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Treatment of the Ewing sarcoma family of tumors

Mark C Gebhardt, MD
Steven G DuBois, MD, MS
Section Editors
Alberto S Pappo, MD
Robert Maki, MD, PhD
Raphael E Pollock, MD
Deputy Editor
Diane MF Savarese, MD


Ewing sarcoma is a rare malignancy that most often presents as an undifferentiated primary bone tumor; less commonly, it arises in soft tissue (extraosseous Ewing sarcoma). Both are part of a spectrum of neoplastic diseases known as the Ewing sarcoma family of tumors (EFT), which also includes the more-differentiated peripheral primitive neuroectodermal tumor (PNET; previously called peripheral neuroepithelioma and Askin's tumor of the chest wall) [1]. PNET can also present either in bone or soft tissue. Because these tumors share similar histological and immunohistochemical characteristics and unique nonrandom chromosomal translocations, they are considered to have a common origin [2-5].

In addition to their immunohistochemical and cytogenetic similarities, the EFT share important clinical features. These include a peak incidence between the age of 10 and 20 (70 percent of affected patients are under the age of 20), a tendency towards early dissemination to the lungs, bone, and bone marrow, and responsiveness to chemotherapy and radiation therapy (RT). Advances in multidisciplinary management over the past 30 years have resulted in a marked improvement in long-term survival. In data derived from the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute, five-year survival rates for patients with Ewing sarcoma rose from 36 to 56 percent during the periods 1975 to 1984 and 1985 to 1994 [6]. (See "Bone sarcomas: Preoperative evaluation, histologic classification, and principles of surgical management".)

Here we will discuss the management of the EFT. The epidemiology, pathology, molecular genetics, clinical presentation, and diagnosis of these tumors, surgical principles, indications for limb-sparing surgery, and indications for RT are discussed elsewhere. In addition, central nervous system (supratentorial) PNET tumors and undifferentiated sarcomas lacking classic Ewing sarcoma chromosomal translocations (so-called Ewing-like sarcomas) are biologically distinct from Ewing sarcoma and peripheral PNET. Management of these tumors is discussed elsewhere. (See "Epidemiology, pathology, and molecular genetics of the Ewing sarcoma family of tumors" and "Clinical presentation, staging, and prognostic factors of the Ewing sarcoma family of tumors" and "Uncommon brain tumors", section on 'Ewing sarcoma/primitive neuroectodermal tumor' and "Bone sarcomas: Preoperative evaluation, histologic classification, and principles of surgical management".)


Despite the fact that fewer than 25 percent of patients have overt metastases at the time of diagnosis, EFT is a systemic disease. Because of the high relapse rate (80 to 90 percent) in patients undergoing local therapy alone, it is surmised that the majority of patients have subclinical metastatic disease at the time of diagnosis, even in the absence of overt metastases.

Chemotherapy can successfully eradicate these deposits, and modern treatment plans all include chemotherapy, usually administered prior to and following local treatment. For patients with localized disease, the addition of several months of intensive multiagent chemotherapy to local therapy has had a dramatic impact on survival, and reported 5- and 10-year survival rates are now approximately 70 and 50 percent, respectively [7-16].

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Literature review current through: Nov 2017. | This topic last updated: Oct 13, 2017.
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