Journal of Pediatric Ophthalmology and Strabismus

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Short Subjects 

Primary Orbital Ewing Sarcoma

Yuli Yang, PhD; Yong Liu, PhD; Zhengqin Yin, PhD

Abstract

The authors report the clinical manifestations, pathologic character, management, and prognosis of primary orbital Ewing sarcoma, and review pertinent literature. A 6-year-old boy presented with the complaint of abruptly decreasing visual acuity and right proptosis. Computed tomography scan revealed a mass in the region of the right infratemporal orbit with extension into the infratemporal fossa and masseteric space, and there was erosion of the right lateral orbital wall. An anterior orbitotomy eyelid crease was performed to remove the tumor. The tumor cells were positive in membranous pattern for CD99 immunostaining; all other reactions were negative. The EWS-FLI-1 fusion gene was not detected by reverse-transcription polymerase chain reaction. Combined radiotherapy was applied. Primary orbital Ewing sarcoma is an extremely rare malignant tumor that shows a predilection for males (ratio: 1.4 to 1). Appropriate treatment consists of local resection, radiotherapy, and adjunctive chemotherapy.

Abstract

The authors report the clinical manifestations, pathologic character, management, and prognosis of primary orbital Ewing sarcoma, and review pertinent literature. A 6-year-old boy presented with the complaint of abruptly decreasing visual acuity and right proptosis. Computed tomography scan revealed a mass in the region of the right infratemporal orbit with extension into the infratemporal fossa and masseteric space, and there was erosion of the right lateral orbital wall. An anterior orbitotomy eyelid crease was performed to remove the tumor. The tumor cells were positive in membranous pattern for CD99 immunostaining; all other reactions were negative. The EWS-FLI-1 fusion gene was not detected by reverse-transcription polymerase chain reaction. Combined radiotherapy was applied. Primary orbital Ewing sarcoma is an extremely rare malignant tumor that shows a predilection for males (ratio: 1.4 to 1). Appropriate treatment consists of local resection, radiotherapy, and adjunctive chemotherapy.

From the Southwest Hospital, Southwest Eye Hospital, Third Military Medical University, Chongquing, China.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Yuli Yang, PhD, Southwest Hospital, Southwest Eye Hospital, Third Military Medical University, No. 30 Gaotanyan Main Street, Shapingba District, Chongqing, 400038 China. E-mail: yangyuli1979@yahoo.com.cn

Received: April 09, 2011
Accepted: June 01, 2011
Posted Online: July 19, 2011

Introduction

Primitive neuroectodermal tumor (PNET) is a term for a group of small round cell tumors that arise from primitive neuroectodermal progenitor cells. Once believed to be confined to the central nervous system, PNETs are now increasingly recognized to occur outside the central nervous system and are designated as peripheral PNETs (pPNETs).1 According to different degrees of neuroectodermal differentiation, pPNETs were further divided into pigmented neuroectodermal tumor, Askin tumor, and Ewing sarcoma/pPNET groups.2 Ewing sarcoma/pPNETs most commonly affects the long bones of the limbs, the ribs, and the pelvis.3–6 Ewing sarcoma/pPNET of the head and neck region is unusual, comprising 1% to 4% of all cases of Ewing sarcoma/pPNET.7,8 Although Ewing sarcoma/pPNETs occur more commonly in children, primary orbital involvement is extremely rare. Only 20 reports on Ewing sarcoma/pPNET of the orbit appear in the literature.9–25 We describe an additional patient with an orbital Ewing sarcoma/pPNET, features of which are compared with the previous cases in the literature.

Case Report

A 6-year-old boy presented with the complaint of abruptly decreasing visual acuity for approximately 50 days in February 2009. His best-corrected visual acuity was 20/250 in his right eye and 20/20 in his left eye. External ocular examination showed localized swelling and erythema of the right lower eyelid. There was 6 mm of right proptosis with supranasal duction (Fig. 1). Examination of the extra-ocular movements revealed limitation of downward and lateral gaze of the right eye. Bilateral anterior segments and intraocular pressures were normal. Funduscopy of the right eye also showed normal findings.

A 6-year-old boy with a mass in the infratemporal portion of the right orbit.

Figure 1. A 6-year-old boy with a mass in the infratemporal portion of the right orbit.

Computed tomography scan revealed a mass in the region of the right inferotemporal orbit with extension into the infratemporal fossa and masseteric space, and there was erosion of the lateral orbital wall (Fig. 2). A complete blood cell count and serum laboratory values were normal. Bone marrow aspiration showed normal findings. Chest and long bone radiography were normal and abdominal ultrasonography revealed no organ involvement.

