Abstract
Neuroendocrine tumors most frequently involve the gastrointestinal tract and bronchopulmonary system. Few cases of presumed primary neuroendocrine tumors in the orbit have been reported so far and most of the orbital cases are actually metastatic.
We describe the unusual occurrence of this tumor in the orbit of a 16-year-old boy. The lesion was initially thought to be primary; however, the diagnosis of a metastatic orbital lesion was later supported by the histopathological appearance of his orbital biopsy, characteristic immunohistochemical profile and the presence of a primary mediastinal tumor. The patient did not have any symptoms suggestive of a carcinoid syndrome during the course of his disease. Unfortunately, tests showed lymph node involvement and distant metastatic lesions and he died from these a few months later while on palliative therapy.