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Radiation therapy for Ewing sarcoma family of tumors

Thomas F DeLaney, MD
Section Editors
Robert Maki, MD, PhD
Alberto S Pappo, MD
Deputy Editor
Diane MF Savarese, MD


Ewing sarcoma (ES) is a rare malignancy that most often presents as an undifferentiated primary bone tumor; less commonly, it arises in soft tissue (extraosseous Ewing sarcoma [EES]). 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, adult neuroblastoma, 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. (See "Epidemiology, pathology, and molecular genetics of the Ewing sarcoma family of tumors".)

EFT also share important clinical features, including a peak incidence between the age of 10 and 20, a tendency towards rapid spread to lungs, bone, and bone marrow, and responsiveness to the same chemotherapeutic regimens and radiation therapy (RT). Because relapse rates are high in patients undergoing local therapy alone (80 to 90 percent), it is surmised that the majority have subclinical metastatic disease at the time of diagnosis, even in the absence of overt metastases. The routine administration of intensive multiagent chemotherapy, which can eradicate these deposits, has had a dramatic impact on outcomes. Five-year survival rates for patients with ES in the United States rose from 36 to 56 percent during the periods 1975 to 1984 and 1985 to 1994 [2]. (See "Treatment of the Ewing sarcoma family of tumors".)

Local control at the primary tumor site can be accomplished by surgery, RT, or both. The choice of modality usually represents a tradeoff between functional result and treatment-related morbidity, particularly the risk of a secondary radiation-induced malignancy. Although modern treatment protocols emphasize surgery for optimal local control, patients who lack a function-preserving surgical option because of tumor location or extent, and those who have clearly unresectable primary tumors following induction chemotherapy are appropriate candidates for RT.

Since more than 90 percent of patients with EFT have either detectable or subclinical metastases at diagnosis, local therapy, if delivered correctly, is probably not the critical event in determining survival. However, if local therapy is delivered poorly or given in such a way that it compromises the delivery of adequate chemotherapy, survival can be greatly compromised. Moreover, local failure is associated with a very poor survival outcome [3].

Here we will discuss the role of RT in the local management of the EFT. Epidemiology, pathology, molecular genetics, clinical presentation, and diagnosis of these tumors, as well as surgical principles and the use of chemotherapy are presented elsewhere. Central (supratentorial) PNET tumors are also 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 "Treatment of the Ewing sarcoma family of tumors" and "Bone sarcomas: Preoperative evaluation, histologic classification, and principles of surgical management" and "Uncommon brain tumors", section on 'CNS embryonal tumor, NOS'.)

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Literature review current through: Nov 2017. | This topic last updated: Apr 17, 2017.
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