Ewing's Sarcoma of the Head and Neck: A Retrospective Analysis of 24 Cases

[摘要] Introduction and purpose. Primary Ewing's sarcoma arising from thebones of the head and neck region is extremely rare representing only 1– 4% of all Ewing'ssarcoma cases. Previous reports suggest a better prognosis for that particular anatomic site.The purpose of this study was to analyze the clinico-epidemiologic characteristics of that rare clinical presentation, aswell as its patterns of failure and prognosis following treatment.Materials and methods.This study included a retrospective review of the medical records of patients with the diagnosis of Ewing's sarcoma of the head and neck region treated at King Faisal Specialist Hospital and Research Center between 1975 and 1996.Results. Out of a total number of 24 cases analyzed, there were 17 males and7 females with a ratio of 2.4:1. The median age at diagnosis was 16.5 years. A painful swelling wasthe most common clinical presentation.The maxilla was the most common site of presentation(9/24 cases). There were 3/24 cases who presented with metastatic disease at diagnosis.Themajority of patients (16/24 cases) had a tumor size >10 cm. Most patients were treated with systemicchemotherapy plus localized irradiation following an initial biopsy.With a mean followup of 3.4 years, the 5-year actual overall survival (OS) for the whole group was 53%, whilethe 5-year actuarial disease-free survival (DFS) was 30%. These figures were higher than thoserepor ted from our institution for young patients (≤ 14 years treated for Ewing' s sarcoma in otheranatomic locations (30% v 15%). The response to chemotherapy was the only prognostic factorthat affected both the OS and DFS.Conclusion.The prognosis of Ewing's sarcoma of the head and neckregion is slightly better than that of other anatomic sites.The response to systemic chemotherapyis one of the most important prognostic factors affecting both DFS and OS of Ewing's sarcoma ofthe head and neck. Multimodality therapy consisting of an initial biopsy, aggressive combinationchemotherapy and localized radiotherapy is the treatment of choice for Ewing's sarcoma of the head and neck region and may result in long-term survival.

[学科分类] 肿瘤学