Teaching casesImmunocytochemical investigation of Langerin (CD207) is a valuable adjunct in the cytological diagnosis of Langerhans cell histiocytosis of the thyroid
Introduction
Langerhans cell histiocytosis (LCH, formerly referred to as histiocytosis X) is an uncommon disease of hitherto unknown pathogenesis [5]. The entity shows numerous different clinical manifestations and has been subcategorized into three distinct forms: (1) eosinophilic granuloma, a benign disease involving the skeletal system and lungs [14]; (2) Hand-Schüller-Christian disease, usually affecting children younger than 10 years presenting with diabetes insipidus, exophtalmos, hepatosplenomegaly, and bony lesions [1], [11]; and (3) Abt-Letterer-Siewe disease, the rarest and most aggressive form of Langerhans histiocytic proliferations affecting children under 2 years with visceromegaly, lymphadenopathy, bile duct damage, and neuropsychological deficits [7]. Attempts to outline genetic, infectious, or environmental etiological factors that cause LCH have failed [1]. Recently, monoclonality was demonstrated and supports a neoplastic origin of LCH [17]. Despite being a multisystemic disease, involvement of the thyroid gland is rare and may be either isolated or presented as part of a multifocal process. Involvement of the thyroid gland can be focal or diffuse and results in organ enlargement, occasionally accompanied by nodular goiter, lymphocytic thyreoiditis, or papillary carcinoma [4].
The principal cell present in all LCH forms is a non-pigmentary dentritic cell of the monocytic lineage residing in the epidermis [5]. Langerhans cells (LC) resemble other monocytic elements, but show a vesicular nucleus with a groove parallel to the long axis and vacuolated cytoplasm (“coffee-bean-appearance”) [8]. By electron microscopy, characteristic linear rod-shaped cytoplasmic inclusions are demonstrated (Birbeck granules) [13]. Moreover, numerous eosinophilic granulocytes are often found in LCH lesions. LC can be demonstrated with antibodies directed against CD40, CD52, CD154, CD1a, and S-100 protein [10]. Langerin is a recently identified 40 kDa glycoprotein, which is selectively expressed on both the surface and the cytoplasm of immature dentritic cells/Langerhans cells [16]. Antibodies directed against this protein are commercially available and can be used for immunocytochemical characterization of LC. We report a case of a 28-year-old male patient, who presented with LCH with thyroid involvement, which was diagnosed in a fine needle aspiration (FNA) biopsy based on both cytomorphology and immunocytochemistry.
Section snippets
Case report
A 28-year-old male patient was referred to the department of hematooncology in another hospital, presenting with progressive hearing loss and skin lesions. Apart from histologically confirmed skin involvement, LCH infiltration of the left mastoid and petrous bone was suspected, but no biopsy had been obtained from these lesions. Cranial magnetic resonance imaging (MRI), high-resolution computed tomography (CT) of the chest and abdomen, and skeletal scintigraphy did not reveal any additional
Material and methods
The FNA material was obtained from the right thyroid lobe. The specimens were worked up by the Thin-Prep® (Cytyc GmbH, Frankfurt am Main, Germany) technique and stained according to Papanicolaou.
Results
The specimens showed inconspicuous thyreocytes and a sparse lymphocytic background. Eosinophilic granulocytes were occasionally seen. In addition, relatively large cells were observed with longitudinal nuclei with a moderately dense chromatin pattern and nuclear grooves (“coffee-bean-like” appearance), as well as foamy cytoplasm, suspicious to be cells of Langerhans cell lineage (Fig. 2). Focally, mitoses were observed in these cells (Fig. 2, inset). To confirm the diagnosis,
Discussion
Involvement of the thyroid gland by Langerhans cell histiocytosis either as an isolated manifestation or as part of multisystemic disease is very rare [2]. Among adults, LCH involvement of the thyroid is usually isolated, and when it presents as part of a multisystemic disease, it is usually discovered at autopsy [15], [16]. Ultrasound findings are rather unspecific and resemble subacute thyroiditis de Quervain [9]. Computed tomography may show uncharacteristic diffuse thyroid enlargement with
Conflict of interest
The authors declare that there is no conflict of interest.
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