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. 1996 Dec;80(12):1083–1086. doi: 10.1136/bjo.80.12.1083

Primary localised amyloidosis of the orbit.

I E Murdoch 1, T J Sullivan 1, I Moseley 1, P N Hawkins 1, M B Pepys 1, S Y Tan 1, A Garner 1, J E Wright 1
PMCID: PMC505708  PMID: 9059275

Abstract

AIMS/BACKGROUND: Primary localised amyloidosis is rarely encountered in the orbit. The typical clinical and radiological appearances have not been clearly established, in particular the single site deposition of amyloid has never been proved. METHODS: Six cases were reviewed in detail and their clinical and radiological characteristics are described here. Four patients had scintigraphy with 123I serum amyloid P component and one patient had typing of the amyloid fibrils. RESULTS: All the patients had a firm mass in the upper orbit with a predilection for the region of the lacrimal gland. Computed tomography showed a homogeneous mass with thickening and irregularity of the adjacent bone and/or calcification within the mass. None was associated with systemic disease. Scintigraphy with 123I serum amyloid P component demonstrated that the amyloid was confined to the orbit. In one patient the amyloid fibrils were derived from an IgG4 heavy chain constant domain. The lesions were partially excised with subsequent clinical stability over 6 months to 18 years in all but one patient who had continued enlargement of the lesion. CONCLUSION: There is a characteristic clinical and radiological pattern for primary localised amyloidosis of the orbit. The disease process is truly local and not part of a systemic process. A majority respond to simple debulking with subsequent observation.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Cohen M. M., Lessell S. Amyloid tumor of the orbit. Neuroradiology. 1979 Sep 26;18(3):157–159. doi: 10.1007/BF00346872. [DOI] [PubMed] [Google Scholar]
  2. Conlon M. R., Chapman W. B., Burt W. L., Larocque B. J., Hearn S. A. Primary localized amyloidosis of the lacrimal glands. Ophthalmology. 1991 Oct;98(10):1556–1559. doi: 10.1016/s0161-6420(91)32088-8. [DOI] [PubMed] [Google Scholar]
  3. EASTON J. A., SMITH W. T. Non-specific granuloma of orbit ("orbital pseudotumour"). J Pathol Bacteriol. 1961 Oct;82:345–354. doi: 10.1002/path.1700820212. [DOI] [PubMed] [Google Scholar]
  4. Gean-Marton A. D., Kirsch C. F., Vezina L. G., Weber A. L. Focal amyloidosis of the head and neck: evaluation with CT and MR imaging. Radiology. 1991 Nov;181(2):521–525. doi: 10.1148/radiology.181.2.1924798. [DOI] [PubMed] [Google Scholar]
  5. Groniowski J., Bernardczykowa A., Norn M. S. Orbital amyloidosis. Acta Ophthalmol (Copenh) 1965;43(5):725–729. doi: 10.1111/j.1755-3768.1965.tb00344.x. [DOI] [PubMed] [Google Scholar]
  6. HANDOUSA A. Localized intra-orbital amyloid disease. Br J Ophthalmol. 1954 Aug;38(8):510–511. doi: 10.1136/bjo.38.8.510. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Hawkins P. N., Lavender J. P., Pepys M. B. Evaluation of systemic amyloidosis by scintigraphy with 123I-labeled serum amyloid P component. N Engl J Med. 1990 Aug 23;323(8):508–513. doi: 10.1056/NEJM199008233230803. [DOI] [PubMed] [Google Scholar]
  8. Hawkins P. N. Studies with radiolabelled serum amyloid P component provide evidence for turnover and regression of amyloid deposits in vivo. Clin Sci (Lond) 1994 Sep;87(3):289–295. doi: 10.1042/cs0870289. [DOI] [PubMed] [Google Scholar]
