Eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease comprise this category. The disease can be focal as in eosinophilic granuloma (80% of cases), or involve multiple skeletal and extraskeletal sites as in Letterer-Siwe disease. The histologic and cytologic findings are similar in all subtypes. The tumor is made up predominantly of two components, Langerhans cells and eosinophils in varying quantities. Other inflammatory cells may also be present. The Langerhan cell is a round-to-oval histiocyte-like cell containing an oval nucleus and clearly demarcated cytoplasm. A prominent nuclear groove, which runs along the long axis, is present in most of these cells (Fig. 20). Cellular atypia as well as mitoses is minimal. Multinucleated giant cells and ordinary macrophages can be present. Eosinophils may be so
prominent in the sample material that the term "eosinophilic abscess" has been used (67,9). Immunohistochemical stains will be positive for S-100 and CD1a (68).
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