Neurothekoma of orbit

Abstract

Myxoid neurothekeoma, also known as dermal nerve sheath myxoma, is a rare tumor of presumed nerve sheath origin, which affects adolescents and young adults. A middle-aged woman came with the complaint of inferior displacement of left eyeball for 4 years. She was diagnosed previously as pseudotumor and treated with systemic steroids twice before, following which symptoms were relieved for short duration. There is a history of ptosis surgery done in childhood. Orbit examination showed firm, nonmovable, nonreducible mass below superior orbital margin. Computed tomography scan of orbit showed a round well-circumscribed, isodense mass located in the suprabulbar extraconal space. Surgical excision of mass through anterior orbitotomy was performed. One large size nodule with rest small daughter nodules was removed. Histopathological examination revealed myxoid lobules and fascicles of spindle cells with no atypia, suggestive of nerve sheath myxoma. It is so far rarest tumor in the orbit.