Computed tomography scan showing a mass in the region of the right inferotemporal orbit with extension into the infra-temporal fossa and masseteric space and there was erosion of the lateral orbital wall: (A) horizontal view and (B) coronal view.

Figure 2. Computed tomography scan showing a mass in the region of the right inferotemporal orbit with extension into the infra-temporal fossa and masseteric space and there was erosion of the lateral orbital wall: (A) horizontal view and (B) coronal view.

An anterior orbitotomy eyelid crease was performed to remove the tumor. The mass adhered to the adjacent structures. After the loose adhesions were overcome by careful dissection, the mass was extracted without damage. Following the surgery, the patient was administered radiation therapy for the entire right orbit and chemotherapy for the residual tumors. The chemotherapeutic regimen consisted of multiple cycles of vincristine, actinomycin D, and cyclophosphamide. However, the tumor recurred, and the patient died approximately 14 months after surgery because the tumor had metastasized to the brain.

The excised tumor mass was fixed in 4% buffered formaldehyde and embedded in paraffin. Serial sections through the tumor were performed using routine histopathologic techniques and stained with hematoxylin–eosin and periodic acid-Schiff stains. Immunohistochemical reactions were performed using the streptavidin-biotin method. The following antibodies were applied: desmin, myogenin, leukocyte common antigen, synaptophysin, neurospecific enolase, and MIC2 (CD99). These antibodies were applied synchronously with appropriate positive control slides. For the negative control, buffered saline was used.

Total RNA was extracted from paraffin-embedded tissues. The sections were deparaffinized in three changes of xylene and three washes with 100% ethanol. After drying, tissue pellets were re-suspended in 200 μL of lysis buffer (20 mmol/L Tris-HCL, PH 8.0, 20 mmol/L ethylenediaminetetraacetic acid, 2% sodium dodecyl sulfate) containing proteinase K. After incubation at 55°C overnight, 1.0 mL of Trizol (Invitrogen, Carlsbad, CA) was added to the sample and total RNA was extracted according to the manufacturer’s instructions. The RNA pellets were reconstituted in 20 μL of diethylpyrocarbonate-treated water and stored at −80°C. Thereafter, 1 μg of the extracted RNA was reverse-transcribed into cDNA using 50 units of SuperScript II (Invitrogen, Carlsbad, CA) reverse transcriptase, 100 ng of random primer, 0.5 mmol/L dNTP, and 40 units of RNase inhibitor, and was then incubated with 2 units of RNase H for 20 minutes at 37°C.

Polymerase chain reactions (PCR) were performed to detect the EWS-FLI-1 fusion transcripts. PCR was performed for EWS-FLI-1. PCR amplifications were performed in a total volume of 50 μL containing 5 μL of RT reaction product as template DNA, 1 × PCR buffer, 1.5 mmol/L of magnesium chloride, 0.1 mmol/L of each deoxynucleotide, 100 ng of each sense and antisense primer, and 1.25 U of Taq DNA polymerase. Programmable temperature cycling was performed with the following cycle profile: 95°C for 5 minutes, followed by 40 cycles of 94°C for 1 minutes, 65°C for 1 minutes, and 72°C for 2 minutes. After the last cycle, an extended 10 minutes at 72°C was followed by cooling to 4°C. Cultured cells of the human Ewing sarcoma cell line were used as known positive controls carrying the EWS exon 7-FLI-1 exon 6 translocation. A negative control in which the RT enzyme was omitted was used to exclude DNA contamination. Cases of unrelated tumors were used as negative controls. A reaction mixture of reagents devoid of template was included in each PCR procedure as a blank control.

Results

On gross inspection, the excised tumor consisted of white, soft, and lobulated parts and measured 3.7 × 3.6 × 1.8 mm. It was partially encapsulated with moderate contexture. Microscopically, the tumor was composed of irregularly shaped islands of small round cells with basophilic cytoplasm and pleomorphic nuclei. There were frequent abnormal mitoses. Near the borders of the tumor, the cells infiltrated the surrounding fibrous tissue in cords. Homer–Wright pseudorosettes were not found (Fig. 3). Regarding immunostaining (Fig. 4), the tumor cells were strongly and diffusely positive in membranous pattern for CD99 immunostaining. All other reactions were negative. There were intervening fibrous septa among tumor cells that were stained positively with periodic acid-Schiff (Fig. 5). EWS-FLI-1 fusion genes were not detected by reverse-transcription PCR.

Histology of the Ewing sarcoma showing islands of monotonous small round cells with foamy cytoplasm and pleomorphic nuclei. There were no rosettes (hematoxylin–eosin, original magnification ×50).

