• Rare to involve thyroid, even with multisystemic involvement

• Poor prognosis if systemic disease and thyroid involvement (Mod Pathol 1996;9:145)

• 5 month old girl with isolated thyroid involvement (Eur J Pediatr 2007;166:1151)
• 19 year old woman with parathyroid and thyroid involvement (Mod Pathol 2001;14:111)
• 28 year old woman with isolated thyroid involvement (Hum Pathol 1988;19:239)
• 28 year old woman with thyroid and gingival involvement (Jpn J Clin Oncol 1997;27:180)

• Diffuse or focal involvement by Langerhans cells (histiocytes with bean-shaped, folded nuclei)
• Also eosinophils, destruction of thyroid follicles, background of lymphocytic thyroiditis

Diffuse infiltration of thyroid gland

Involvement of parathyroid gland

Nests of tumor cells and eosinophils

CD1a

S100

• Isolated, loose aggregates and histiocyte-like cells with grooved nuclei mixed with eosinophils, small lymphocytes, multinucleated giant cells and foamy histiocytes (Acta Cytol 2004;48:278)

• CD1a, S100, lysozyme; also Langerin (Pathol Res Pract 2009;205:433)

• Birbeck granules: intracytoplasmic tennis racquet shaped structures with a central zipper like striation

• Poorly differentiated carcinoma

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