National Cancer Institute (NCI) web site at http://cancertrials.nci.nih.gov or the Pediatric Oncology Branch of the National Cancer Institute web site at http://www.dcs.nci.nih.gov/pedonc/

Prevention

Since the causes of Ewing's sarcoma are not known, there are no recommendations about how to prevent its development. Among families with an inherited tendency to develop soft tissue sarcomas, careful monitoring may help to ensure early diagnosis and treatment of the disease.

Questions to Ask the Doctor

• How big is the tumor?
• Has the Ewing's sarcoma spread?
• What are the recommended treatments?
• What are the side effects of the recommended treatments?
• Is treatment expected to cure the disease or only to prolong life?

Special Concerns

Ewing's sarcoma, like other cancer diagnoses, may produce a range of emotional reactions in patients and families. Education, counseling and participation in group support programs can help to reduce feelings of guilt, fear, anxiety and hopelessness. For many parents suffering from spiritual distress, visits with clergy members and participation in organized prayer may offer comfort.

Resources

Books

Pelletier, Kenneth R. The Best of Alternative Medicine. New York: Simon & Schuster, 2000.

Periodicals

Arndt, Carola A.R., and William M. Crist. "Medical Progress: Common Musculoskeletal Tumors of Childhood and Adolescence." New England Journal of Medicine 29 (July 1999): 342–352.

Organizations

American Cancer Society. 1599 Clifton Road, N.E., Atlanta, GA 30329. (800)227–2345.

Cancer Research Institute. 681 Fifth Avenue, New York, NY 10022. (800)992–2623.

National Cancer Institute Clinical Cancer Trials. .

National Cancer Institute (National Institutes of Health). 9000 Rockville Pike, Bethesda, MD 20892. (800)422–6237.

The Pediatric Oncology Branch of the National Cancer Institute. (877) 624–4878 or (301)496–4256. .

—Barbara Wexler, M.P.H.

This article uses bare URLs for citations. Please consider adding full citations so that the article remains verifiable. Several templates and the Reflinks tool are available to assist in formatting. (Reflinks documentation) (September 2011)

Ewing sarcoma
Classification and external resources
Micrograph of metastatic Ewing sarcoma (right of image) in normal lung (left of image). PAS stain.
ICD-9	170.9
ICD-O:	M9260/3
OMIM	133450
DiseasesDB	4604
MedlinePlus	001302
eMedicine	ped/2589
MeSH	D012512

Ewing sarcoma is a malignant round-cell tumour. 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, the ribs and clavicle.

Because a common genetic locus is responsible for a large percentage of Ewing sarcoma and primitive neuroectodermal tumors, these are sometimes grouped together in a category known as the Ewing family of tumors.^[1] The diseases are, however, considered to be different: peripheral primitive neuroectodermal tumours are generally not associated with bones, while Ewing sarcomas are most commonly related to bone.

Ewing sarcoma occurs most frequently in teenagers, with a male/female ratio of 1.6:1.^[2]

Although usually classified as a bone tumour, Ewing sarcoma can have characteristics of both mesodermal and ectodermal origin, making it difficult to classify.^[3]

James Ewing (1866–1943) first described the tumour, establishing that the disease was separate from lymphoma and other types of cancer known at that time.^[4][5]

Contents 1 Causes 2 Symptoms 3 Clinical findings 4 Imaging findings 5 Clinical differential diagnosis 6 Diagnosis 7 Epidemiology 8 Treatment 8.1 Fertility preservation 9 Prognosis 10 Research, Information and Support 11 References 12 External links

Causes

Genetic exchange between chromosomes can cause cells to become cancerous. Ewing 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.

EWS/FLI functions as the master regulator.^[6]

Other translocations are at t(21;22)^[7] and t(7;22).^[8]

Symptoms

According to The Bone Cancer Research Trust (BCRT) the most common symptoms reported are: Localised pain: bone pain; may come and go and vary in its intensity. Swelling, this can be seen if it is on a bone near the surface of the body but in other places, like on the pelvis, it may not be visible.^[9]

Clinical findings

Distribution of Ewing sarcoma. Most frequent locations are the large long bones and the pelvis.

