Multimodality Treatment in Ewing's Sarcoma Family Tumors of the Maxilla and Maxillary Sinus: Review of the Literature.

David Thorn,Frank Metternich,Fatime Krasniqi,Christoph Mamot,Sven Prestin

doi:10.1155/2016/3872768

David Thorn, Frank Metternich + Show 3 more

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https://doi.org/10.1155/2016/3872768

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Journal: Sarcoma	Publication Date: Jan 1, 2016
Citations: 94	License type: CC BY 4.0

Affiliation: Kantonsspital Aarau, University Hospital of Basel

Abstract

The Ewing sarcoma family of tumors (ESFT) encompasses a group of highly aggressive, morphologically similar, malignant neoplasms sharing a common spontaneous genetic translocation that affect mostly children and young adults. These predominantly characteristic, small round-cell tumors include Ewing's sarcoma of the bone and soft tissue, as well as primitive neuroectodermal tumors (PNETs) involving the bone, soft tissue, and thoracopulmonary region (Askin's tumor). Extraosseous ESFTs are extremely rare, especially in the head and neck region, where literature to date consists of sporadic case reports and very small series. We hereby present a review of the literature published on ESFTs reported in the maxilla and maxillary sinus region from 1968 to 2016.

Highlights

Since the latest WHO classification of 2013 [1], Ewing sarcoma family of tumors (ESFT) encompasses a group of highly aggressive, morphologically similar, malignant neoplasms sharing a common spontaneous genetic translocation
primitive neuroectodermal tumors (PNETs), a group of tumors classified by their common neuroectodermal origin were formerly subdivided into three major groups: (1) central PNETs, including tumors arising from the central nervous system, such as medulloblastoma; (2) neuroblastoma, including tumors arising from the autonomic nervous system; and (3) peripheral PNETs referring to PNETs arising outside the central nervous system [3]
From 1968 to 2016, we found a total of 93 cases of ESFTs involving the maxilla or maxillary sinus published in the English medical literature (Table 1)

Summary

Introduction

Since the latest WHO classification of 2013 [1], Ewing sarcoma family of tumors (ESFT) encompasses a group of highly aggressive, morphologically similar, malignant neoplasms sharing a common spontaneous genetic translocation. These predominantly characteristic, small round-blue-cell tumors include classical Ewing’s sarcoma of the bone, extraosseous and soft tissue Ewing’s sarcoma, as well as primitive neuroectodermal tumors (PNETs) involving the bone, soft tissue, and the chest wall, and the latter is referred to as Askin’s tumor [2]. In the 1980s, the term peripheral PNET was reestablished to describe a group of soft tissue tumors of presumed neural-crest origin that presented outside the CNS and in the sympathetic nervous system

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