Primary orbital Ewing's sarcoma: Report of a case and review of the literature

Lam, Dennis S.C.; Li, Chi Kong; Cheng, Lulu; Teo, Jennifer; Chik, Ki Wai; Kwan, W. H.; Ng, Joan S.K.; Poon, William; Yuen, Patrick Man Pan

doi:10.1038/eye.1999.8

Cited by 21 publications

(17 citation statements)

References 17 publications

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“…18 ES most commonly affects the long bones of limbs, the ribs, and the pelvis, infrequently arises primarily in the head and neck (1%–4% of all ES), and is rare to involve the orbit with the mandible and maxilla more likely to be affected. 16 Primary extraskeletal (soft tissue) orbital ES is very rare with this case adding to the small number previously reported in the literature. 12–14 More commonly, orbital ES represents metastases from a distant site.…”

Section: Discussionmentioning

confidence: 81%

“…12–14 Recurrent ES carries a poor prognosis 15 with no past reports of successful treatment of recurrent primary ES of the orbit, which often progresses rapidly and has a propensity to metastasize. 9,10,16,17 The authors report a case of primary extraskeletal ES of the orbit in a 6-year-old boy and successful treatment using a unique multimodal treatment approach. This case report is in compliance with the Health Information Portability and Accountability Act and followed the principles of the Declaration of Helsinki.…”

Section: Case Reportmentioning

confidence: 99%

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Exenteration and Custom Implant Brachytherapy as a Treatment for Recurrent Primary Extraskeletal Orbital Ewing Sarcoma

Klufas

Wolden

Bohle³

et al. 2015

Ophthalmic Plastic &Amp; Reconstructive Surgery

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A 6-year-old boy initially presented to an outside hospital with a right orbital mass with biopsy positive for translocation involving EWS RNA-binding protein 1 gene and imaging consistent with primary extraskeletal Ewing sarcoma (ES). There was no evidence of metastatic disease. Patient underwent gross tumor resection and adjuvant chemotherapy (VAdriaC/IE) followed by postoperative 45-Gy proton beam radiation. After 19 months, a solitary infield local recurrence occurred, which was unsuccessfully surgically resected. Thereafter, treatment commenced with irinotecan and temozolomide, and the patient presented to the center of the authors. MRI showed locally recurrent disease without evidence of metastatic disease. Right orbital exenteration was performed, and an orbital mold was fashioned to deliver brachytherapy. There were no complications. The patient had no evidence of recurrent disease at 37-month follow up. This is the first report of orbital implant brachytherapy for recurrent primary ES of the orbit, and an additional report of primary extraskeletal ES of the orbit, which is a rare primary orbital tumor.

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Section: Discussionmentioning

confidence: 81%

Section: Case Reportmentioning

confidence: 99%

Exenteration and Custom Implant Brachytherapy as a Treatment for Recurrent Primary Extraskeletal Orbital Ewing Sarcoma

Klufas

Wolden

Bohle³

et al. 2015

Ophthalmic Plastic &Amp; Reconstructive Surgery

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“…Identification of EWS rearrangements using Flourosence in situ hybridization (FISH) or Reverse transcriptase polymerase chain reaction or RT-PCR is useful in differentiating EWS from other round cell tumors. [12] Most cases have a balanced translocation between chromosomes 11 and 22 which fuses portions of EWS gene on chromosome 22q12 with FLI1 gene on 11q24. Other translocations observed are t (21;22) which results in the fusion between EWS and ERG gene and t (7;22) which is due to fusion between EWS and ETV1 located on chromosome 7p22.…”

Section: Discussionmentioning

confidence: 99%

Primary Ewing′s sarcoma of the cranium: Case series and review of literature

et al. 2014

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Primary Ewing's sarcoma (EWS) of the cranium is extremely rare. It accounts for <1% of cases. We retrospectively analyzed our EWS cases to determine those which had a primary cranial involvement. Out of a total of 332 cases of EWS registered between the years 2000 and 2011, 7 were of the primary cranial involvement. Treatment modalities included surgery, chemotherapy, and radiotherapy (RT), as indicated. The follow-up ranged from 8 months to 7.5 years. In our study, parieto-occipital region was the commonest site. Most patients presented with localized disease and swelling of the scalp. Excision followed by chemotherapy or RT appears to have good survival rates. At a median follow-up of 32.2 months, only one patient had a recurrence, and was successfully salvaged with second line chemotherapy. These cases illustrate that a multi-disciplinary approach in patients with EWS of the cranium results in a good outcome.

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“…It represents about 6–9% of all malignant bone tumours and has a predilection for long bones, ribs and the pelvis [8, 9]. A literature search revealed only eleven cases of primary cranial Ewing’s sarcoma [1,2,3,4,5,6,7,8,9,10,11,12,13] of which only seven were involving the orbit [1,2,3,4,5,6,7]. Orbital tumours usually grow outwards, leading to commonly seen localized swelling.…”

Section: Discussionmentioning

confidence: 99%

“…Preoperative imaging could thus prove crucial in such a scenario. Surprisingly, of the seven cases of primary orbital Ewing’s sarcoma described in the literature [1,2,3,4,5,6,7], none had a magnetic resonance imaging (MRI) study performed. We report a case of primary orbital Ewing’s sarcoma, the MR findings in which were very unusual and suggest that the MR findings of orbital Ewing’s sarcoma could be different from those of Ewing’s sarcoma elsewhere in the body.…”

Section: Introductionmentioning

confidence: 99%

Magnetic Resonance Imaging of Primary Orbital Ewing’s Sarcoma

2005

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The authors describe the MRI findings of a primary orbital Ewing’s sarcoma in a 5-year-old boy, who underwent an extensive tumour surgery as these findings were mistaken for more chemoresistant sarcomas such as rhabdomyosarcoma. This case illustrates the fact that MR findings of primary orbital Ewing’s sarcoma may be different from those of Ewing’s sarcoma present elsewhere in the body. An accurate preoperative diagnosis may therefore result in a more conservative approach and prove extremely useful in such tumours.

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Primary orbital Ewing's sarcoma: Report of a case and review of the literature

Cited by 21 publications

References 17 publications

Exenteration and Custom Implant Brachytherapy as a Treatment for Recurrent Primary Extraskeletal Orbital Ewing Sarcoma

Exenteration and Custom Implant Brachytherapy as a Treatment for Recurrent Primary Extraskeletal Orbital Ewing Sarcoma

Primary Ewing′s sarcoma of the cranium: Case series and review of literature

Magnetic Resonance Imaging of Primary Orbital Ewing’s Sarcoma

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