Orbital neuromuscular choristoma (benign triton tumor) presenting as a painful blind eye: A rare case report

ABSTRACT

Neuromuscular choristoma (NMC), also known as benign Triton tumor, is an exceptionally rare benign peripheral nerve lesion characterized by the presence of mature skeletal muscle fibers intimately admixed with nerve tissue. Most reported cases occur in childhood and involve major peripheral nerves; orbital involvement in adults is exceedingly uncommon. We report a rare case of orbital NMC in a 25-year-old female who presented with progressive pain and complete visual loss, ultimately requiring orbital exenteration. Histopathological examination and immunohistochemistry (IHC) confirmed NMC with associated desmoid-type fibromatosis. This case highlights the diagnostic challenges posed by this rare entity and underscores the importance of recognizing its characteristic histomorphology to avoid misdiagnosis and inappropriate management.