Multimodal therapy including surgery, chemotherapy, and radiation can achieve long-term remission in rare cases of multiple-systemic LCH involving endocrine organs.
May warrant LCH consideration in goiter with multisystem endocrine or hepatic involvement; leaves open mechanistic and epidemiologic characterization.
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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Xie et al. (2018) studied this question.
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