Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy
Open Access
- 1 January 2014
- journal article
- Published by Korean Endocrine Society in Endocrinology and Metabolism
- Vol. 29 (3), 394-399
- https://doi.org/10.3803/enm.2014.29.3.394
Abstract
We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH) involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT). He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated.Keywords
This publication has 17 references indexed in Scilit:
- Langerhans Cell Histiocytosis in the Thyroid and Draining Lymph Nodes: A Case ReportEndocrinology and Metabolism, 2012
- Multisystem Langerhans Cell Histiocytosis in ChildrenPediatric Drugs, 2011
- Clinical Characteristics of Langerhans Cell Histiocytosis with Hypothalamo-Pituitary InvolvementEndocrinology and Metabolism, 2011
- Endocrine manifestations in Langerhans cell histiocytosisTrends in Endocrinology & Metabolism, 2007
- Adult Langerhans cell histiocytosisEuropean Journal of Haematology, 2006
- Risk factors for diabetes insipidus in langerhans cell histiocytosisPediatric Blood & Cancer, 2005
- A Case of Langerhans Cell Histiocytosis Presented with Central Diabetes InsipidusJournal of Korean Society of Endocrinology, 2005
- Langerhans cell histiocytosis in adults: more questions than answers?European Journal of Cancer, 2004
- Diabetes insipidus in langerhans cell histiocytosi: Results from the DAL‐HX 83 studyMedical and Pediatric Oncology, 1995
- Pulmonary Langerhans Cell Granulomatosis (Histiocytosis X) A Clinicopathologic Study of 48 CasesThe American Journal of Surgical Pathology, 1993