A rare case of subconjunctival nerve sheath myxoma presenting as orbital fat prolapse

Abstract

Nerve sheath myxoma (NSM) is an extremely rare myxoid peripheral nerve sheath tumor with a predilection for the extremities, particularly common in the fingers and knees. Only 14% has been found around the rest of the body.1 The local recurrence rate is high and it has a peak incidence in the fourth decade of life.1 There are no characteristic clinical features of NSM, which is why pathological examination is paramount for diagnosis.2 To our knowledge, only a few cases of NSMs around the eye have been reported in the literature of which only 3 are located in the orbit3 and 4 in the eyelid.2,4–6 Up until recently, NSM was thought to be a myxoid type of neurothekeoma.7 At present, there is a consensus that both are separate entities based on their immunohistochemical characteristics.7 Written informed consent was obtained from the patient for publication of this case report, in accordance with the Declaration of Helsinki.