Ewing 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, especially around the growth plates. The diaphyses of the femur are the most common sites, followed by the tibia and the humerus. Thirty percent are overtly metastatic at presentation. Patients usually experience extreme bone pain.

It is positive for CD99 and negative for CD45.^[10]

Imaging findings

X-Ray of a child with Ewing sarcoma of the tibia.

Magnetic resonance imaging slice showing Ewing's sarcoma of the left hip (white area shown right).

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 differentiation of benign versus aggressive or malignant lytic lesions.

MRI should be routinely used in the work-up of malignant tumours. MRI will show the full bony and soft tissue extent and relate the tumour 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 tumour, thus help in determining response to treatment prior to surgery.

CT can also be used to define the extraosseous extent of the tumour, especially in the skull, spine, ribs and pelvis. Both CT and MRI can be used to follow response to radiation and/or chemotherapy.

Bone scintigraphy can also be used to follow tumour response to therapy.

In the group of malignant small round cell tumours which include Ewing's sarcoma, bone lymphoma and small cell osteosarcoma, the cortex may appear almost normal radiographically, while there is permeative growth throughout the Haversian channels. These tumours may be accompanied by a large soft tissue mass while there is almost no visible bone destruction. The radiographs frequently do not shown any signs of cortical destruction.

Radiographically Ewing's Sarcoma presents as "Moth-eaten" destructive radiolucencies of the medulla and erosion of the cortex with expansion.

Clinical differential diagnosis

This section requires expansion.

Other entities that may have a similar clinical presentation include osteomyelitis, osteosarcoma (especially telangiectatic osteosarcoma) and eosinophilic granuloma. Soft tissue neoplasms such as pleomorphic undifferentiated sarcoma (malignant fibrous histiocytoma) that erode into adjacent bone may also have a similar appearance.

Diagnosis

Micrograph of a metastatic Ewing sarcoma with the characteristic cytoplasmic clearing on H&E staining, which was showing to be PAS positive.

The definitive diagnosis is based on histomorphologic findings, immunohistochemistry and molecular pathology.

Ewing sarcoma is a small round cell tumor, that typically has a clear cytoplasm on H&E staining, due to glycogen. The presence of the glycogen can be demonstrated with positive PAS staining and negative PAS diastase staining. The characteristic immunostain is CD99 which diffusely marks the cell membrane. Morphologic and immunohistochemical findings are corroborated with an associated chromosomal translocation, of which there are several. The most common translocation, present in approximately 90% of Ewing sarcoma cases, is t(11;22)(q24;q12).^[11][12]

The pathologic differential diagnosis is the grouping of Small, round, blue cell tumours, which includes lymphoma, alveolar rhabdomyosarcoma and desmoplastic small round cell tumor, among others.

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.^[13] 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 hypothesis.

Treatment

Because almost all patients with apparently localized disease at diagnosis have occult metastatic disease, multidrug chemotherapy (often including ifosfamide and etoposide)^[14] as well as local disease control with surgery and/or radiation is indicated in the treatment of all patients.^[15]

Treatment often consists of neo-adjuvant chemotherapy generally followed by a limb salvage or an amputation and may also include radiotherapy. Complete excision at the time of biopsy may be performed if malignancy is confirmed at the time it is examined. 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.

