Abstract
Background: Primitive neuroectodermal tumors (PNETs) are malignant soft tissue tumors of neuroepithelial origin with an aggressive nature and associated with early disseminated metastasis, resulting in poor prognosis. It occurs more frequently in men of age < 35 years, and in several parts of the body, such as pulmonary tract, nasal cavity, paranasal sinuses, and/or neck. Orbital location is infrequent and has been reported in less than 20 cases in the English literature so far.
 Case presentation: In this paper, we report our experience with a 77 year-old man who was referred with a 2.8 cm mass in the superior-anterior quadrant of his left eye for 2 weeks prior to admission, as well as having a 15-kg weight loss during the past six months. The patient developed rapidly progressive visual loss, decreased eye movements, and conjunctivitis within a week. Thus, the patient underwent orbitotomy and the results of histological examination and immunohistochemistry showed an orbital PNET. Metastasis to frontal sinuses and ethmoid sinuses were also discovered. The patient passed away before referral to the oncologist.
 Conclusion: This report confirms the highly aggressive nature of PNET and reports its rare occurrence in orbital cavity.
Highlights
Primitive neuroectodermal tumors (PNETs) are malignant tumors of neuroectodermal origin, in the Ewing’s sarcoma (EWS) family, and is categorized into central and peripheral primitive neuroectodermal tumor (PNET) based on the presenting site 1
This case is very rare, as PNETs are generally considered a disease of childhood and adolescents, with only a few cases having been reported in adults [8,9]
Orbit is an infrequent location for PNET with less than 20 cases reported in the English literature so far, most of which were reported in children 7 as there have been very few cases in adults
Summary
Primitive neuroectodermal tumors (PNETs) are malignant tumors of neuroectodermal origin, in the Ewing’s sarcoma (EWS) family, and is categorized into central and peripheral PNET based on the presenting site 1. The results of the computed tomography (CT) scan reported a mass in the anterior section of the eye with involvement of ethmoid and frontal sinuses, in addition to erosion in the upper rim of the orbit (Figure 2). These results suggested metastasis as the main diagnosis in this patient. The patient was referred with the results of CT scan after one week and on the second examination, he had severe limitations in eye movement with severe proptosis and hypoglobus, and his visual acuity reached 2/20, he reported no pain. The main factors of the patient expiring included the age of patient, uncontrolled DM, cachexia, and aggressive nature of disease
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