Surgical resection of orbital primitive neuroectodermal tumor without adjuvant chemotherapy

Primitive neuroectodermal tumor (PNET) of the orbit is a very rare entity. We report a case of a 10-year-old boy having progressive protrusion of the eyeball, restricted ocular motility, ptosis, and ocular pain for 2 months. On examination, a fixed, nontender, firm, hyperemic, nonpulsatile globular mass was palpated in the superomedial quadrant of the orbit. Computed tomography scans of the orbit depicted a well-defined, soft tissue density lesion within the orbit without evidence of any bone defect or erosion. Surgical resection of the encapsulated lesion through anterior orbitotomy followed by histopathology and immunohistochemistry examination confirmed the diagnoses of PNET. The patient has been on regular follow-up for 2 years and has shown no sign of recurrence.