title: Anterior orbit and adnexal amyloidosis.
abstract: OBJECTIVE
  To describe six cases of anterior orbital and adnexal amyloidosis and to report on proteomic analysis to characterize the nature of amyloid in archived biopsies in two cases.
  
  
  METHODS
  The clinical features, radiological findings, pathology, and outcome of six patients with anterior orbit and adnexal amyloidosis were retrieved from the medical records. The biochemical nature of the amyloid was determined using liquid chromatography/mass spectroscopy archived paraffin-embedded tissue in two cases.
  
  
  RESULTS
  Of the six cases, three had unilateral localized anterior orbit and lacrimal gland involvement. Four of the six patients were female with an average duration of 12.8 years from the time of onset to presentation eyelid infiltration by amyloid caused ptosis in five cases. CT scan in patients with lacrimal gland involvement (n = 3) demonstrated calcified deformable anterior orbital masses and on pathological exmaintionamyloid and calcific deposits replaced the lacrimal gland acini. Ptosis repair was performed in three patients with good outcomes. One patient required repeated debulking of the mass and one patient had recurrenct disease. Proteomic analysis revealed polyclonal IgG-associated amyloid deposition in one patient and AL kappa amyloid in the second patient.
  
  
  CONCLUSIONS
  Amyloidosis of the anterior orbit and lacrimal gland can present with a wide spectrum of findings with good outcomes after surgical excision. The nature of amyloid material can be precisely determined in archival pathology blocks using diagnostic proteomic analysis.
authors.affiliation: King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia.
authors.forename: Hailah
authors.initials: H
authors.lastname: Al Hussain
authors.forename: Deepak P
authors.initials: DP
authors.lastname: Edward
mesh_terms: D000328:Adult; D000368:Aged; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D001706:Biopsy; D002853:Chromatography, Liquid; D003229:Conjunctival Diseases / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D007766:Lacrimal Apparatus Diseases / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009916:Orbital Diseases / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D040901:Proteomics; D053719:Tandem Mass Spectrometry; D014057:Tomography, X-Ray Computed
chemical_list: D000682:Amyloid
keywords: Amyloidosis; Lacrimal Gland; Orbit; Pathology