Ewing′s sarcoma of maxilla: A rare case report

Abstract

Ewing's sarcoma is uncommon malignancy of childhood, frequently involving the mandible. The occurrence in maxilla is rare. It is histopathologically characterized by sheets of round cells positive for CD99. Although the prognosis is poor but early diagnosis and long term follow up can improve the survival.This article presents a rare case of Ewing's sarcoma of maxilla in a 15 year old male patient showing excessive fibro-osseous response which is not a frequent presentation. A retrospective analysis of cases of Ewings sarcoma of maxilla published in the English litreture is reviewed. In our case, diagnosis was confirmed by immunohistochemistry where sheets of round tumor cells were positive for CD 99. Ewings sarcoma of maxilla is a rare and aggressive tumor. Hence early diagnosis, combined therapy and long term follow up is suggested in such cases.

Early recognition and diagnosis of Ewing sarcoma of the cervical spine

“…ES is slightly more prevalent in males than in females, with male to female ratios within the approximate range of 1.3-1.5:1 [3] . ES is more so found in younger individuals, most frequently adolescents ranging from ages 10 to 20 years old, of Caucasian background and less frequently reported in individuals of African and Asian ethnicity [4] , [5] , [6] . In the head and neck region, the mean age of occurrence is 10.9 years old [3] .…”

“…First defined by Dr. James Ewing in 1921, ES (also known as Ewing's sarcoma) is a small round blue cell tumor which, after osteosarcoma, is the next most frequent occurring form of bone cancer in children and young adults [1] , [2] , [3] , [4] , [5] . ES is characterized by Ewing's sarcoma family tumor containing 1 of 2 chromosomal translocations, with over 90% having a t(11;22) (q24;q12) translocation and the balance expressing a t(21;12)(22;12) translocation [1] , [3] , [4] , [5] , [6] , [7] , [8] . Annual estimates of cases in the United States range from 200 to 500, and for children and adolescents being 4%-10% of all primary bone malignancies [2] , [3] .…”

“…In the head and neck region, the mean age of occurrence is 10.9 years old [3] . ES is also more often observed occurring first in long bones and the pelvic girdle, with lower occurrences witnessed and clinically reported in the head and neck region, but it is capable of occurring in just about any bone [3] , [5] , [6] , [9] . Treatment for ES includes systemic multidrug chemotherapy (aiding with the suppression of micro metastasis and tumor reduction before surgery), surgery (eg, tumor resection), and radiotherapy (especially for unresectable primaries) [1] , [3] .…”

“…The case reported is of particular interest as it illustrates a finding of ES in the cervical spine, a place where ES does not frequently develop. About a mere 3% of diagnosed ES is in the bones of the neck and head (excluding the jaw), with 0.9% of studied cases involving the nonsacral spine [5] , [9] . This is especially important because of the nature of ES and its characteristically negative prognosis.…”

Aggressive high-grade Ewing's sarcoma of maxilla: A rare case report

“…The prognosis of ES is poor because hematogenous spread and lung metastases occur within a few months after diagnosis, although the tumor burden is considered today as an important factor of prognosis. [ 12 ]…”