2011
DOI: 10.1007/s12185-011-0955-z
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Treatment of patients with hypothalamic-pituitary lesions as adult-onset Langerhans cell histiocytosis

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 wa… Show more

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Cited by 24 publications
(10 citation statements)
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“…As mentioned, LCH can involve CNS and the most common manifestation is DI due to the hypothalamic‐pituitary mass lesion. Mass lesions in hypothalamus and/or pituitary glands are often treated with vinblastine/prednisone or 2‐CdA 63,64 . Regardless of the treatment, most reported cases have shown that symptoms of DI or hormone dysfunction could not be reversed and requires constitutive hormone replacement therapy.…”
Section: Treatments For Hypothalamic‐pituitary Mass Lesionmentioning
confidence: 99%
“…As mentioned, LCH can involve CNS and the most common manifestation is DI due to the hypothalamic‐pituitary mass lesion. Mass lesions in hypothalamus and/or pituitary glands are often treated with vinblastine/prednisone or 2‐CdA 63,64 . Regardless of the treatment, most reported cases have shown that symptoms of DI or hormone dysfunction could not be reversed and requires constitutive hormone replacement therapy.…”
Section: Treatments For Hypothalamic‐pituitary Mass Lesionmentioning
confidence: 99%
“…There are reports suggesting the use of chemotherapy as a first-line treatment for these patients [18,19]. This treatment strategy was discussed both on initial presentation and at the time of recurrence in this case.…”
Section: Discussionmentioning
confidence: 92%
“…To date, only six cases of isolated pituitary lesions in adults treated with radiotherapy have been described in the literature [11-19]. The five most recent cases were treated with 20Gy in 10 fractions (three patients) [15,16], 21Gy in 15 fractions (one patient) [11], 21Gy with the addition of chemotherapy (one patient) [19], or radiosurgery (one patient) [17].…”
Section: Discussionmentioning
confidence: 99%
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“…-mutated LCH is described (13). Central nervous system is affected in 6% of patients with LHC, mostly the hypothalamopituitary region and pineal gland, cerebellum and basal ganglia (14). Patients present with central DI and pituitary stalk thickening.…”
Section: Pituitary Metastasismentioning
confidence: 99%