{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,28]],"date-time":"2026-03-28T06:11:33Z","timestamp":1774678293134,"version":"3.50.1"},"reference-count":28,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2013,1,1]],"date-time":"2013-01-01T00:00:00Z","timestamp":1356998400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Shanghai Pujiang Program","award":["11PJ1407700"],"award-info":[{"award-number":["11PJ1407700"]}]},{"name":"National 973 Program of China","award":["2011CB504001"],"award-info":[{"award-number":["2011CB504001"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2013,1,1]]},"abstract":"<jats:title>abstract<\/jats:title><jats:sec><jats:title>CME Learning Objectives<\/jats:title><jats:p>Distinguish Erdheim-Chester disease from Langerhans cell histiocytosis. Cite the keys to diagnosis of Hand-Sch\u00fcller-Christian disease in a patient with only central diabetes insipidus. List the signs linking a Hand-Sch\u00fcller-Christian disease patient to coexisting ECD.<\/jats:p><jats:p>Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) share similar clinical features and mechanisms. In very rare circumstances, the two diseases coexist in the same patient. Here we report such a patient, who was first diagnosed with Hand-Sch\u00fcller-Christian disease (HSC), a type of LCH. Several years later, the patient presented with severe exophthalmos and osteosclerosis on radiograph. New biopsy revealed ECD. We also analyze 54 cases of LCH and 6 cases of ECD diagnosed in our hospital, as well as their progression during a follow-up period of 8 years. In five cases of HSC (9.3% of LCH), a triad of central diabetes insipidus, hyperprolactinemia, and pituitary stalk thickening on magnetic resonance imaging (MRI) preceded the typical bone lesions by 4\u20139 years. In addition, LCH was featured as elevated plasma alkaline phosphatase (ALP), which was normal in ECD. Combined with a literature review, several features are summarized to differentiate ECD from HSC. In patients with diabetes insipidus, concomitant hyperprolactinemia and pituitary stalk thickening on MRI indicate a possible HSC. Additionally, if osteosclerosis is observed in a patient with LCH, the coexistence of ECD should be considered.<\/jats:p><\/jats:sec>","DOI":"10.1634\/theoncologist.2012-0234","type":"journal-article","created":{"date-parts":[[2013,1,9]],"date-time":"2013-01-09T14:04:12Z","timestamp":1357740252000},"page":"19-24","source":"Crossref","is-referenced-by-count":18,"title":["Hand-Sch\u00fcller-Christian Disease and Erdheim-Chester Disease: Coexistence and Discrepancy"],"prefix":"10.1093","volume":"18","author":[{"given":"Jun","family":"Yin","sequence":"first","affiliation":[{"name":"a Shanghai Clinical Center for Diabetes, Department of Endocrinology and Metabolism, Shanghai Key Laboratory of Diabetes Mellitus, Shanghai Diabetes Institute, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Feng","family":"Zhang","sequence":"additional","affiliation":[{"name":"a Shanghai Clinical Center for Diabetes, Department of Endocrinology and Metabolism, Shanghai Key Laboratory of Diabetes Mellitus, Shanghai Diabetes Institute, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huizhen","family":"Zhang","sequence":"additional","affiliation":[{"name":"b Department of Pathology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Li","family":"Shen","sequence":"additional","affiliation":[{"name":"c Department of Clinical Nutrition, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qing","family":"Li","sequence":"additional","affiliation":[{"name":"a Shanghai Clinical Center for Diabetes, Department of Endocrinology and Metabolism, Shanghai Key Laboratory of Diabetes Mellitus, Shanghai Diabetes Institute, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shundong","family":"Hu","sequence":"additional","affiliation":[{"name":"d Department of Radiology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qinghua","family":"Tian","sequence":"additional","affiliation":[{"name":"d Department of Radiology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuqian","family":"Bao","sequence":"additional","affiliation":[{"name":"a Shanghai Clinical Center for Diabetes, Department of Endocrinology and Metabolism, Shanghai Key Laboratory of Diabetes Mellitus, Shanghai Diabetes Institute, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Weiping","family":"Jia","sequence":"additional","affiliation":[{"name":"a Shanghai Clinical Center for Diabetes, Department of Endocrinology and Metabolism, Shanghai Key Laboratory of Diabetes Mellitus, Shanghai Diabetes Institute, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2013,1,8]]},"reference":[{"key":"2021122308342616600_B1","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1002\/mpo.2950170527","article-title":"Histiocytosis