  9. Holmström G. E., Nyman K. G. Primary orbital amyloidosis localised to an extraocular muscle. Br J Ophthalmol. 1987 Jan;71(1):32–33. doi: 10.1136/bjo.71.1.32. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Howard G. M. Amyloid tumours of the orbit. Br J Ophthalmol. 1966 Jul;50(7):421–425. doi: 10.1136/bjo.50.7.421. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Jensen O. A. Bilateral amyloidosis of the orbit. A case report with pathogenetical considerations. Ophthalmologica. 1976;173(1):70–78. doi: 10.1159/000307822. [DOI] [PubMed] [Google Scholar]
  12. Kaiser-Kupfer M. I., McAdam K. P., Kuwabara T. Localized amyloidosis of the orbit and upper respiratory tract. Am J Ophthalmol. 1977 Nov;84(5):721–728. doi: 10.1016/0002-9394(77)90391-9. [DOI] [PubMed] [Google Scholar]
  13. Knowles D. M., 2nd, Jakobiec F. A., Rosen M., Howard G. Amyloidosis of the orbit and adnexae. Surv Ophthalmol. 1975 May-Jun;19(6):367–384. [PubMed] [Google Scholar]
  14. Lawton A. W., Leone C. R., Jr, Hunter D. M. Optic nerve pseudomeningioma secondary to localized amyloidosis. Ophthal Plast Reconstr Surg. 1989;5(1):52–55. doi: 10.1097/00002341-198903000-00009. [DOI] [PubMed] [Google Scholar]
  15. Levine M. R., Buckman G. Primary localized orbital amyloidosis. Ann Ophthalmol. 1986 May;18(5):165–167. [PubMed] [Google Scholar]
  16. Liesegang T. J. Amyloid infiltration of the levator palpebrae superioris muscle: case report. Ann Ophthalmol. 1983 Jul;15(7):610–613. [PubMed] [Google Scholar]
  17. Motta A. O., Han J. S., Levine M., Benson J. E. Primary amyloid tumor of the lacrimal gland: CT findings. J Comput Assist Tomogr. 1983 Dec;7(6):1079–1080. doi: 10.1097/00004728-198312000-00027. [DOI] [PubMed] [Google Scholar]
  18. Nehen J. H. Primary localized orbital amyloidosis. Acta Ophthalmol (Copenh) 1979 Apr;57(2):287–295. doi: 10.1111/j.1755-3768.1979.tb00493.x. [DOI] [PubMed] [Google Scholar]
  19. RICHLIN J. J., KUWABARA T. Amyloid disease of the eyelid and conjunctiva. Arch Ophthalmol. 1962 Feb;67:138–142. doi: 10.1001/archopht.1962.00960020140006. [DOI] [PubMed] [Google Scholar]
  20. Raab E. L. Intraorbital amyloid. Br J Ophthalmol. 1970 Jul;54(7):445–449. doi: 10.1136/bjo.54.7.445. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Radnót M., Lapis K., Fehér J. Amyloid tumor in the lachrymal gland. Ann Ophthalmol. 1971 Jul;3(7):727–passim. [PubMed] [Google Scholar]
  22. Savino P. J., Schatz N. J., Rodrigues M. M. Orbital amyloidosis. Can J Ophthalmol. 1976 Jul;11(3):252–255. [PubMed] [Google Scholar]
  23. Schubert E. Amyloidtumor der Orbita. Beitrag zur Klinik, Histopathologie und Pathogenese. Klin Monbl Augenheilkd. 1972 Apr;160(4):467–468. [PubMed] [Google Scholar]
  24. Tan S. Y., Murdoch I. E., Sullivan T. J., Wright J. E., Truong O., Hsuan J. J., Hawkins P. N., Pepys M. B. Primary localized orbital amyloidosis composed of the immunoglobulin gamma heavy chain CH3 domain. Clin Sci (Lond) 1994 Nov;87(5):487–491. doi: 10.1042/cs0870487. [DOI] [PubMed] [Google Scholar]
  25. Tan S. Y., Pepys M. B. Amyloidosis. Histopathology. 1994 Nov;25(5):403–414. doi: 10.1111/j.1365-2559.1994.tb00001.x. [DOI] [PubMed] [Google Scholar]

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