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WHO Classification of Tumours
Langerhans cell histiocytosis
Thyroid gland


Definition

Langerhans cell histiocytosis (LCH) is a rare clonal neoplastic proliferation of Langerhans-type cells that express CD1a, langerin and S100 protein, and shows Birbeck granules by ultrastructural examination
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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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Children are most frequently affected. There is a marked predilection for males (M:F ratio 3.7:1) and whites of northern European descent while the disease is uncommon in blacks. There is a high concordance rate for identical twins. The clinical course is generally related to the number of organs affected at presentation

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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



. Although the etiology remains unknown, considerations include a neoplastic process
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Willman CL (1994)
Detection of clonal histiocytes in Langerhans cell histiocytosis: biology and clinical significance.
Br J Cancer Suppl 23: S29-33



, a possible viral origin
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Leahy MA, Krejci SM, Friednash M, Stockert SS, Wilson H, Huff JC, Weston WL, Brice SL (1993)
Human herpesvirus 6 is present in lesions of Langerhans cell histiocytosis.
J Invest Dermatol 101: 642-5




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McClain K, Weiss RA (1994)
Viruses and Langerhans cell histiocytosis: is there a link?
Br J Cancer Suppl 23: S34-6



, or an abnormal proliferative process
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Mahzoon S, Wood MG (1980)
Multifocal eosinophilic granuloma with skin ulceration. Histiocytosis X of the Hand-Schüller-Christian type.
Arch Dermatol 116: 218-20



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> Synonyms:
Histiocytosis X, eosinophilic granuloma (solitary lesion)
Hand-Schüller-Christian syndrome (multiple lesions)
Letterer-Siwe disease (if disseminated or visceral involvement).

Isolated Langerhans cell histiocytosis in the thyroid gland is very rare

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Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



. Most patients have been less than 20 years of age. There is no gender predilection.