Langerhans cell histiocytosis of the thyroid gland - A case report

BACKGROUND: The variant of Langerhans histiocytosis commonly encountered in adults is the benign, localized form (eosinophilic granuloma). The more ag gressive or diffuse type (Letterer-Siwe disease) is rare in adults. CASE: A 28-year-old woman presented with enlargement of the thyroid gland t hree years after she had been diagnosed with and placed on treatment for di abetes insipidus. Thyroidectomy was performed following an initial fine nee dle aspiration cytology report of either papillary thyroid carcinoma or Lan gerhans cell histiocytosis. The latter diagnosis was confirmed on histopath ology and immunohistochemical staining for S-100 protein. Intracellular Bir beck granules were also demonstrated by electron microscopy. The disease pr ogressed over a 10-week period to involve the kidneys, resulting in venal d ysfunction. CONCLUSION: Synchronous or metachronous involvement of the hypothalamus and thyroid gland by Langerhans histiocytosis could not be excluded in the pre sent case, with subsequent progression to involve other organs. This was an unusual presentation of the disease in an adult.