General
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• Clonal proliferation of Langerhans cells
• Thyroid involvement is rare
• Isolated or part of disseminated disease

Epidemiology
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• Adults more than children, reported in both
• No sex predilection

Clinical features
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• Thyroid mass or enlargement, must rule out malignant lesions
• Thyroid function may be affected depending on degree of involvement

Diagnosis
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• Combination of clinical, radiologic, cytologic and histopathologic features
• Immunohistochemistry and electron microscopy may play a role

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

Case reports
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• 5 month old girl with isolated Langerhans cell histiocytosis of thyroid (Eur J Pediatr 2007;166:1151)
• 19 year old woman with Langerhans cell histiocytosis involving thyroid and parathyroid glands (Mod Pathol 2001;14:111)
• 27 year old man (Int J Clin Exp Pathol 2014;7:1229)
• 28 year old women with masses simulating thyroid carcinoma (Jpn J Clin Oncol 1997;27:180, Hum Pathol 1988;19:239)
• 37 year old man with Langerhans cell histiocytosis of thyroid and multiple cervical lymph node involvement (Case Rep Pathol 2014;2014:184237)
• 54 year old woman (J Clin Endocrinol Metab 2015;100:15)
• Langerhans cell histiocytosis and papillary thyroid carcinoma (Cytopathology 2014 Mar 25 [Epub ahead of print], Rev Clin Esp (Barc) 2014;214:e19)
• Langerhans cell histiocytosis presenting as hypothyroid goiter (BMJ Case Rep 2014;2014)

Treatment
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• Chemotherapy and surgery

Micro description
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• Patchy to extensive involvement of thyroid gland, with follicle invasion and destruction
• May show extrathyroid involvement
• Langerhans cells have moderate eosinophilic cytoplasm and oval nuclei with grooves and a thin nuclear membrane
• Also eosinophils, lymphocytes, multinucleated histiocytes

Micro images
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Langerhans cell centrally located

High cellularity

Diffuse infiltration of thyroid gland

Involvement of parathyroid gland

 

Nests of tumor cells and eosinophils

CD1a

S100

Cytology description
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• Smears contain numerous histiocytic cells with moderate to abundant pale eosinophilic cytoplasm, ovoid to rounded vesicular nuclei, nuclear grooves
• Moderate to marked pleomorphism is seen
• Also eosinophils, lymphocytes, giant cells
• Normal thyroid follicular cells and histiocytes are present (Acta Cytol 2004;48:278)

Positive stains
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• S100, CD1a, Langerin (Pathol Res Pract 2009;205:433)

Negative stains
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• Thyroglobulin, CK

Electron microscopy description
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• Birbeck Granules: tennis racquet or zipper-shaped structures, 33 nm wide with central serrated line

Differential diagnosis
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• Anaplastic / undifferentiated carcinoma
• Granulomatous thyroiditis
• Lymphocytic thyroiditis
• Lymphoma
• Medullary thyroid carcinoma
• Papillary thyroid carcinoma

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