Antisense oligodeoxynucleotides have been proposed as possible treatment by down-regulating the expression of the oncogenic fusion protein associated with the development of Ewing sarcoma resulting from the EWS-ETS gene translocation.^[16][17] In addition, the synthetic retinoid derivative fenretinide (4-hydroxy(phenyl)retinamide) has been reported to induce high levels of cell death in Ewing sarcoma cell lines in vitro and to delay growth of Ewing sarcoma xenografts in vivo mouse models.^[18][19]

Fertility preservation

In women, chemotherapy may damage the ovaries and cause infertility. To avail for future pregnancies, the woman may preserve oocytes or ovarian tissue by oocyte cryopreservation or ovarian tissue cryopreservation prior to starting chemotherapy. However, the latter may reseed the cancer upon reinsertion of the ovarian tissue.^[20] If it is performed, the ovarian tissue should be examined for traces of malignancy at both the pathological and molecular levels prior to the grafting of the cryopreserved tissue.^[20]

Prognosis

Staging attempts to distinguish patients with localized from those with metastatic disease.^[21] Most commonly, metastases occur in the chest, bone and/or bone marrow. Less common sites include the central nervous system and lymph nodes.

Five-year survival for localized disease is 70% to 80% when treated with chemotherapy.^[22] Long term survival for metastatic disease can be less than 10% but some sources state it is 25-30%.^[23]

Research, Information and Support

In the UK and Ireland The Bone Cancer Research Trust (BCRT) funds research and provides information on Ewing sarcoma and other bone cancers. This includes information for teenagers who have this condition.

References

20. Bone Tumors - Differential diagnosis. Henk Jan van der Woude and Robin Smithuis.Radiology department of the Onze Lieve Vrouwe Gasthuis, Amsterdam and the Rijnland hospital,Leiderdorp,the Netherlands.

