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Successful management of multiple-systemic Langerhans cell histiocytosis involving endocrine organs in an adult
Author(s) -
Junhui Xie,
Zhen Li,
Yi Tang
Publication year - 2018
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000011215
Subject(s) - medicine , langerhans cell histiocytosis , endocrine system , histiocytosis , pathology , hormone , disease
Rationale: Langerhans cell histiocytosis (LCH) involving non-endocrine organs has been frequently reported, whereas LCH involving endocrine organs is rare and the mechanism is unclear. Patient concerns: We report a case of multiple-systemic Langerhans cell histiocytosis (LCH) that first manifested with thyroid goiter, followed by pituitary and liver involvement. Diagnoses: The diagnosis was confirmed based on immunohistochemistry of the thyroid and liver. Interventions: The patient was treated with thyroidectomy combined with chemotherapy and radiation therapy for thyroid and liver, respectively. Outcomes: Surprisingly, the patient presented with clinical remission and no new lesion of LCH was found during follow-up over 10 years. Lessons: LCH involving the endocrine system is unusual and easily misdiagnosed or delayed, especially when the thyroid and pituitary glands are involved. Pathological examination is necessary for a definitive diagnosis. Regular examinations, such as anterior and posterior pituitary hormones, should be especially evaluated annually in the patients with LCH involving endocrine system.

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