Endocrine Abstracts

Langerhans cell histiocytosis is a disease caused by clonal proliferation of a group of antigen-presenting cells known as Langerhans cells. Its manifestations range from isolated lesions to multisystem disease usually involving bone, skin and the pituitary stalk. Thyroid involvement is rare.

A 44-year old woman presented to the joint thyroid clinic with a short history of neck swelling, confirmed to be a smooth goitre on clinical examination. Initial blood tests results were: TSH 0.24 mu/l (0.30–4.20), T4 8.2 pmol/l (9–19), TPO antibody negative.

A neck ultrasound demonstrated a diffuse thyroid abnormality with generalised hypoechoic echotexture, widespread microcalcification and level 6 lymphadenopathy. No focal lesions were identified. FNA of the thyroid was suggestive but not diagnostic of medullary carcinoma. Carcinoembryonic antigen was 6.5 ug/l (1–5), but calcitonin was undetectable.

Total thyroidectomy was recommended by the MDT. However, dissection of the left lobe was technically challenging and a left hemi-thyroidectomy only was performed as the Recurrent Laryngeal Nerve failed to stimulate post lobectomy. As the pathology was unknown at the time it was decided not to embark upon surgery on the right side.

Histologically, histiocytes were seen on a background of numerous eosinophils and a lymphoid infiltrate. Immunohistochemistry revealed positivity for CD1a and S-100. These features were in keeping with a definitive diagnosis of LCH.

Following surgery she developed multiple skin lesions suggestive of multifocal involvement and more recently she has been found to have pituitary dysfunction (Table 1).

LCH initially presenting in the thyroid as seen in our case is extremely rare. The management of this multisystem disease remains a challenge.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.