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KMID : 1035520160040010026
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Brain Tumor Research and Treatment : BTRT
2016 Volume.4 No. 1 p.26 ~ p.29
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A Case of Langerhans Cell Histiocytosis Manifested as a Suprasellar Mass
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Yoon Ju-Young
Park Byung-Kiu
Yoo Heon
Lee Sang-Hyun
Hong Eun-Kyung
Park Weon-Seo
Kwon Young-Joo
Yoon Jong-Hyung
Park Hyeon-Jin
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Abstract
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Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis.
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KEYWORD
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Langerhans cell histiocytosis, Germinoma, Central nervous system neoplasms, Sella turcica, Diabetes insipidus.
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