Figure 3. Histology of the Ewing sarcoma showing islands of monotonous small round cells with foamy cytoplasm and pleomorphic nuclei. There were no rosettes (hematoxylin–eosin, original magnification ×50).

The tumor cells immunostained with anti-MIC2 antibodies (original magnification ×200).

Figure 4. The tumor cells immunostained with anti-MIC2 antibodies (original magnification ×200).

There were intervening fibrous septa among tumor cells that were stained positively with periodic acid-Schiff (original magnification ×50).

Figure 5. There were intervening fibrous septa among tumor cells that were stained positively with periodic acid-Schiff (original magnification ×50).

Discussion

Ewing sarcoma/pPNET is one of the most malignant bone and soft tissue tumors in adolescents and young adults.26 Among the phenotypic spectrum of the Ewing family of tumors, Ewing sarcoma is at the most undifferentiated end of the spectrum, lacking most of the features of neural differentiation; the other end of the spectrum comprises the most differentiated pPNET.27 Recently, cytogenic findings revealed that Ewing sarcoma and pPNETs share the same specific reciprocal chromosome translocation t(11;22)(q24;q12).28,29 pPNET may demonstrate varying degrees of neural differentiation and the progressive process begins with neurospecific enolase expression, followed by Homer–Wright rosette formation, phenotypic ganglion cell differentiation, and neurofilament protein expression.30 At the light microscopy level, dense clumping of chromatin and mitotic figures are more common in pPNET. The rosette formation and fibrous background are necessary for the diagnosis of pPNET.

Regarding the immunophenotypes, Ewing sarcoma and pPNET may share some similar immunophenotypes, with the exception that pPNET should be positive for some neural markers, such as neurospecific enolase synaptophysin. According to the immunohistochemical and cytogenic findings, our case favors Ewing sarcoma rather than pPNET.

Primitive neuroectodermal tumor of the ulnar nerve was initially described in 1918 by Stout.31 Until the late 1970s, Ewing sarcoma/pPNET was not recognized as an entity. Ewing sarcoma/pPNET most commonly affects the long bones and it accounts for 8% to 10% of all primary malignant bone tumors.32 Primary orbital Ewing sarcoma/pPNETs are extremely rare. Harbert and Tabor reported the first case in a 19-year-old boy presenting with a proptosis due to a mass in the lateral portion of the right orbit.9 To our knowledge, only 20 cases of primary orbital Ewing sarcoma/pPNET have been reported. Orbital Ewing sarcoma/pPNETs are heterogeneous from a clinical and histopathologic standpoint (Table). Among reported cases there was a male-to-female ratio of 12 to 8 and the mean age at presentation was 17 years (range: < 1 to 61 years). One common feature of these tumors seems to be their proclivity to arise in the lateral orbit. Extraorbital extension has been noted in 12 cases. There are 4 patients with systemic metastases.21,22,24

Reported Cases of Primary Ewing’s Sarcoma Involving the Orbit

Table: Reported Cases of Primary Ewing’s Sarcoma Involving the Orbit

The differential diagnosis of small round cell tumors of childhood and adolescence34 may be difficult. Small round cell tumors of childhood and adolescence are a diverse group of tumors of neural, mesenchymal, and lymphoid derivation that may share a similar histologic appearance of small, poorly differentiated cells, including neuroblastoma, Ewing sarcoma, rhabdomyosarcoma, and malignant lymphoma. However, accurate diagnosis and staging of these tumors is essential because therapy and prognosis are tumor specific. Currently, CD99-O13 immunostaining and molecular studies using PCR are definitive for Ewing sarcoma to detect characteristic chromosomal translocations. Approximately 90% of tumors exhibit a fusion of the EWS and FLI-1 genes resulting from a characteristic t(11;22). This results in the juxtaposition of the 5′ end of the EWS gene to the 3′ end of the FLI-1 gene.

The EWS gene has been poorly characterized, but is known to encode an RNA-binding protein. The human FLI-1 gene is a member of the ETS family of DNA transcription factors. The translocation results in a novel fusion protein that has been shown to have transforming activity in NIH 3T3 cells.34 In approximately 5% of the Ewing sarcoma family of tumors, a fusion of the EWS and ERG genes resulting from a t(11;22) can be detected. In these tumors, the ERG gene is believed to function similarly to the FLI-1 gene present in the classic translocation because both ERG and FLI-1 are members of the ETS family of DNA-binding factors and share significant homology.35 The presence of the fusion proteins resulting from these translocation events can be detected in tissues using reverse-transcription PCR.

