title: Langerhans cell histiocytosis of the thyroid: a series of seven cases and a review of the literature.
abstract: Langerhans cell histiocytosis is a rare disorder, with a few reports describing isolated thyroid gland involvement. We report seven cases, which included four females and three males ranging in age from 2 months to 55 years, with a median age of 37 years. Histologically, the cases demonstrated either diffuse or focal involvement of the thyroid gland by Langerhans cell histiocytes, characterized by bean-shaped, lobated, folded nuclei. In association with the histiocytic infiltrate, there was a prominent eosinophilic cellular component, as well as destruction of the thyroid follicles. All cases occurred in a background of lymphocytic thyroiditis. One case demonstrated adenomatoid nodules, whereas another had a microscopic papillary carcinoma. Immunohistochemical staining demonstrated positive reactivity with S-100 protein, lysozyme, and KP-1. Four patients with isolated thyroid disease, treated by surgical resection alone, are alive without systemic disease from 3 to 19 years after initial presentation. The three patients with systemic disease died within 1 year of the initial diagnosis with disease-related complications. Localized disease portends a favorable prognosis as compared to the thyroid involvement as part of systemic disease.
authors.affiliation: Department of Otolaryngic and Endocrine Pathology, Armed Forces Institute of Pathology, Washington, DC, USA.
authors.forename: L D
authors.initials: LD
authors.lastname: Thompson
authors.forename: B M
authors.initials: BM
authors.lastname: Wenig
authors.forename: C F
authors.initials: CF
authors.lastname: Adair
authors.forename: B C
authors.initials: BC
authors.lastname: Smith
authors.forename: C S
authors.initials: CS
authors.lastname: Heffess
mesh_terms: D000328:Adult; D002675:Child, Preschool; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D013959:Thyroid Diseases / Q000378:metabolism / Q000473:pathology*