The Spectrum of Ophthalmic PathologIC Manifestations of the Histiocytoses
AM Bartlett, CT Welsh, A Duong. Medical University of South Carolina, Charleston, SC
Background: Ophthalmic involvement by the histiocytoses most commonly include Langerhans cell histiocytosis (LCH) and non-LCH proliferations such as juvenile xanthogranuloma (JXG) and extranodal Rosai-Dorfman disease (RDD). There are many parallels between these histiocytic lesions other than just the obvious overlaps in histology. Many of the cranial lesions in all three diseases will be solitary and will regress. All may affect skin and soft tissue. Cranial lesions in both LCH and RDD are frequently associated with bone. The differential diagnosis for all three includes many other inflammatory, reactive, and neoplastic lesions. They may be distinguished from each other by their immunohistochemical (IHC) staining patterns. All three have the potential for more widespread involvement which may require more than the minimal intervention typical for these cranial lesions, and the patterns of involvement vary among them, so the specific diagnosis will lead to the correct clinical investigation.
Design: Cases during the years 1995-2008 were searched for histiocytic lesions confined to the orbit/periorbit. The clinical aspects and histologic as well as immunohistochemical features that distinguished among the different types of histiocytoses were evaluated.
Results: Sixteen cases of orbital/periorbital histiocytoses were reviewed. There were six patients with LCH, six with JXG, two with RDD, and two patients with granuloma annulare. The age range was from 4 to 47 years. The granuloma annulare and JXG cases involved only skin. The LCH involved skin and bone. The patients in all three cases of RDD were female, African-American, and demonstrated entirely extranodal disease with multifocal and/or penetrating bone involvement.
Conclusions: Solitary bone lesions in LCH may be simply curetted out and rarely recur. JXG usually regresses spontaneously. Extranodal RDD may be seen in addition to nodal disease, or less often (as in our cases) is exclusively extranodal. There is controversy in the literature regarding cranial cases of RDD as to whether they are considered high risk, which we will discuss. While the literature suggests that LCH in the orbit is rare in adults, our six cases include two adults, which will expand the clinical spectrum. Clinical decision-making regarding treatment, followup and the value of staging require biopsy of these lesions. We will discuss the morphologic and immunohistochemical differentiation between these entities, and development of a definitive diagnosis.
Tuesday, March 10, 2009 9:30 AM
Poster Session III # 216, Tuesday Morning