Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2003:101:93-103; discussion 103-5.

Eosinophilic granuloma of the orbit: a paradox of aggressive destruction responsive to minimal intervention

Affiliations

Eosinophilic granuloma of the orbit: a paradox of aggressive destruction responsive to minimal intervention

Gerald J Harris et al. Trans Am Ophthalmol Soc. 2003.

Abstract

Purpose: To describe the findings and outcomes in eosinophilic granuloma (unifocal Langerhans cell histiocytosis [LCH]) of the orbit, and to explain the paradox of aggressive bone destruction responsive to minimal intervention.

Methods: Retrospective, consecutive, interventional case series of patients treated from 1985 through 2001. Minimum inclusion criteria were demonstration of CD1a positivity or Birbeck granules, treatment by a single surgeon, systemic evaluation by a pediatric oncologist, and follow-up of 12 months. A pathogenetic construct was assembled from general LCH concepts and the specific orbital findings.

Results: Seven patients met study criteria. All were male, 2 to 16 years of age. All had eyelid or forehead swelling and osteolytic defects, with symptoms of 2 to 6 weeks' duration. All underwent incisional biopsy, with frozen-section examination suggestive of LCH in 6 of 7 cases. The 2 earliest patients received low-dose irradiation after simple biopsy. The 5 most recent patients had subtotal curettage at the time of biopsy; 4 of 5 received simultaneous intralesional corticosteroid injection. In all cases, systemic evaluation showed no other focus of LCH, reossification was timely, and no local recurrence or additional focus was noted in follow-up of 1 to 17 years.

Conclusions: Transient immune dysfunction may provoke the cytokine-mediated proliferation of pathologic Langerhans cells within the hematopoietic marrow of the anterolateral frontal bone. These cells cause osteolysis through elaboration of interleukin-1 and prostaglandin E2. Corticosteroids can inhibit the mediators. We recommend incisional biopsy, frozen-section provisional diagnosis, subtotal curettage, intralesional corticosteroid instillation, postoperative systemic evaluation, and long-term follow-up.

PubMed Disclaimer

Similar articles

Cited by

References

    1. Hematol Oncol Clin North Am. 1998 Apr;12(2):287-305 - PubMed
    1. Nature. 1983 Nov 24-30;306(5941):378-80 - PubMed
    1. Hematol Oncol Clin North Am. 1998 Apr;12(2):385-406 - PubMed
    1. J Pediatr Ophthalmol Strabismus. 1998 Jul-Aug;35(4):234-6 - PubMed
    1. Int J Radiat Biol. 1998 Sep;74(3):367-78 - PubMed

Publication types

MeSH terms