Successful management of multiple-systemic Langerhans cell histiocytosis involving endocrine organs in an adult: A case report and review of literature.

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.