1. ^ Iwamoto Y (February 2007). "Diagnosis and treatment of Ewing sarcoma". Jpn. J. Clin. Oncol. 37 (2): 79–89. doi:10.1093/jjco/hyl142. PMID 17272319. http://jjco.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=17272319. 
2. ^ Burt M, Karpeh M, Ukoha O et al. (January 1993). "Medical tumours of the chest wall. Solitary plasmacytoma and Ewing sarcoma". J. Thorac. Cardiovasc. Surg. 105 (1): 89–96. PMID 8419714. http://jtcs.ctsnetjournals.org/cgi/content/abstract/105/1/89. 
3. ^ Longtin R (November 2003). "Ewing sarcoma: a miracle drug waiting to happen?". J. Natl. Cancer Inst. 95 (21): 1574–6. PMID 14600088. http://jnci.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=14600088. 
4. ^ synd/2367 at Who Named It?
5. ^ Ewing, J. (1921). "Diffuse endothelioma of bone". Proceedings of the New York Pathological Society 21: 17–24. 
6. ^ Owen LA, Kowalewski AA, Lessnick SL (2008). Wu, Xiaolin. ed. "EWS/FLI mediates transcriptional repression via NKX2.2 during oncogenic transformation in Ewing sarcoma". PLoS ONE 3 (4): e1965. doi:10.1371/journal.pone.0001965. PMC 2291578. PMID 18414662. http://www.plosone.org/article/info:doi/10.1371/journal.pone.0001965. 
7. ^ Sorensen PH, Lessnick SL, Lopez-Terrada D, Liu XF, Triche TJ, Denny CT (February 1994). "A second Ewing's sarcoma translocation, t(21;22), fuses the EWS gene to another ETS-family transcription factor, ERG". Nat. Genet. 6 (2): 146–51. doi:10.1038/ng0294-146. PMID 8162068. 
8. ^ Jeon IS, Davis JN, Braun BS et al. (March 1995). "A variant Ewing's sarcoma translocation (7;22) fuses the EWS gene to the ETS gene ETV1". Oncogene 10 (6): 1229–34. PMID 7700648. 
9. ^ See http://www.bcrt.org.uk/bci_symptoms_of_ewings_sarcoma.php
10. ^ Bernstein M, Kovar H, Paulussen M et al. (May 2006). "Ewing sarcoma family of tumours: current management". Oncologist 11 (5): 503–19. doi:10.1634/theoncologist.11-5-503. PMID 16720851. http://theoncologist.alphamedpress.org/cgi/pmidlookup?view=long&pmid=16720851. 
11. ^ URL: http://atlasgeneticsoncology.org/Tumors/Ewing5010.html. Accessed on: 23 February 2010.
12. ^ Turc-Carel C, Aurias A, Mugneret F et al. (June 1988). "Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12)". Cancer Genet. Cytogenet. 32 (2): 229–38. doi:10.1016/0165-4608(88)90285-3. PMID 3163261. 
13. ^ Ewing Sarcoma Imaging at eMedicine
14. ^ Lahl M, Fisher VL, Laschinger K (February 2008). "Ewing sarcoma family of tumours: an overview from diagnosis to survivorship". Clin J Oncol Nurs 12 (1): 89–97. doi:10.1188/08.CJON.89-97. PMID 18258578. http://ons.metapress.com/openurl.asp?genre=article&doi=10.1188/08.CJON.89-97. 
15. ^ Randall, RL (2005). "Ewing's Sarcoma Family of Tumours (ESFT)". ESUN. http://sarcomahelp.org/learning_center/ewings_sarcoma.html. Retrieved 2009-04-15. 
16. ^ Asami S, Chin M, Shichino H et al. (March 2008). "Treatment of Ewing sarcoma using an antisense oligodeoxynucleotide to regulate the cell cycle" (– ^{Scholar search}). Biol. Pharm. Bull. 31 (3): 391–4. doi:10.1248/bpb.31.391. PMID 18310898. http://joi.jlc.jst.go.jp/JST.JSTAGE/bpb/31.391?from=PubMed. ^{[dead link]}
17. ^ Mateo-Lozano S, Gokhale PC, Soldatenkov VA, Dritschilo A, Tirado OM, Notario V (November 2006). "Combined transcriptional and translational targeting of EWS/FLI-1 in Ewing sarcoma". Clin. Cancer Res. 12 (22): 6781–90. doi:10.1158/1078-0432.CCR-06-0609. PMID 17121899. http://clincancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=17121899. 
18. ^ Myatt SS, Redfern CP, Burchill SA (April 2005). "p38MAPK-Dependent sensitivity of Ewing's sarcoma family of tumors to fenretinide-induced cell death". Clin. Cancer Res. 11 (8): 3136–48. doi:10.1158/1078-0432.CCR-04-2050. PMID 15837770. 
19. ^ Myatt SS, Burchill SA (February 2008). "The sensitivity of the Ewing's sarcoma family of tumours to fenretinide-induced cell death is increased by EWS-Fli1-dependent modulation of p38(MAPK) activity". Oncogene 27 (7): 985–96. doi:10.1038/sj.onc.1210705. PMID 17700534. 
20. ^ ^{a b} Abir R, Feinmesser M, Yaniv I et al. (May 2010). "Occasional involvement of the ovary in Ewing sarcoma". Hum Reprod 25 (7): 1708–12. doi:10.1093/humrep/deq121. PMID 20472912. 
21. ^ McTiernan AM, Cassoni AM, Driver D, Michelagnoli MP, Kilby AM, Whelan JS (2006). "Improving Outcomes After Relapse in Ewing Sarcoma: Analysis of 114 Patients From a Single Institution". Sarcoma 2006: 83548. doi:10.1155/SRCM/2006/83548. PMC 1698143. PMID 17496997. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1698143. 
22. ^ "ACS :: How Is the Ewing Family of Tumors Staged?". http://www.cancer.org/docroot/CRI/content/CRI_2_4_3X_How_is_Ewings_Family_of_tumors_staged_48.asp?sitearea=. 
23. ^ Thacker, MM; Temple, HT; Scully, SP (2005). "Current treatment for Ewing's sarcoma". Expert review of anticancer therapy 5 (2): 319–31. doi:10.1586/14737140.5.2.319. PMID 15877528. 