syndromes in children: II. Approach to the clinical and laboratory evaluation of children with langerhans cell histiocytosis","volume":"17","author":"Broadbent","year":"1989","journal-title":"Med Pediatr Oncol"},{"key":"2021122308342616600_B2","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1097\/BOR.0b013e32834db53e","article-title":"Langerhans cell histiocytosis and Erdheim-Chester disease","volume":"24","author":"Wilejto","year":"2012","journal-title":"Curr Opin Rheumatol"},{"key":"2021122308342616600_B3","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1136\/jcp.2009.067819","article-title":"Differential immunophenotypic analysis of dendritic cell tumours","volume":"63","author":"Orii","year":"2010","journal-title":"J Clin Pathol"},{"key":"2021122308342616600_B4","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1111\/j.1365-2249.2010.04162.x","article-title":"Human skin and oral mucosal dendritic cells as \u2018good guys\u2019 and \u2018bad guys\u2019 in allergic immune responses","volume":"161","author":"Novak","year":"2010","journal-title":"Clin Exp Immunol"},{"key":"2021122308342616600_B5","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1097\/PAS.0b013e31820654e4","article-title":"Gastrointestinal tract Langerhans cell histiocytosis: A clinicopathologic study of 12 patients","volume":"35","author":"Singhi","year":"2011","journal-title":"Am J Surg Pathol"},{"key":"2021122308342616600_B6","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1016\/S0022-3476(48)80129-0","article-title":"Eosinophilic granuloma of bone and hand-schuller-christian disease","volume":"32","author":"Love","year":"1948","journal-title":"J Pediatr"},{"key":"2021122308342616600_B7","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1007\/s12519-010-0205-0","article-title":"Langerhans cell histiocytosis of bone in children: A clinicopathologic study of 108 cases","volume":"6","author":"Wang","year":"2010","journal-title":"World J Pediatr"},{"key":"2021122308342616600_B8","doi-asserted-by":"crossref","first-page":"405","DOI":"10.2169\/internalmedicine1962.29.405","article-title":"Hand-Schuller-Christian disease with occult diabetes insipidus, cardiac failure and renal dysfunction","volume":"29","author":"Kimura","year":"1990","journal-title":"Jpn J Med"},{"key":"2021122308342616600_B9","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1097\/BOR.0b013e32834d861d","article-title":"Erdheim-Chester Disease","volume":"24","author":"Haroche","year":"2012","journal-title":"Curr Opin Rheumatol"},{"key":"2021122308342616600_B10","first-page":"685","article-title":"Langerhans' cell histiocytosis: Experience from a single center","volume":"45","author":"Bansal","year":"2008","journal-title":"Indian Pediatr"},{"key":"2021122308342616600_B11","first-page":"76","article-title":"Histiocytosis X (eosinophilic granuloma of bone, letterer-siwe disease, and schueller-christian disease)","volume":"46","author":"Lichtenstein","year":"1964","journal-title":"Further observations of pathological and clinical importance J Bone Joint Surg Am"},{"key":"2021122308342616600_B12","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1007\/s00405-003-0690-z","article-title":"Multisystem Langerhans' cell histiocytosis (Hand-Schuller-Christian disease) in an adult: A case report and review of the literature","volume":"261","author":"Scolozzi","year":"2004","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"2021122308342616600_B13","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1097\/MED.0b013e3283050844","article-title":"Pituitary stalk lesions","volume":"15","author":"Rupp","year":"2008","journal-title":"Curr Opin Endocrinol Diabetes Obes"},{"key":"2021122308342616600_B14","doi-asserted-by":"crossref","first-page":"594","DOI":"10.1002\/pbc.20102","article-title":"Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis","volume":"43","author":"Prosch","year":"2004","journal-title":"Pediatr Blood Cancer"},{"key":"2021122308342616600_B15","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1097\/DCC.0b013e31821b7dff","article-title":"Erdheim-Chester disease: A case study and literature review","volume":"30","author":"Andrysek","year":"2011","journal-title":"Dimens Crit Care Nurs"},{"key":"2021122308342616600_B16","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1097\/00005792-199605000-00005","article-title":"Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases","volume":"75","author":"Veyssier-Belot","year":"1996","journal-title":"Medicine (Baltimore)"},{"key":"2021122308342616600_B17","doi-asserted-by":"crossref","first-page":"2778","DOI":"10.1182\/blood-2010-06-294108","article-title":"CNS involvement and treatment