Background: Langerhans cell histiocytosis (LCH) is a monoclonal disease of specialised histiocytes characterised by the proliferation of neoplastic Langerhans cells (LCs) with a varying admixture of mature lymphocytes, eosinophils and plasma cells. LCH commonly occurs in the paediatric population and young adults with the involvement of bone, skin and lymph nodes. LCH has a protracted clinical course with an overall mortality rate of 3%. Primary involvement of the thyroid gland in LCH at presentation is a rare phenomenon that can result in misdiagnosis with consequent mismanagement. Case: Ultrasound-guided fine-needle aspiration cytology (FNAC) of the thyroid was performed in 3 cases at a tertiary cancer centre, including 2 referral cases where the patient presented with the only symptom of progressive thyroid enlargement. These cases were reported initially or on review and the results were correlated with histology/ancillary techniques. A cytological diagnosis of suspicion for LCH was offered in 2 cases at our centre and 1 case was referred to our centre with a diagnosis of suspected papillary thyroid carcinoma. On review of outside smears at our centre, the diagnosis was changed to suspected LCH. The diagnosis was confirmed by immunopositivity for CD68, CD1a and S100 in 1 case and Tru-cut biopsy followed by immunohistochemistry for CD1a, S100, TTF-1 and LCA in the other 2 cases. Conclusion: FNAC was helpful in accurately diagnosing LCH even though the presentation was unusual. Thus, unnecessary surgery was obviated.

Hand A: General tuberculosis. Trans Pathol Soc Philadelphia 1883;16:282.
Nicholson HS, Egeler RM, Nesbit ME: The epidemiology of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998;12:379-384.
Layfield LJ, Bhuta S: Fine needle aspiration cytology of histiocytosis X: a case report. Diagn Cytopathol 1988;4:140-143.
Chu T, Jaffe R: The normal Langerhans cell and the LCH cell. Br J Cancer Suppl 1994;70:S4-S10.
Haupt R, Minkov M, Astigarraga I, Schäfer E, Nanduri V, Jubran R, Egeler RM, Janka G, Micic D, Rodriguez-Galindo C, van Gool S, Visser J, Weitzman S, Donadieu J: Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer 2013;60:175-184.
Sinisi AA, Criscuolo T, Palombini L, Bellastella A, Faggiano M: Thyroid localization in adult histiocytosis X. J Endocrinol Invest 1986;9:417.
Ruco LP, Pulford KAF, Mason DY, Ceccamea A, Uccini S, Pileri S, et al: Expression of macrophage associated antigens in tissue involved by Langerhans' cell histiocytosis (histiocytosis X). Am J Clin Pathol 1989;92:273-279.
Hage C, William CL, Favara BE, Isaacson PG: Langerhan's cell histiocytosis (histiocytosis X): immunophenotype and growth fraction. Hum Pathol 1993;24:840.
Ornvold K, Ralfkiaer E, Carstenson H: Immunohistochemical study of the abnormal cells in Langerhans' cell histiocytosis (histiocytosis X). Virchows Arch A Pathol Anat Histopathol 1990;416:403.
Patten DK, Wani Z, Tolley N: Solitary Langerhans histiocytosis of the thyroid gland: a case report and literature review. Head Neck Pathol 2012;6:279-289.
Sahoo M, Karak AK, Bhatnagar D, Bal CS: Fine needle aspiration cytology in a case of isolated involvement of thyroid with Langerhans cell histiocytosis. Diagn Cytopathol 1998;19:33-37.
Lieberman PH, Jones CR, Steinman RM, Erlandson RA, Smith J, Gee T, Huvos A, Garin-Chesa P, Filippa DA, Urmacher C, Gangi MD, Sperber M: Langerhans cell (eosinophilic) granulomatosis: a clinicopathologic study encompassing 50 years. Am J Surg Pathol 1996;20:519-552.
Malpas JS: Langerhans cell histiocytosis in adults. Hematol Oncol Clin North Am 1998;12:259-268.
Coode PE, Shaikh MU: Histiocytosis X of the thyroid masquerading as thyroid carcinoma. Hum Pathol 1988;19:239.
Thomson LDR: Langerhans' cell histiocytosis isolated to the thyroid gland. Eur Arch Otorhinolaryngol 1996;253:62-65.
Thompson LD, Weing BM, Adair CF, Smith BC, Heffess CS: Langerhans' cell histiocytosis of thyroid: a series of 7 cases and a review of literature. Mod Pathol 1996;9:145-149.
Wenig BM, Heffess CS, Adair CF: Atlas of Endocrine Pathology. Philadelphia, WB Saunders, 1997, pp 136-137.
Herzog KM, Tubbs RR: Langerhans' cell histiocytosis. Adv Anat Pathol 1998;5:347-358.
Kirchgraber PR, Weaver MG, Arafah BM, Abdul-Karim FW: Fine needle aspiration cytology of Langerhans' cell histiocytosis involving the thyroid gland: a case report. Acta Cytol 1994;38:101-106.
Akhtar M, Ali MA, Bakry M, Sackey K, Sabbah R: Fine needle aspiration biopsy of Langerhans' histiocytosis (histiocytosis-X). Diagn Cytopathol 1993;9:527-533.
Yap WM, Chuah KL, Tan P: Langerhans' cell histiocytosis involving the thyroid and parathyroid glands. Mod Pathol 2001;14:111-115.
Writing Group of the Histiocyte Society: Histicytosis syndromes in children. Lancet 1987;1:208-209.
Malone M: The histiocytosis of childhood. Histopathology 1991;19:105-119.
Azumi N, Sheibani K, Swartz WG, et al: Antigenic phenotype of Langerhans cell histiocytosis: an immunohistochemical study demonstrating the value of LN-2, LN-3 and vimentin. Hum Pathol 1988;19:1376-1382.
El-Labban NG: The nature of Langerhans' cell granules: an ultrastructural study. Histopathology 1982;6:317-325.
Pusztaszeri M, Sauder K, Cibas E, Farquin W: Fine needle aspiration of primary Langerhans cell histiocytosis of the thyroid gland, a potential mimic of papillary thyroid carcinoma. Acta Cytol 2013;57:406-412.
Vergez S, Rouquette I, Ancey M, Serrano E, Caron P: Langerhans cell histiocytosis of the thyroid is a rare entity, but an association with a papillary thyroid carcinoma is often described. Endocr Pathol 2010;21:274-276.
Hilly O, Koren R, Raz R, et al: The role of S100- positive dendritic cells in the prognosis of papillary thyroid carcinoma. Am J Clin Pathol 2013;139:87-92.
Behrens RJ, Levi AW, Westra WH, Dutta D, Cooper DS: Langerhans cell histiocytosis of the thyroid: a report of two cases and review of the literature. Thyroid 2001;11:697-705.
Dey P, Luthra UK, Sheikh ZA: Fine needle aspiration cytology of Langerhans cell histiocytosis of the thyroid. Acta Cytol 1999;43:429-431.
Broadbent V, Gadner H: Current therapy for Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1998;12:327-338.
Selch MT, Parker RG: Radiation therapy in the management of Langerhans cell histiocytosis. Med Pediatr Oncol 1990;18:97-102.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.