External links

• Cancer.Net: Ewing Family of Tumors, Childhood
• Ewing family of tumors entry in the public domain NCI Dictionary of Cancer Terms

v d e Small blue round cell tumors B-cell chronic lymphocytic leukemia · Hepatoblastoma · Rhabdomyosarcoma  · Ewing's sarcoma · Neuroblastoma · Retinoblastoma · Wilms' tumor

v d e Connective tissue neoplasm: Osseous and Chondromatous tumors (ICD-O 9180–9269) (C40–C41/D16, 170/213) Diaphysis Myeloid Multiple myeloma Epithelial Adamantinoma PNET/Ewing family Ewing's sarcoma Metaphysis Osteoblast Osteoid osteoma · Osteoblastoma Osteoma/osteosarcoma Chondroblast Chondroma/ecchondroma/enchondroma (Enchondromatosis, Extraskeletal chondroma) · Chondrosarcoma (Mesenchymal chondrosarcoma, Myxoid chondrosarcoma) Osteochondroma (Osteochondromatosis) Chondromyxoid fibroma Fibrous Ossifying fibroma · Fibrosarcoma Epiphysis Chondroblast Chondroblastoma Myeloid Giant cell tumor of bone Other/ungrouped Notochord Chordoma M: BON/CAR anat(c/f/k/f, u, t/p, l)/phys/devp/cell noco/cong/tumr, sysi/epon, injr proc, drug(M5)

v d e Pathology: chromosome abnormalities (Q90–Q99 · 758) Autosomal Trisomies Down syndrome (21) · Edwards syndrome (18) · Patau syndrome (13) Trisomy 9 · Trisomy 8/Warkany syndrome 2 (8) · Trisomy 22/Cat eye syndrome (22) · Trisomy 16 Monosomies/deletions 1q21.1 deletion syndrome/1q21.1 duplication syndrome/TAR syndrome (1) · Wolf-Hirschhorn syndrome (4) · Cri du chat/Chromosome 5q deletion syndrome (5) · Williams syndrome (7) · Jacobsen syndrome (11) · Miller–Dieker syndrome/Smith–Magenis syndrome (17) · DiGeorge syndrome (22) · 22q13 deletion syndrome (22) genomic imprinting (Angelman syndrome/Prader–Willi syndrome (15)) Distal 18q-/Proximal 18q- X/Y linked Monosomy Turner syndrome (XO) Trisomy/tetrasomy, other karyotypes/mosaics Klinefelter's syndrome (47,XXY) · 48,XXYY · 48,XXXY · 49,XXXYY · 49,XXXXY Triple X syndrome (47,XXX) · 48,XXXX · 49,XXXXX 47,XYY · 48,XYYY · 49,XYYYY 46,XX/XY Translocations Leukemia/lymphoma Lymphoid Burkitt's lymphoma t(8 MYC;14 IGH) · Follicular lymphoma t(14 IGH;18 BCL2) · Mantle cell lymphoma/Multiple myeloma t(11 CCND1:14 IGH) · Anaplastic large cell lymphoma t(2 ALK;5 NPM1) · Acute lymphoblastic leukemia Myeloid Philadelphia chromosome t(9 ABL; 22 BCR) · Acute myeloblastic leukemia with maturation t(8 RUNX1T1;21 RUNX1) · Acute promyelocytic leukemia t(15 PML,17 RARA) · Acute megakaryoblastic leukemia t(1 RBM15;22 MKL1) Other Ewing's sarcoma t(11 FLI1; 22 EWS) · Synovial sarcoma t(x SYT;18 SSX) · Dermatofibrosarcoma protuberans t(17 COL1A1;22 PDGFB) · Myxoid liposarcoma t(12 DDIT3; 16 FUS) · Desmoplastic small round cell tumor t(11 WT1; 22 EWS) · Alveolar rhabdomyosarcoma t(2 PAX3; 13 FOXO1) t (1 PAX7; 13 FOXO1) Other Fragile X syndrome · Uniparental disomy · XX male syndrome

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