title: [Neuromuscular hamartoma of the orbit: case report and discussion of the role of corticosteroids in treatment].
abstract: BACKGROUND
  Neuromuscular hamartoma, also known as neuromuscular choristoma or benign triton tumor, is a rare benign tumor of well-differentiated striated muscle fibers mixed with peripheral nerve fibers. According to our research, this is the sixth case of benign triton tumor of the trigeminal nerve and the third case of isolated orbital location reported in the world literature.
  
  
  OBJECTIVE
  To report a rare case of orbital neuromuscular hamartoma and discuss the role of corticosteroids in the treatment of these lesions for which surgical excision is often difficult.
  
  
  METHODS
  A 47-year-old woman, with a history of tuberculous lymphadenitis treated in 2006, presented with a clinical scenario of inflammatory orbitopathy without loss of visual acuity progressing over 20 days. MRI showed a lesion centered on the soft tissues of the infero-lateral right orbit. A biopsy was performed, showing neuromuscular hamartoma on histology. The patient was put on a tapering dose of corticosteroids with clear clinical and anatomical improvement. Orbital CT follow-up was obtained two months after discontinuation of treatment, confirming the disappearance of the tumor mass.
  
  
  CONCLUSIONS
  Hamartoma of the orbit is a very rare entity and may clinically simulate malignant neoplasms; the diagnosis is histologic. Given the difficulties encountered in the resection of these tumors, we believe that corticosteroids might be proposed as an alternative treatment that could modulate inflammation and bring about regression or disappearance of the tumor.
authors.affiliation: Service d'ophtalmologie, hôpital militaire d'instruction Mohamed V, Madinat Al Irfane, Rabat, Maroc. Electronic address: said2214@yahoo.fr.
authors.forename: S
authors.initials: S
authors.lastname: Iferkhass
authors.affiliation: Service d'ophtalmologie, hôpital militaire d'instruction Mohamed V, Madinat Al Irfane, Rabat, Maroc.
authors.forename: A
authors.initials: A
authors.lastname: Elkhoyaali
authors.affiliation: Service d'ophtalmologie, hôpital militaire d'instruction Mohamed V, Madinat Al Irfane, Rabat, Maroc.
authors.forename: S
authors.initials: S
authors.lastname: Chatioui
authors.affiliation: Service d'anatomopathologie, hôpital militaire d'instruction Mohamed V, Madinat Al Irfane, Rabat, Maroc.
authors.forename: A
authors.initials: A
authors.lastname: Elktaibi
authors.affiliation: Service d'ophtalmologie, hôpital militaire d'instruction Mohamed V, Madinat Al Irfane, Rabat, Maroc.
authors.forename: F
authors.initials: F
authors.lastname: Elasri
authors.affiliation: Service d'ophtalmologie, hôpital militaire d'instruction Mohamed V, Madinat Al Irfane, Rabat, Maroc.
authors.forename: K
authors.initials: K
authors.lastname: Reda
authors.affiliation: Service d'ophtalmologie, hôpital militaire d'instruction Mohamed V, Madinat Al Irfane, Rabat, Maroc.
authors.forename: A
authors.initials: A
authors.lastname: Oubaaz
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D005260:Female; D006222:Hamartoma / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009468:Neuromuscular Diseases / Q000175:diagnosis / Q000188:drug therapy; D009916:Orbital Diseases / Q000175:diagnosis* / Q000188:drug therapy*
chemical_list: D000305:Adrenal Cortex Hormones
keywords: Choristome neuromusculaire; Corticosteroids; Corticothérapie; Localisation orbitaire; L’hamartome neuromusculaire; Nerf trijumeau; Neuromuscular choristoma; Neuromuscular hamartoma; Orbital location; Trigeminal nerve