The Ewing sarcoma family of tumors is characterized by the presence of specific translocations resulting in the formation of the MIC2 surface protein36 and this could be detected by the specific antibody CD99-O13 in immunostaining. The absence of MIC2 protein or t(11;22) translocation in the neoplasms would suggest that they are not Ewing sarcoma. CD99 immunostaining was positive in our case, but EWS exon 7-FLI-1 exon 6 translocation was not detected. Ramzi et al.37 reported on a series of 58 patients with Ewing sarcoma; EWS exon 7-FLI-1 exon 6 translocation was only detected in 45 patients (78%) and some other types of gene translocations existed in Ewing sarcoma. That may explain why we did not detect EWS exon 7-FLI-1 exon 6 translocation in our case.

There is no consensus for the best treatment strategy for Ewing sarcoma/pPNETs; they progress rapidly with poor prognosis and often have metastasized at the time of diagnosis.38 Curative treatment now exists for most patients with localized tumors and consists of surgery and systemic chemotherapy or radiation therapy for control of the primary tumor.39 There is no doubt that the addition of adjuvant chemotherapy has been of considerable benefit to these patients. The drugs used, including cyclophosphamide, doxorubicin, vincristine, etoposide, and ifosfamide, produced good results.40 The small number of reported cases makes evaluation of the different therapeutic modalities difficult. Therefore, no comparable data exist for orbital Ewing sarcoma/pPNETs until now.

Ewing sarcoma/pPNET rarely affects the orbit. It is prevalent among the children with no gender proclivity. It progresses rapidly with poor prognosis, therefore accurate diagnosis must be made as soon as possible according to immunohistochemical analysis and cytogenic findings. Appropriate treatment consists of local resection, radiotherapy, and adjunctive chemotherapy.

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Reported Cases of Primary Ewing’s Sarcoma Involving the Orbit

Year PublishedGender/Age (Y)Orbital LocationBone InvasionExtension of TumorTreatmentImmuno-histochemistryEMFollow-up
1950M/19Right lateralExcision+RTNANA10 mo (died)
1957M/61RightEnucleation+RTNANA17 d (died)
1965M/< 1Right inferior+Excision+RTNANA14 mo (died)
1985M/14Right medial+Sinuses (OU); cribriform plate (OU)Excision+RT+CTNAGlycogen12 mo (alive)
1986M/52Right lateralExcision+RT+CTGFAPNA6 mo (alive)
1988F/7Left medial, posterior, superior+Intracranial spaceExcision+RT+CTNSEGlycogen45 mo (alive)
1988M/6Right roof, medial, lateral+Sinuses (OU); cranial fossa (OU)Biopsy+RT+CTVimentinGlycogen9 mo (alive)
1993F/10Left superior, lateral+Cranial fossa (OS); apex of temporal fossa (OS)Excision+RT+CTNANA18 mo (alive)
1994F/10Lateral+Exicision+RT+CTCK, EMA, NSE+9 mo (alive)
1999M/2Bilateral medial+Sinuses (OU)Biopsy+CT+RTPAS, NSENA24 mo (alive)
1999F/43Left supranasal+Ethmoid sinuses (OS)Excision+RT+CTNSENA14 mo (alive)
1999M/28Right lateralExcisionNSE, vimentinNA15 mo (alive)
2000M/2Right supratemporal+L1 vertebraExcision+RT+CTNeurosecretory granules17 mo (died)
2000M/7Left superior+Lung; cranial fossa (OS)Excision+RT+CTNA12 mo (died)
2000F/5Right lacrimal gland intraconalExcision+CTSynaptophysinNeurosecretory granules4 y (alive)
2001F/17Right roof+Anterior cranial fossa (OD); temporal fossaExcision+CTPASNA1 y (alive)
2002F/17Right intraconalLiverExcision+RT+CTNSE, EMANeurosecretory granules5 y (alive)
2003F/12Right superotemporal+Excision+RTNegativeNegative6 mo (alive)
2009M/22Left lateral+Left temporal muscleExcision+RT+CTMIC-2Neurosecretory granules2 y (died)
Current case (2010)M/6Right inferotemporal+Infratemporal fossa (OD) masseteric spaceExcision+RT+CTPASNA14 mo (died)
Authors

From the Southwest Hospital, Southwest Eye Hospital, Third Military Medical University, Chongquing, China.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Yuli Yang, PhD, Southwest Hospital, Southwest Eye Hospital, Third Military Medical University, No. 30 Gaotanyan Main Street, Shapingba District, Chongqing, 400038 China. E-mail: yangyuli1979@yahoo.com.cn

10.3928/01913913-20110712-05

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