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Langerhans cell histiocytosis (LCH) can involve thyroid in multisystem disease or present with LCH-induced goiter as a single site disease. It occurs most often in children or young adults, although it can affect a wide age-group. Isolated thyroid involvement does not develop into systemic disease and usually has good prognosis.

Histologically, the thyroid involvement in LCH may be focal or diffuse. Clusters of Langerhans cells are seen with intermixed eosinophils. The changes in the surrounding thyroid can range from chronic lymphocytic thyroiditis to papillary thyroid carcinoma. Diagnosis can be made on fine needle aspirate and confirmed with the help of CD1a and langerin (CD207) positivity. Treatment usually consists of surgery.

Image courtesy of: Dr. Siba El Hussein; used with permission.

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