{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:25Z","timestamp":1784557045390,"version":"3.55.0"},"reference-count":10,"publisher":"Bioscientifica","license":[{"start":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T00:00:00Z","timestamp":1654041600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,6,1]]},"abstract":"\n Summary<\/jats:title>\n Langerhans cell histiocytosis (LCH) is a rare disease characterized by the proliferation of abnormal Langerhans cells in various tissues and organs, including bone, skin, the lungs, and the pituitary gland. Hypothalamic\u2013pituitary lesions in LCH often cause central diabetes insipidus (CDI), but the natural course of LCH in the CNS remains to be elucidated. In this study, we report an interesting case of altered LCH lesions in the CNS from the pituitary to the hypothalamus in a 45-year-old woman. She developed symptoms of polyuria and was diagnosed with CDI with lymphocytic hypophysitis due to an enlarged pituitary gland with stalk thickening shown on MRI. Short-term glucocorticoid therapy cured pituitary enlargement, but serum prolactin levels gradually increased. Six years later, the immunohistological findings of a skin biopsy revealed positive for leukocyte common antigen, S-100, and CD1a expression, indicating a diagnosis of LCH. MRI revealed a new lesion in the hypothalamus without pituitary involvement, likely due to LCH. Chemotherapy improved LCH lesions both in the skin and hypothalamus, but therapy was stopped on the patient\u2019s request. Although adult-onset LCH is rare, it should be considered as a differential diagnosis in cases of CDI as the primary disease. The clinical course in the present case indicated that LCH lesion was altered from pituitary to suprasellar extension; where such changes were observed, the possibility of LCH should be considered.<\/jats:p>\n <\/jats:sec>\n \n Learning points<\/jats:title>\n \n \n Diagnosing the primary disease of CDI is challenging; therefore, careful observation is necessary in pathologically unknown cases.<\/jats:p>\n <\/jats:list-item>\n \n Enhanced MRI should be performed in cases with suspected hypothalamic lesions, such as elevated serum prolactin.<\/jats:p>\n <\/jats:list-item>\n \n Although adult-onset LCH is rare, it should be considered a differential diagnosis in cases of CDI as the primary disease.<\/jats:p>\n <\/jats:list-item>\n \n The direction of changing CNS lesion from pituitary to suprasellar extension might be a unique MRI finding in LCH.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:sec>","DOI":"10.1530\/edm-22-0232","type":"journal-article","created":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T06:20:04Z","timestamp":1654064404000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":5,"title":["Adult-onset Langerhans cell histiocytosis changing CNS lesion from pituitary to suprasellar extension"],"prefix":"10.1530","volume":"2022","author":[{"given":"Yuji","family":"Kadowaki","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School, Kochi University, Nankoku City, Kochi, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4417-7476","authenticated-orcid":true,"given":"Mitsuru","family":"Nishiyama","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School, Kochi University, Nankoku City, Kochi, Japan"},{"name":"Health Care Center, Kochi University, Kochi City, Kochi, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Makoto","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Department of Hematology, Kochi Medical School, Kochi University, Nankoku City, Kochi, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Morisaka","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Kochi Medical School, Kochi University, Nankoku City, Kochi, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shimpei","family":"Fujimoto","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School, Kochi University, Nankoku City, Kochi, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshio","family":"Terada","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School, Kochi University, Nankoku City, Kochi, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kensuke","family":"Kojima","sequence":"additional","affiliation":[{"name":"Department of Hematology, Kochi Medical School, Kochi University, Nankoku City, Kochi, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"European Journal of Endocrinology","doi-asserted-by":"publisher","first-page":"R151","DOI":"10.1530\/EJE-17-0009","article-title":"MECHANISMS IN ENDOCRINOLOGY: Hypophysitis: diagnosis and treatment","volume":"179","author":"Joshi","year":"2018","unstructured":"Joshi MNWhitelaw BCCarroll PV. MECHANISMS IN ENDOCRINOLOGY: Hypophysitis: diagnosis and treatment. European Journal of Endocrinology2018179R151\u2013R163. (https:\/\/doi.org\/10.1530\/EJE-17-0009)10.1530\/EJE-17-0009"},{"key":"bib2","series-title":"Blood","doi-asserted-by":"publisher","first-page":"1319","DOI":"10.1182\/blood.2019000934","article-title":"Langerhans cell histiocytosis","volume":"135","author":"Rodriguez-Galindo","year":"2020","unstructured":"Rodriguez-Galindo CAllen CE. Langerhans cell histiocytosis. Blood20201351319\u20131331. (https:\/\/doi.org\/10.1182\/blood.2019000934)10.1182\/blood.201900093428944988"},{"key":"bib3","series-title":"Orphanet Journal of Rare Diseases","article-title":"Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net","volume":"8","author":"Girschikofsky","year":"2013","unstructured":"Girschikofsky