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Treatment of patients with hypothalamic-pituitary lesions as adult-onset Langerhans cell histiocytosis

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Abstract

We report four cases of adult-onset Langerhans cell histiocytosis (LCH) with central nervous system (CNS) lesions in the hypothalamic-pituitary region. The first clinical symptoms were diabetes insipidus (two patients), hypothyroidism (one patient), and decreased libido/erectile dysfunction (one patient). Diagnosis was delayed as the CNS lesion was not initially suspected to be secondary to LCH, with a median time from symptom onset to treatment of 3.0 (range <1–5.3) years. In three patients, the tumor mass was effectively reduced by chemotherapy; however, all patients continue to exhibit hypopituitarism. Early diagnosis and initiation of treatment are required to improve the outcome of CNS-LCH in adult patients.

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Authors and Affiliations

  1. Division of Pediatrics, Takasago-seibu Hospital, 1-10-41 Nakasuji, Takasago, 676-0812, Japan

    Shinsaku Imashuku, Naoko Kudo & Shigehiro Kaneda

  2. Division of Pediatrics, Kyoto City Hospital, Kyoto, Japan

    Hiroshi Kuroda

  3. Department of Endocrinology, Hiroshima University Faculty of Medicine, Hiroshima, Japan

    Tsuguka Shiwa

  4. Department of Endocrinology, Osaka Medical College, Takatsuki, Japan

    Tetsuya Hiraiwa

  5. Department of Internal Medicine, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan

    Atsushi Inagaki

  6. Department of Pediatrics, Jichi Medical University, Shimotsuke, Japan

    Akira Morimoto

Authors
  1. Shinsaku Imashuku
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  2. Naoko Kudo
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  3. Shigehiro Kaneda
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  4. Hiroshi Kuroda
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  5. Tsuguka Shiwa
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  6. Tetsuya Hiraiwa
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  7. Atsushi Inagaki
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  8. Akira Morimoto
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Correspondence to Shinsaku Imashuku.

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Imashuku, S., Kudo, N., Kaneda, S. et al. Treatment of patients with hypothalamic-pituitary lesions as adult-onset Langerhans cell histiocytosis. Int J Hematol 94, 556–560 (2011). https://doi.org/10.1007/s12185-011-0955-z

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  • DOI: https://doi.org/10.1007/s12185-011-0955-z

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