Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytosis X is a disease of histiocyte proliferation in response to some unknown etiology. Thyroid involvement is extremely rare in the literature. In this paper, we present an 18-year-old female with histiocytosis X with thyroid involvement. This patient had had a goiter with normal thyroid function since 12 years of age. A thyroidectomy was done under the suspicion of thyroid cancer. Pathology revealed histiocytosis X. Hypoparathyroidism and hypothyroidism were noted after the operation and were treated with thyroid hormone, vitamin D and calcium carbonate. This patient also had lesions on the left side of mandible, in the suprasellar region and possibly in the right mastoid. A curettage biopsy of her mandibular lesion was also compatible with histiocytosis X. She was proven to have hypothalamic and pituitary dysfunction including hypogonadism and hypoadrenalism. Her thyroid lesion did not recur after the thyroidectomy. The toothache that she had also experienced subsided after the curettage biopsy of the mandibular lesion. Hypothalamic and pituitary dysfunction were controlled by hormone replacement. Because her disease had been running a benign clinical course, no chemotherapy was given.
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PMID:Histiocytosis X with thyroid involvement: report of a case. 136 23

A case of histiocytosis X involving the thyroid and hypothalamus is reported. A 16 year old female presented with amenorrhoea and diabetes insipidus. She subsequently developed a painful goitre with biochemical hypothyroidism, and stridor. The stridor and goitre responded to cyclophosphamide. Previous publications on the use of cytotoxics in histiocytosis X involving the thyroid are reviewed. We describe for the first time both the ultrasound appearances of the thyroid in this condition and the use of serial volumetric measurements to monitor therapy.
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PMID:Histiocytosis X involving the thyroid and hypothalamus. 192 59

A 27 year old woman with histiocytosis X had an unusual initial presentation with features of primary hypothyroidism and a goitre. Diagnosis was made by lung and thyroid biopsies. Endocrine tests showed the presence of hypopituitarism and a discrete suprasellar mass, consistent with hypothalamic histiocytosis X, was demonstrated by computerised tomography. Radiotherapy and chemotherapy arrested the clinical progression of the disease.
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PMID:Primary hypothyroidism and essential hypernatremia in a patient with histiocytosis X. 387 33

A thyroid involvement is reported in a 10 year-old boy with histiocytosis X. It was revealed by goiter and hypothyroidism following diabetes insipidus and growth hormone deficiency. Hypothyroidism was exclusively due to the invasion of thyroid by the tumor, while the other endocrine changes were in favor of the histiocytic infiltration of hypothalamus. The exceptional feature of thyroid involvement in disseminated histiocytosis X is emphasized.
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PMID:[Thyroid involvement in histiocytosis X]. 698 32

We report a case of Langerhans cell histiocytosis (histiocytosis X) in a 36-year-old female with an euthyroid nodular goitre as the rare initial presentation. She subsequently developed a pathological fracture of the lumbar spine which was initially thought to be due to malignant infiltration, possibly from metastatic thyroid carcinoma. Open vertebral biopsy and total thyroidectomy were performed. Histological features of histiocytic proliferation with erythrophagocytosis were found in both the thyroid gland and in the involved vertebral body. The diagnosis of histiocytosis X was made on the basis of the positive immunoperoxidase staining of the histiocytic cells with S-100 and peanut agglutinin (PNA) as well as the clinical picture. Despite systemic chemotherapy with low dose corticosteroids and vinblastine, diabetes insipidus with a suprasellar mass occurred three years post thyroidectomy. Successful management of this uncommon disease is difficult to conduct and evaluate at the present time.
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PMID:Langerhans cell histiocytosis presenting as a goitre: a case report. 825 67