MArico MCastillo DChu ADoberauer CFichter JHaroche JKaltsas GAMakras PMarzano AV, Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net. Orphanet Journal of Rare Diseases20138 72. (https:\/\/doi.org\/10.1186\/1750-1172-8-72)30633157"},{"key":"bib4","series-title":"Journal of Clinical Endocrinology and Metabolism","doi-asserted-by":"publisher","first-page":"1370","DOI":"10.1210\/jcem.85.4.6501","article-title":"Hypothalamo-pituitary abnormalities in adult patients with Langerhans cell histiocytosis: clinical, endocrinological, and radiological features and response to treatment","volume":"85","author":"Kaltsas","year":"2000","unstructured":"Kaltsas GAPowles TBEvanson JPlowman PNDrinkwater JEJenkins PJMonson JPBesser GMGrossman AB. Hypothalamo-pituitary abnormalities in adult patients with Langerhans cell histiocytosis: clinical, endocrinological, and radiological features and response to treatment. Journal of Clinical Endocrinology and Metabolism2000851370\u20131376. (https:\/\/doi.org\/10.1210\/jcem.85.4.6501)10.1210\/jcem.85.4.6501"},{"key":"bib5","series-title":"Pediatric Blood and Cancer","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1002\/pbc.20003","article-title":"Course and clinical impact of magnetic resonance imaging findings in diabetes insipidus associated with Langerhans cell histiocytosis","volume":"43","author":"Grois","year":"2004","unstructured":"Grois NPrayer DProsch HMinkov MP\u00f6tschger UGadner H. Course and clinical impact of magnetic resonance imaging findings in diabetes insipidus associated with Langerhans cell histiocytosis. Pediatric Blood and Cancer20044359\u201365. (https:\/\/doi.org\/10.1002\/pbc.20003)10.1002\/pbc.2000328944988"},{"key":"bib6","series-title":"Pediatric Blood and Cancer","article-title":"Evaluation and treatment of Langerhans cell histiocytosis patients with central nervous system abnormalities: current views and new vistas","volume":"65","author":"Yeh","year":"2018","unstructured":"Yeh EAGreenberg JAbla OLongoni GDiamond EHermiston MTran BRodriguez-Galindo CAllen CEMcClain KL, Evaluation and treatment of Langerhans cell histiocytosis patients with central nervous system abnormalities: current views and new vistas. Pediatric Blood and Cancer201865 e26784. (https:\/\/doi.org\/10.1002\/pbc.26784)30633157"},{"key":"bib7","series-title":"Endocrine Journal","doi-asserted-by":"publisher","first-page":"441","DOI":"10.1507\/endocrj.EJ21-0341","article-title":"Clinical features and difficulty in diagnosis of Langerhans cell histiocytosis in the hypothalamic-pituitary lesion","volume":"69","author":"Oda","year":"2022","unstructured":"Oda YAmano KSeki YKimura SYamashita KMasui KKomori TIchihara AKawamata T. Clinical features and difficulty in diagnosis of Langerhans cell histiocytosis in the hypothalamic-pituitary lesion. Endocrine Journal202269441\u2013449. (https:\/\/doi.org\/10.1507\/endocrj.EJ21-0341)10.1507\/endocrj.EJ21-0341"},{"key":"bib8","series-title":"Annals of Hematology","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1007\/s00277-021-04694-7","article-title":"Langerhans cell histiocytosis in adults: a retrospective, single-center case series","volume":"101","author":"Chiaravalli","year":"2022","unstructured":"Chiaravalli SFerrari ABergamaschi LPuma NGattuso GSironi GNigro OLivellara VSchiavello EBiassoni V, Langerhans cell histiocytosis in adults: a retrospective, single-center case series. Annals of Hematology2022101265\u2013272. (https:\/\/doi.org\/10.1007\/s00277-021-04694-7)10.1007\/s00277-021-04694-7"},{"key":"bib9","series-title":"Medicine","article-title":"Isolated hypothalamic-pituitary Langerhans\u2019 cell histiocytosis in female adult: a case report","volume":"98","author":"Tan","year":"2019","unstructured":"Tan HYu KYu YAn ZLi J. Isolated hypothalamic-pituitary Langerhans\u2019 cell histiocytosis in female adult: a case report. Medicine201998 e13853. (https:\/\/doi.org\/10.1097\/MD.0000000000013853)"},{"key":"bib10","series-title":"NMC Case Report Journal","doi-asserted-by":"publisher","first-page":"101","DOI":"10.2176\/nmccrj.cr.2020-0094","article-title":"Adult Langerhans cell histiocytosis diagnosed by biopsy of the skull tumor generated after craniotomy","volume":"8","author":"Kono","year":"2021","unstructured":"Kono MInomoto CHoriguchi TSugiyama INakamura NSaito R. Adult Langerhans cell histiocytosis diagnosed by biopsy of the skull tumor generated after craniotomy. NMC Case Report Journal20218101\u2013105. (https:\/\/doi.org\/10.2176\/nmccrj.cr.2020-0094)10.2176\/nmccrj.cr.2020-0094"}],"container-title":["Endocrinology, Diabetes & Metabolism Case Reports"],"original-title":[],"link":[{"URL":"https:\/\/edm.bioscientifica.com\/view\/journals\/edm\/2022\/1\/EDM22-0232.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/edm.bioscientifica.com\/downloadpdf\/journals\/edm\/2022\/1\/EDM22-0232.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/edm.bioscientifica.com\/downloadpdf\/journals\/edm\/2022\/1\/EDM22-0232.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:47:54Z","timestamp":1784555274000},"score":1,"resource":{"primary":{"URL":"http:\/\/journals.bioscientifica.com\/edm\/article\/3224"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6,1]]},"references-count":10,"URL":"https:\/\/doi.org\/10.1530\/edm-22-0232","relation":{},"ISSN":["2052-0573"],"issn-type":[{"value":"2052-0573","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6,1]]}}}