Langerhans cell histiocytosis of the orbit: five clinicopathologic cases and review of the literature

Abstract

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as unisystem (unifocal or multifocal) or multisystem disease. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. We describe the clinical and histologic spectrum of LCH of the orbit in our five cases. One patient exhibited multifocal unisystem disease; the other four had a localized process. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. Transmission electron microscopy demonstrated characteristic intracytoplasmic Birbeck granules. We review the different ophthalmic manifestations of LCH and treatment strategies. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH should included in the differential diagnosis in tumors of the ocular adnexae, especially in young children.

Figure 1

Clinical appearance of an 8-years-old patient with a superior temporal mass in the left orbit (A). Computed tomography (CT) demonstrating the orbital lesion with bone erosion (B). Histology of the lesion showing infiltrates composed of histiocytes, giant cells (C, arrow; H&E, 80x), eosinophils (D, arrow; H&E, 128x), and lymphocytes. Birbeck granules exhibiting the shape of tennis rackets were detected by transmission electron microscopy (E; TEM, 30000x).

Figure 2

Clinical appearance of a three-years-old patient with an erythematous eyelid swelling (A). CT reavealing the orbital lesion with bone erosion (B). Histology showing sheets histiocytes (inset) intermixed with giant cells, lymphocytes, and a few eosinophils (C; H&E, 40x).

Figure 3

Clinical appearance of a one-years-old patient with a mass in the anterior orbit (A). CT revealing bony erosion in the orbital rim (B), the tibia (C), and the skull (D). Histology showing sheets of histiocytes intermixed with giant cells (arrowhead) and eosinophils (arrow) (E; H&E, 40x).

Figure 4

Clinical appearance of an eight-months-old patient with a right facial erythematous swelling (A). CT showing with a mass in his right temporal inferior orbit (B, C). Histologic picture illustrating the histiocytic infiltrate interspersed with occasional eosinophils (D; H&E, 40x).

Figure 5

Clinical appearance of a nine-years-old patient with a mass in the right upper orbit (A). CT demonstrating the orbital lesion with bone erosion (B). Histology illustrating the Langerhans cell infiltrate with giant cells and eosinophils (C; H&E, 40x). The Langerhans cells were positive for S100 (D; 40x) and CD1a (E; 40x). Birbeck granules were detected by TEM (F; 14000x).