Involvement of the thyroid gland by Langerhans' cell histiocytosis is quite rare. We describe the case of a 58-year-old man referred for treatment of a progressively enlarging goitre. The trachea was severely stenotic and adjacent structures such as the left carotid vein and the thyroid cartilage were also involved. Central diabetes insipidus and severe combined immunodeficiency were associated. Although fine needle aspiration biopsy of the thyroid was initially interpreted as papillary carcinoma, anaplastic thyroid cancer was suspected. Treatment with prednisolone, doxorubicin and irradiation controlled the tracheal compression. A diagnosis of thyroid Langerhans' cell histiocytosis was finally made on the basis of the presence of Birbeck granules and CD1a and CD4 antigen in the thyroid tumour cells. Furthermore, positive staining for CD68 and lysozyme suggested that the tumour cells may have had the character of phagocytic cells in addition to their dendritic cell nature. This is the first case of thyroid involvement by malignant histiocytosis of Langerhans' cell type with unusual phagocytic markers.
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PMID:Thyroid involvement by malignant histiocytosis of Langerhans' cell type. 894 75

A 28-year-old woman was admitted to our Hospital with a chief complaint of progressive gingival swelling and loosening of teeth over about a year. According to past history, she had received total thyroidectomy 2 years previously due to thyromegaly. The thyroidectomy specimen was at first interpreted as 'poorly differentiated carcinoma of the thyroid'. One year ago, she began to be aware of gingival swelling and loosening of teeth. A gum biopsy was taken and the pathologic features were similar to her 'thyroid carcinoma'. Subsequent investigations, including immunohistochemical stain, showed the gum was heavily infiltrated with histiocyte-like Langerhans' cells which were positive for S-100 protein. Ultrastructural examination of the cells under electron microscope revealed many typical intra-cytoplasmic Birbeck granules. Langerhans' cell histiocytosis was diagnosed. Langerhans' cell histiocytosis with thyroid involvement is extremely rare and may run a relatively indolent course. Even on a retrospective examination, it may easily be confused with poorly differentiated carcinoma of the thyroid. We suspect that this error may have been made on other occasions and that the occurrence of this condition may be underreported.
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PMID:Langerhans' cell histiocytosis with thyroid involvement masquerading as thyroid carcinoma. 925 74

Langerhans cell histiocytosis may be seen with goiter and histiocytic infiltration of the thyroid. We report a 2 1/2-year-old boy who had goiter and primary hypothyroidism develop, later had pulmonary disease, and died of neurologic involvement. Autopsy lesions suggested a transitional dendritic cell precursor of the epidermal Langerhans cell. Of the reported cases of Langerhans cell histiocytosis with goiter in children and adolescents, 82% were male when the relative incidence of Langerhans cell histiocytosis is two males to one female.
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PMID:Atypical dendritic cell-related histiocytosis with goiter and primary hypothyroidism. 950 58

Langerhans cell histiocytosis (LCH) is a rare illness, and the disease afflicting the thyroid gland is very uncommon, even in the presence of multisystem involvement. In this report, we document histologically, for the first time, concurrent involvement of the thyroid and parathyroid glands by LCH. A young Chinese woman with a history of diabetes insipidus and hypogonadism underwent a total thyroidectomy for enlarged thyroid gland secondary to LCH causing airway obstruction. Microscopic examination of the excised specimen disclosed CD1a- and S-100-positive LCH cells involving the thyroid and parathyroid glands. In a patient with LCH affecting the thyroid gland, parathyroid gland disease should be suspected when the serum calcium levels are depressed in association with an inappropriate serum parathyroid hormone level, such as a normal parathyroid hormone level in this case.
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PMID:Langerhans cell histiocytosis involving the thyroid and parathyroid glands. 1123 2

Langerhans cell histiocytosis (LCH) is a monoclonal disease of histiocytes that can involve many or very few organ systems. It is a relatively benign disorder with a 3% mortality in adults. LCH rarely involves the thyroid gland. We report two cases, both presenting in males with a goiter. Both patients were treated with subtotal thyroidectomy. The first patient also received radiotherapy to his thyroid bed and scalp. We summarize the prior reported cases of LCH involving the thyroid and review the current treatment modalities used for LCH.
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PMID:Langerhans cell histiocytosis of the thyroid: a report of two cases and review of the literature. 1148


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