Journal List > Endocrinol Metab > v.26(1) > 1085921

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Hong, Ohn, Kim, Hwang-Bo, Kim, Kwon, Lee, Choi, Lee, Cho, Shin, Park, Jang, Cho, Lee, Shin, Yang, and Kim: Clinical Characteristics of Langerhans Cell Histiocytosis with Hypothalamo-Pituitary Involvement
Original Article
Endocrinology and Metabolism

Endocrinology and Metabolism 2011; 26(1): 38-43.

Published online: 31 March 2011

DOI: https://doi.org/10.3803/EnM.2011.26.1.38

Clinical Characteristics of Langerhans Cell Histiocytosis with Hypothalamo-Pituitary Involvement

Eun Shil Hong, Jung Hun Ohn, Jung Hee Kim, Yul Hwang-Bo, Jin Joo Kim, Jung Hee Kwon, Jung Won Lee, Se Youn Choi, Eun Kyung Lee, Sun Wook Cho, Chan Soo Shin, Kyong Soo Park, Hak Chul Jang, Bo Youn Cho, Hong Kyu Lee, Choong Ho Shin1, Sei Won Yang1, Seong Yeon Kim

Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.

1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.

Corresponding author: Seong Yeon Kim. Division of Endocrinology, Department of Internal Medicine, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 110-744, Korea. Tel: +82-2-2072-3216, Fax: +82-2-762-9662, seongyk@plaza.snu.ac.kr

Received 27 July 2010       Accepted 12 September 2010

Copyright © 2011 Korean Endocrine Society

Abstract

Background

Langerhans cell histiocytosis (LCH) is a rare disease that involves a clonal proliferation of Langerhans cells. LCH has a predilection for hypothalamo-pituitary axis (HPA) dysfunction, and this leads to diabetes insipidus (DI) and/or anterior pituitary dysfunction. Here, we describe the endocrine dysfunction and clinical characteristics of adult patients with LCH and we analyzed the differences between an adult-onset type and a childhood-onset type.

Methods

The data was obtained from a retrospective chart review of the patients with LCH that involved the HPA and who attended Seoul National University Hospital. The patients were classified into the adult-onset type (age at the time of diagnosis ≥ 16) and the childhood-onset type (age at the time of diagnosis ≤ 15).

Results

Ten patients (9 males and 1 female) were diagnosed with LCH involving the HPA. Five patients were classified as an adultonset type and the other five patients were classified as a childhood-onset type. The median follow-up duration was 6 (3-12) years for the adult-onset type and 16 (15-22) years for the childhood-onset type. All the patients presented with DI as the initial manifestation of HPA involvement. Four adult-onset patients and three childhood-onset patients had a multi-system disease. Panhypopituitarism developed in three adult-onset patients and in one childhood-onset patient. The pituitary lesion of the three adult-onset patients had spread to the brain during the follow-up duration. In contrast, the pituitary lesion of the other two adult-onset patients without panhypopituitarism and all the childhood-onset patients had not changed.

Conclusion

DI was the initial presentation symptom of HPA involvement. Anterior pituitary hormone deficiency followed in some patients. Compared with the childhood-onset patients, the adult-onset patients were more likely to have panhypopituitarism and a poor prognosis.

Keywords: Anterior pituitary hormone deficiency, Diabetes insipidus, Hypopituitarism, Hypothalamo-pituitary axis, Langerhans cell histiocytosis

Figures and Tables

Fig. 1
Langerhans cell histiocytosis of the mastoid in a 52-year old man. A. This shows the typical morphology of an inflammatory infiltrate including eosinophils and histiocyte-like mononuclear cells having abundant amphophilic cytoplasm and grooved nuclei (H&E staining, × 400). B. Langerhans cells disclose strong immunoreactivity for S-100 (immunohistochemistry, × 200).
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Fig. 2
Different prognosis of pituitary lesion (sella MRI). A. Pituitary stalk mass is seen at baseline (Patient 3). B. Pituitary stalk mass increased after 2 years (Patient 3). C. Pituitary stalk mass is seen at baseline (Patient 4). D. Pituitary stalk mass had no change after 3 years (Patient 4). E. Pituitary stalk thickening is seen at baseline (Patient 5). F. Pituitary stalk thickening disappeared after 12 years (Patient 5).
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Table 1
Clinical characteristics, disease extent, endocrine involvement and treatment modality of the ten patients
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*Biopsy site. ACTH, adrenocorticotrophic hormone; DI, diabetes insipidus; GH, growth hormone; LH-FSH, luteinizing hormone-follicle stimulating hormone; PRL, prolactin; TSH, thyroid stimulating hormone.

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