with interferon-alpha are independent prognostic factors in Erdheim-Chester disease: A multicenter survival analysis of 53 patients","volume":"117","author":"Arnaud","year":"2011","journal-title":"Blood"},{"key":"2021122308342616600_B18","doi-asserted-by":"crossref","first-page":"2992","DOI":"10.1182\/blood-2005-06-2238","article-title":"Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha","volume":"106","author":"Braiteh","year":"2005","journal-title":"Blood"},{"key":"2021122308342616600_B19","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1016\/j.crad.2010.12.016","article-title":"Imaging Paget's disease of bone\u2014from head to toe","volume":"66","author":"Cortis","year":"2011","journal-title":"Clin Radiol"},{"key":"2021122308342616600_B20","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1002\/mpo.1306","article-title":"Cholestasis, sclerosing cholangitis, and liver transplantation in langerhans cell histiocytosis","volume":"38","author":"Braier","year":"2002","journal-title":"Med Pediatr Oncol"},{"key":"2021122308342616600_B21","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1148\/radiology.151.1.6608118","article-title":"Erdheim-Chester disease: A distinct lipoidosis or part of the spectrum of histiocytosis?","volume":"151","author":"Brower","year":"1984","journal-title":"Radiology"},{"key":"2021122308342616600_B22","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1097\/WNO.0b013e31820a204e","article-title":"Presence of Erdheim-Chester disease and Langerhans cell histiocytosis in the same patient: A report of 2 cases","volume":"31","author":"Pineles","year":"2011","journal-title":"J Neuroophthalmol"},{"key":"2021122308342616600_B23","doi-asserted-by":"crossref","first-page":"2681","DOI":"10.4049\/jimmunol.149.8.2681","article-title":"Interactions of tumor necrosis factor with granulocyte-macrophage colony-stimulating factor and other cytokines in the regulation of dendritic cell growth in vitro from early bipotent cd34+ progenitors in human bone marrow","volume":"149","author":"Reid","year":"1992","journal-title":"J Immunol"},{"key":"2021122308342616600_B24","doi-asserted-by":"crossref","first-page":"e470","DOI":"10.1200\/JCO.2010.34.0307","article-title":"Scintigraphic diagnosis of Erdheim-Chester disease","volume":"29","author":"Balink","year":"2011","journal-title":"J Clin Oncol"},{"key":"2021122308342616600_B25","doi-asserted-by":"crossref","first-page":"1919","DOI":"10.1182\/blood-2010-04-279083","article-title":"Recurrent BRAF mutations in Langerhans cell histiocytosis","volume":"116","author":"Badalian-Very","year":"2010","journal-title":"Blood"},{"key":"2021122308342616600_B26","doi-asserted-by":"crossref","first-page":"2700","DOI":"10.1182\/blood-2012-05-430140","article-title":"High prevalence of BRAF v600e mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses","volume":"120","author":"Haroche","year":"2012","journal-title":"Blood"},{"key":"2021122308342616600_B27","doi-asserted-by":"crossref","first-page":"e28","DOI":"10.1182\/blood-2012-06-429597","article-title":"BRAFv600e mutant protein is expressed in cells of variable maturation in Langerhans cell histiocytosis","volume":"120","author":"Sahm","year":"2012","journal-title":"Blood"},{"key":"2021122308342616600_B28","doi-asserted-by":"crossref","first-page":"596","DOI":"10.1038\/nature09454","article-title":"Clinical efficacy of a Raf inhibitor needs broad target blockade in BRAF-mutant melanoma","volume":"467","author":"Bollag","year":"2010","journal-title":"Nature"}],"container-title":["The Oncologist"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pericles-gcp.literatumonline.com\/doi\/pdf\/10.1634\/theoncologist.2012-0234","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/pericles-gcp.literatumonline.com\/doi\/full-xml\/10.1634\/theoncologist.2012-0234","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/academic.oup.com\/oncolo\/article-pdf\/18\/1\/19\/41856633\/oncolo_18_1_19.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/oncolo\/article-pdf\/18\/1\/19\/41856633\/oncolo_18_1_19.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,4]],"date-time":"2024-05-04T13:48:07Z","timestamp":1714830487000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/oncolo\/article\/18\/1\/19\/6410175"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,1,1]]},"references-count":28,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2013,1,8]]},"published-print":{"date-parts":[[2013,1,1]]}},"URL":"https:\/\/doi.org\/10.1634\/theoncologist.2012-0234","relation":{},"ISSN":["1083-7159","1549-490X"],"issn-type":[{"value":"1083-7159","type":"print"},{"value":"1549-490X","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2013,1,1]]},"published":{"date-parts":[[2013,1,1]]}}}