Langerhans Cell Sarcoma of Thyroid: A Rare Differential for a Thyroid Swelling

Abstract

Langerhans Cell Sarcoma (LCS) is a rare disease arising from antigen-presenting cells. The literature on LCS is largely limited to case series and only 67 such cases have been reported till date. We report a case of 27-year-old female presenting as a thyroid swelling. Computerized tomography (CT) scan showed diffuse enlargement of thyroid and patient underwent total thyroidectomy. Final diagnosis on histopathology and Immunohistochemistry (IHC) was langerhans cell sarcoma of the thyroid. Patient was advised adjuvant chemotherapy, however she defaulted and later presented to us with a disseminated disease. This is the 18th case of head and neck LCS to be reported and the first case of primary thyroid origin in the world literature. The diagnosis of LCS is by combination of histopathology and IHC and multi- modality treatment offers the best chance of cure.

Keywords

• immunohistochemistry
• lymphadenopathy
• rare sarcomas