Primary Orbital Ewing Sarcoma Research Articles

Challenges in primary orbital Ewing sarcoma: A case report

Primary Orbital Ewing Sarcoma (POES) is an exceptionally uncommon cancer. We present a case study of POES in a young woman that highlights the intricate diagnostic process, therapeutic interventions, and the essential role of a multidisciplinary healthcare team.

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

PurposeThe purpose of this study is to discuss the clinical presentation, management, and outcomes of patients with primary orbital Ewing sarcoma family of tumors (ESFTs).Patients and methodsRetrospective study of 12 patients with biopsy-proven primary orbital ESFT.ResultsThe mean age at presentation of primary orbital ESFT was 12 years (median, 8 years; range, 5 months to 28 years). There were seven (58%) females and five (42%) males. The presenting complaints included proptosis (n=10; 83%) and swelling in the upper eyelid (n=2; 17%). The mean duration of symptoms was 9 weeks (median, 5 weeks; range, 2-24 weeks). Tumor epicenter was located in the superior orbit (n=6; 50%), lateral orbit (n=3; 25%), inferior orbit (n=2; 17%), and medial orbit (n=1; 8%). Computed tomography of the orbits revealed predominant bony lesion (n=10; 83%) or isolated soft tissue/extraosseous lesion (n=2, 17%). At presentation, extraorbital extension was noticed in 10 patients including intra cranial extension (n=7; 58%), extension into temporal fossa (n=4; 33%), nasal cavity (n=2; 17%), maxillary sinus (n=2; 17%), and ethmoid sinus (n=1, 8%). Systemic metastases at presentation was detected in five (42%) patients involving the bone marrow (n=4; 33%), kidney (n=1; 8%), and retroperitoneal lymphnode (n=1; 8%). Multi-modal treatment including a combination of neoadjuvant chemotherapy, excision biopsy/debulking, and/or radiotherapy was given. Over a mean follow-up period of 21 months (median, 7 months; range, 1-152 months), disease-related death occurred in 11 (92%) cases.ConclusionPrimary orbital ESFT is aggressive at presentation and is associated with poor prognosis.

CT and multimodal imaging findings of primary orbital Ewing's sarcoma involving the middle cranial fossa: a case report

This report details the CT and MR imaging findings of a primary orbital Ewing’s sarcoma case involving the middle cranial fossa in a 13-year old boy. CT showed an ill-circumscribed homogeneous soft-tissue density mass with needle-like bone reaction. On MRI, the mass showed homogeneous iso-intensity on T1WI, heterogeneous hyper-intensity on T2WI, and marked homogeneous enhancement. Meanwhile, the mass was hyper-intense on DWI, and ADC value was 575×10-6 mm2/s. The mass showed as high rCBV and rCBF, prolonged MTT based on DCS-PWI, and wash-out pattern of TIC derived from the DCE-MRI. Our case suggests that functional MRI modalities, including DWI, DSC-PWI or DCE-MR, could provide additional information for differential diagnoses. Both CT and MRI should be performed and comprehensively analyzed for limiting differential diagnoses, determining lesion extension and facilitating operative approach.

Primary orbital Ewing's sarcoma presenting with local recurrence to maxillary sinus shortly after tumor resection

Ewing's sarcoma (ES) is a malignant, small-round-cell neoplasm that normally affects the long bones of the limbs or the pelvis. Primary orbital ES of the skull has been considered extremely rare. We describe the case of a 19-year-old female patient with primary ES originating from the inferior orbital rime and, shortly after tumor resection, local recurrence to the maxillary sinus.

Primary Orbital Ewing Sarcoma

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.

Primary Ewing’s sarcoma of the orbit: case report

A 22-year-old man presented with tenderness and swelling of the left lateral part of the orbit. Computed tomography revealed a left intraorbital mass measuring 3 cm x 3 cm involving the left lateral wall of the orbit and the greater wing of the left sphenoid bone. Magnetic resonance imaging revealed that the intraorbital mass was extraneuroaxial. During surgery, the tumor was seen to arise from the lateral wall of the orbit and infiltrate into the left temporal muscle. Following the surgery, the patient was administered radiation therapy for the whole cranium and chemotherapy for the residual tumors. However, the tumor recurred, and the patient died about 2 years following the first surgery because the tumor had metastasized to the lung. On light microscopy, the tumor cells were closely packed with uniform, small, and round cells. Immunohistochemical studies showed that the tumor cell membrane stained positive for MIC2. Furthermore, the MIB-1 labeling index was 36.2%. On electron microscopy, small quantities of cytoplasm containing glycogen accumulations without neurosecretory granules and neurofilaments were observed. Based on these results, the tumor was diagnosed to be primary Ewing's sarcoma. Primary orbital Ewing's sarcoma of the skull has been considered to be extremely rare, and a review of the literature was performed.

Primary orbital Ewing's sarcoma presenting with unilateral proptosis

Primary orbital Ewing's sarcoma presenting with unilateral proptosis

Primary Orbital Ewing Sarcoma in a Middle-Aged Man

A 54-year-old man presented with a painful left proptosis and a soft tissue mass at the inferolateral aspect of the left orbit with bone involvement. There was no clinical or investigational evidence of systemic disease. Both light microscopy and immunohistochemistry were required for the diagnosis of Ewing sarcoma. After failure of chemotherapy alone, management included extensive surgical excision and postoperative adjuvant radiotherapy and chemotherapy. Despite intervention, the patient died of widespread metastatic disease 17 months after initial presentation. Although rare, Ewing sarcoma should be included in the differential diagnosis of uniform round cell orbital tumors in adults.