Childhood Ewing Sarcoma of the Orbit

Abstract

In the span of the last 48 years, only 33 cases of children with orbital Ewing sarcoma have been reported. This study is to present 3 cases that were admitted to Children's Cancer Hospital Egypt 57357, during the period from 2009 to 2013. We have 2 cases treated using the hospital standard Ewing sarcoma treatment protocol, to completion, whereas the third discontinued treatment. All tumors have confirmed CD99 positivity, although translocation (11;22) was positive in 1 patient and negative in the third. With earlier diagnosis and adequate surgical resection and integration of chemotherapy and radiotherapy 1 patient survived for about 4 years, whereas the other 2 cases died due to disease progression or recurrence.

Primary orbital Ewing sarcoma family of tumors: a study of 12 cases

“…Immunostaining with CD99 is the keystone in the diagnosis and is a common feature of ESFTs. 4 In our study, CD99 was positive in all cases. Fluorescence in situ hybridization studies to detect chromosome (11;22) translocation can also be used for definite diagnosis of ESFTs.…”

“…23 There are only 41 cases of primary orbital ESFTs published in the literature. 4,24 Based on the available data, the mean age at presentation of these published cases was 7 years (median, 7 years; range, 0.5-20 years). 4,24 In our series, the mean age at presentation was higher at 12 years, whereas the median age at presentation was comparable at 8 years.…”

“…Differential diagnosis of small round cell tumors include ESFT, neuroblastoma, rhabdomyosarcoma, lymphoma, osteogenic sarcoma, and mesenchymal chondrosarcoma. 4,27 Immunohistochemical and/or ultrastructural techniques are useful to establish the diagnosis. Immunostaining with CD99 is the keystone in the diagnosis and is a common feature of ESFTs.…”

“…Fluorescence in situ hybridization studies to detect chromosome (11;22) translocation can also be used for definite diagnosis of ESFTs. 4 ESFTs exhibits rapid progression and have poor prognosis. Local tumor extension and clinical metastases are often found during initial diagnosis of ESFTs.…”

“…3 Primary orbital ESFT is extremely rare with isolated case reports and small case series reported in literature. [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Herein we discuss the demographic profile, clinical features, radiological features, treatment modalities, and outcomes of primary orbital ESFTs.…”