Abstract
Langerhans cell histiocytosis (LCH) is a rare granulomatous disease of unknown etiology. We retrospectively reviewed data from four patients (3 males and 1 female), mean age 33.5 years old (range: 21–40), with histopathological diagnosis of LCH. All of them presented with symptoms suggestive of endocrine involvement. The main complaint was goiter in two patients and polyuria and polydipsia in three. Before the LCH diagnosis, two patients had unevaluated symptoms of diabetes insipidus (DI) and hypogonadism. The mean time from symptoms onset to diagnosis was 6.25 years (range: 2–13). Histopathological diagnosis was established by total thyroidectomy (TT) biopsy in two patients, skin lesion biopsy in one, and pituitary stalk biopsy in the other. In the two-first patients, surgery was indicated after the fine-needle aspiration biopsy (FNAB) showed a false positive result of differentiated thyroid carcinoma and immunohistochemistry was used for diagnosis confirmation. Three cases were treated with chemotherapy; one of them had already received radiation therapy on the hypothalamic-pituitary region, developing post-radiation hypopituitarism.
Similar content being viewed by others
References
Kobayashi TK, Ueda M, Nishino T, Bamba M, Echigo T, Oka H et al (2007) Langerhans cell histiocytosis of the skull on cytologic squash preparations. Diagn Cytopathol 35(3):154–157
Park ET, Suh SI, Seol HY, Lee YH, Seo HS, Shin BK (2008) Sonography of biopsy-provenLangerhans cell histiocytosis in lymph nodes of the neck. J Ultrasound Med 28:519–525
Saliba I, Sidani K (2009) Prognostic indicators for sensorineural hearing loss in temporal bone histiocytosis. Int J Pediatr Otorhinolaryngol 73:1616–1620
Ouyang DL, Roberts BK, Gibbs IC, Katznelson L (2006) Isolated Lahgerhans cells histiocytosis in an adult with central diabetes insipidus: case report and review of literature. Endocr Pract 12(6):660–663
Berhens RJ, Levi AW, Westra WH, Dutta D, Cooper DS (2001) Langerhans cell histiocytosis of the thyroid: a report of two cases and review of the literature. Thyroid 11(7):697–705
García de Marcos JA, Dean Ferrer A, Alamillos Granados F, Ruiz Masera JJ, Barrios Sánchez G, Romero Ortiz AI, Calderón Bohórquez JM et al (2007) Langerhans cell histiocytosis in the maxillofacial area in adults. Report of three cases. Med Oral Patol Oral Cir Bucal 12:E145–E150
Lo WC, Chen CC, Tsai CC, Cheng PW (2009) Isolated adult Langerhans′ cell histiocytosis in cervical lymph nodes: should it be treated? J Laryngol Otol 123:1055–1057
Malpas JS et al (1998) Langerhans cell histiocytosis in adults. Hematol Oncol Clin North Am 12(2):259–268
Kaltsas GA, Powles TB, Evanson J, Plowman PN, Drinkwater JE, Jenkins PJ et al (2000) Hypothalamo-pituitary abnormalities in adult patients with Langerhans cell histiocytosis: clinical, endocrinological, and radiological features and response to treatment. JCEM 85(4):1370–1376
Satter EK, High WA (2008) Langerhans cell histiocytosis: a review of the current recommendations of the histiocyte society. Pediatr Dermatol 25(3):291–295
Aricò M, Girschikofsky M, Généreau T, Klersy C, McClain K, Grois N et al (2003) Langerhans cell histiocytosis in adults. Report from the international registry of the histiocytosis society. Eur J Cancer 39:2341–2348
Aricò M, Nichols K, Whitlock JA, Arceci R, Haupt R, Mittler U et al (1999) Familial clustering of Langerhans cell histiocytosis. Br J Haematol 107:883–888
Kandpal H, Subramanian S, Hari S (2007) Langerhans cell histiocytosis of pituitary stalk. Neurol India 55(1):91–92
Makras P, Alexandraki KI, Chrousos GP, Grossman AB, Kaltsas GA (2007) Endocrine manifestations in Langerhans cell histiocytosis. Trends Endocrinol Metab 18:252–257
Shinmi K, Nagai Y, Matsushima Y, Tamura A, Ishikawa O (2007) Adult case of Langerhans cell histiocytosis. J Dermatol 34:275–277
Zeppa P, Cozzolino I, Russo M, Cataldo PT, Vetrani A, Palombini L (2007) Pulmonary Langerhans cell histiocytosis (histiocytosis X) on bronchoalveolar lavage. Acta Cytol 51(3):480–482
Ladisch S (1998) Langerhans cell histiocytosis. Curr Opin Hematol 5(1):54–58
Carpinteri R, Patelli I, Casanueva FF, Giustina A (2009) Inflammatory and granulomatous expansive lesions of the pituitary. Best Pract Res Clin Endocrinol Metabol 23:639–650
Mittheisz E, Seidl R, Prayer D, Waldenmair M, Neophitou B, Pötschger U et al (2007) Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis. Pedriatr Blood Cancer 48:50–56
Rupp D, Molitch M (2008) Pituitary stalk lesions. Curr Opin Endocrinol Diabetes Obes 15:339–345
Zhu H, Hu DX (2004) Langerhans cell histiocytosis of the thyroid diagnosed by fine needle aspiration cytology. Acta Cytol 48(2):278–280
Murray S, Rowlinson Busza G, Morris JF, Chu AC (2000) Diagnostic and therapeutic evaluation of an anti-Langerhans cell histiocytosis monoclonal antibody (NA 1/34) in a new xenograft model. J Invest Dermatol 114(1):127–134
Imashuku S, Okazaki NA, Nakayama M, Fujita N, Fukuyama T, Koike K et al (2008) Treatment of neurodegenerative CNS disease in Langerhans cell histiocytosis with a combination of intravenous immunoglobulin and chemotherapy. Pediatr Blood Cancer 50(2):308–311
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
García Gallo, M.S., Martínez, M.P., Abalovich, M.S. et al. Endocrine manifestations of Langerhans cell histiocytosis diagnosed in adults. Pituitary 13, 298–303 (2010). https://doi.org/10.1007/s11102-010-0233-8
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11102-010-0233-8