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

Meyel, S.W.F. van; Verdijk, R.M.; Therssen, N.; Paridaens, D.

doi:doi:10.1097/IOP.0000000000002752

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 orbit^³ 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...Show more 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 orbit^³ 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.
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A rare case of subconjunctival nerve sheath myxoma presenting as orbital fat prolapse