Spontaneous Thyroid Hemorrhage Caused by Langerhans Cell Histiocytosis: A Case Report and Literature Review

Abstract

PurposeLangerhans cell histiocytosis (LCH) is a rare clonal disorder of Langerhans antigen-presenting cells. However, thyroid LCH involvement is relatively rare. We present the first case of spontaneous thyroid hemorrhage due to LCH progression and discuss the clinical features, diagnosis, and treatments of thyroid LCH in a literature review.MethodsClinical data were collected. Previously published articles on thyroid LCH involvement were reviewed to assess the clinical features, diagnosis, and treatments for thyroid LCH.ResultsA 54-year-old female presented with a multi-system LCH, affecting the uterus, liver, pituitary gland, and thyroid gland. Clinical stability was achieved after systemic chemotherapy. After 7 years of regular follow up, the patient complained of a sudden painful neck swelling and progressive dyspnea. Computed Tomography revealed bilateral goiter with hematoma, and the patient was diagnosed with spontaneous thyroid bleeding based on her clinical symptoms and radiological findings. The patient was incubated to relieve airway compromise and partial thyroidectomy was performed for definitive treatment. Pathological evaluation further confirmed the diagnosis of thyroid LCH. The patient recovered well after surgery.ConclusionSpontaneous thyroid bleeding due to thyroid LCH progression is extremely rare. Treatments for LCH vary depending on the severity of the disease. We suggest that, for patients with multi-system LCH with thyroid lesion, long-term active surveillance of thyroid hormone concentrations, and thyroid gland volume is required. Physicians should be alert of the potentially life-threatening spontaneous thyroid hemorrhage when aggravated diffuse goiter and hypothyroidism appear. Further investigation is required to establish the guidelines for thyroid LCH treatment.

Analysis of thyroid involvement in children and adult Langerhans cell histiocytosis: An underestimated endocrine manifestation

“…As for thyroid function status, it has been reported that 37.9-40.9% of patients have euthyroidism, 19.7%-31.03% have hypothyroidism, 6.9%-10.6% have subclinical hypothyroidism, and 1.5%-3.45% have hyperthyroidism (11,12). Our results largely parallel these reports.…”

“…Furthermore, neck pain and obstructive symptoms like dyspnea are rare. Zhang reported a rare case of painful neck swelling and progressive dyspnea diagnosed as a spontaneous thyroid hemorrhage (12).…”

Thyroid Langerhans cell histiocytosis concurrent with papillary thyroid carcinoma: A case report and literature review

“…The patient had no smoking habit, but chest CT was still strongly recommended in the future (31). Fine needle aspiration cytology can preliminarily diagnose most thyroid diseases, but the diagnosis of LCH of the thyroid using FNAC is challenging due to the similarity in HE morphology between PTC cells and LCH cells (8,32). In Table 1, only 64.7% of PTC, 41.2% of LCH, and 29.4% of LCH concurrent with PTC were identified among the 22 cases which underwent preoperative FNAC examination.…”

Langerhans cell histiocytosis misdiagnosed as thyroid malignancy: A case report

“…Patten et al [ 8 ] reviewed a total of 65 cases of LCH involving the thyroid reported from 1961 to 2011 and found that 59% of LCH cases involving the thyroid presented as diffuse thyroid lesions, 25.8% as nodular enlargement, and 13.6% as heterogeneous enlargement. Zhang et al [ 9 ] analysed the clinical data of 29 LCH patients with thyroid involvement reported in the literature from January 2010 to April 2020. A total of 62.07% of the patients presented with goitre or diffuse goitre, and 37.93% presented with single or multiple thyroid nodules.…”

“…In terms of thyroid function, Patten et al [ 8 ] reported that 40.9% of patients had normal thyroid function, 19.7% had hypothyroidism, 10.6% had subclinical hypothyroidism, and 1.5% had hyperthyroidism. Zhang et al [ 9 ] and Cai et al [ 10 ] also suggested that thyroid function may show different manifestations in patients with LCH involving the thyroid. Our patient had a thyroid nodule with no clinical discomfort and normal thyroid function, as reported in the literature.…”