A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidus
Langerhans cell histiocytosis (LCH) is a rare malignancy most commonly characterized by histiocytic infiltration of bone. LCH lesions in the skull place the adjacent central nervous system (CNS) at risk for involvement, which can manifest as central diabetes insipidus (CDI) when there is infiltratio...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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Greater Baltimore Medical Center
2019-11-01
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| Series: | Journal of Community Hospital Internal Medicine Perspectives |
| Subjects: | |
| Online Access: | http://dx.doi.org/10.1080/20009666.2019.1698231 |
| _version_ | 1797971586922315776 |
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| author | P. Daniel Nicholas Ian Garrahy |
| author_facet | P. Daniel Nicholas Ian Garrahy |
| author_sort | P. Daniel Nicholas |
| collection | DOAJ |
| description | Langerhans cell histiocytosis (LCH) is a rare malignancy most commonly characterized by histiocytic infiltration of bone. LCH lesions in the skull place the adjacent central nervous system (CNS) at risk for involvement, which can manifest as central diabetes insipidus (CDI) when there is infiltration of the hypothalamic-pituitary axis. We present a case of a 39-year-old female who presented with polyuria and polydipsia for 1 year and left-sided hearing loss, gait instability, and nystagmus for 5 days. She was found on laboratory evaluation to have CDI and underwent left cortical mastoidectomy for a destructive peripherally enhancing mastoid lesion seen on MRI brain. Pathology revealed CD1a and S100+ LCH and the patient was subsequently discharged to begin outpatient chemotherapy with vinblastine and prednisone. The patient’s CDI was diagnostic of CNS involvement, making her LCH multisystem through the infiltration of both the skull and hypothalamic-pituitary structures. As CDI can be seen in up to 25% of single-system LDH, and up to 50% of multisystem cases, radiologic studies to evaluate for osteolytic skull lesions must be considered as part of the evaluation for LCH when CDI has been diagnosed. |
| first_indexed | 2024-04-11T03:34:59Z |
| format | Article |
| id | doaj.art-dbd37fef3066488db13319088e937e1f |
| institution | Directory Open Access Journal |
| issn | 2000-9666 |
| language | English |
| last_indexed | 2024-04-11T03:34:59Z |
| publishDate | 2019-11-01 |
| publisher | Greater Baltimore Medical Center |
| record_format | Article |
| series | Journal of Community Hospital Internal Medicine Perspectives |
| spelling | doaj.art-dbd37fef3066488db13319088e937e1f2023-01-02T05:38:50ZengGreater Baltimore Medical CenterJournal of Community Hospital Internal Medicine Perspectives2000-96662019-11-019651551710.1080/20009666.2019.16982311698231A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidusP. Daniel Nicholas0Ian Garrahy1Reading Hospital and Medical Center, Tower HealthReading Hospital and Medical Center, Tower HealthLangerhans cell histiocytosis (LCH) is a rare malignancy most commonly characterized by histiocytic infiltration of bone. LCH lesions in the skull place the adjacent central nervous system (CNS) at risk for involvement, which can manifest as central diabetes insipidus (CDI) when there is infiltration of the hypothalamic-pituitary axis. We present a case of a 39-year-old female who presented with polyuria and polydipsia for 1 year and left-sided hearing loss, gait instability, and nystagmus for 5 days. She was found on laboratory evaluation to have CDI and underwent left cortical mastoidectomy for a destructive peripherally enhancing mastoid lesion seen on MRI brain. Pathology revealed CD1a and S100+ LCH and the patient was subsequently discharged to begin outpatient chemotherapy with vinblastine and prednisone. The patient’s CDI was diagnostic of CNS involvement, making her LCH multisystem through the infiltration of both the skull and hypothalamic-pituitary structures. As CDI can be seen in up to 25% of single-system LDH, and up to 50% of multisystem cases, radiologic studies to evaluate for osteolytic skull lesions must be considered as part of the evaluation for LCH when CDI has been diagnosed.http://dx.doi.org/10.1080/20009666.2019.1698231langerhans cell histiocytosiscentral diabetes insipidushypothalamuspituitary glandvinblastine |
| spellingShingle | P. Daniel Nicholas Ian Garrahy A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidus Journal of Community Hospital Internal Medicine Perspectives langerhans cell histiocytosis central diabetes insipidus hypothalamus pituitary gland vinblastine |
| title | A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidus |
| title_full | A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidus |
| title_fullStr | A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidus |
| title_full_unstemmed | A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidus |
| title_short | A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidus |
| title_sort | case of multisystem langerhans cell histiocytosis presenting as central diabetes insipidus |
| topic | langerhans cell histiocytosis central diabetes insipidus hypothalamus pituitary gland vinblastine |
| url | http://dx.doi.org/10.1080/20009666.2019.1698231 |
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