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)

Typically, soft-tissue involvement with Langerhans' cell histiocytosis (LCH) is the result of extension from adjacent bone marrow. We present a patient who developed soft-tissue masses of LCH, which did not arise as a result of extension from bone marrow, but instead produced extrinsic cortical erosion, preserving the marrow signal on MRI.
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PMID:Extraskeletal soft tissue masses of Langerhans' cell histiocytosis. 873 13

The subject of this paper is a 2-year-old child with progressive paraparesis. MRI showed a large lumbosacral intradural-extramedullary mass and the histological diagnosis was Langerhans cell histiocytosis. The histopathological and neuroradiological findings are discussed.
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PMID:Langerhans cell histiocytosis presenting as a lumbosacral intradural-extramedullary mass. 880 8

Central nervous system involvement is uncommon in Langerhans cell histiocytosis. The suprasellar region is more frequently affected. There have been few reports of involvement of the brain parenchyma shown on CT or MRI. We present a case of involvement of the pons, showing marked contrast enhancement on MRI.
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PMID:Langerhans cell histiocytosis with involvement of the pons: case report. 956 20

Langerhans cell granulomatosis, once called histiocytosis X, is a rare disease. A case of multifocal Langerhans cell granulomatosis in the bone, lymph nodes, skin and lungs of an 18-year-old man is described. Head CT and MRI showed a soft tissue mass of the left temporal bone. Lymph node and skin biopsies substantiated a diagnosis of Langerhans cell granulomatosis. A High resolution CT scan of the lung revealed a small cystic lesion, and bronchoalveolar lavage (BAL) showed an increased number of S-100 positive cells. Steroid therapy resulted in complete resolution, and no S-100 positive cells were obtained in the follow-up BAL study.
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PMID:[A case of multifocal Langerhans cell granulomatosis: a BAL follow up study]. 956 87

We used constructive interference in steady state (CISS) 3D Fourier transform (3DFT) MRI data sets to obtain three-dimensional (3D) virtual MRI endoscopic views of the intracranial cerebrospinal fluid (CSF) spaces, processing them with a commercially available perspective endoscopic algorithm. We investigated the potential of the intracranial virtual MRI endoscopy applied to visualisation of the pathology in 13 patients with surgically confirmed trigeminal neuralgia (3), hemifacial spasm (3), acoustic neuroma (3), suprasellar germinoma (1), Langerhans cell histiocytosis (1), lateral ventricle nodules (1) and pituitary dwarfism (1). All images were acquired using a 1.5-T imager employing a circular polarised head coil. The CISS-3DFT data sets were transferred to a workstation for processing with the perspective endoscopic algorithm. Postprocessing for virtual MRI endoscopy was possible for all data sets. The lesions in 12 patients, and their complex anatomical relationships with the surrounding structures, were well seen on the 3D images. A small acoustic neuroma in the internal auditory meatus was not seen using virtual endoscopy. Although virtual MRI endoscopy has limitations, it provides 3D images which cannot be acquired using any other procedure.
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PMID:Virtual MRI endoscopy of the intracranial cerebrospinal fluid spaces. 983 93

The MRI features of two cases of spinal Langerhans' cell histiocytosis with multilevel involvement are presented in which MRI was of help in differentiating active from inactive healing lesions by the demonstration of signal changes in the vertebral body marrow of the active lesion, manifest as low signal intensity on T1-weighted sequences and high signal intensity on T2-weighted sequences. This distinction could not be made by plain radiography or bone scintigraphy. In cases where biopsy is required for diagnosis, MRI is recommended to guide the biopsy towards levels suggestive of active involvement.
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PMID:Langerhans' cell histiocytosis of the spine: use of MRI in guiding biopsy. 992 28

The incidence of diabetes insipidus secondary to Langerhans' cell histiocytosis (LCH) varies among different reports, ranging from 9.5 to 50%, but it has never been reported in literature in Taiwan. Therefore, we presented a case suffering from polyuria, polydipsia, body weight loss for more than one year and seborreic dermatitis-like skin lesions over the scalp and trunk for more than two years. Her body weight and body length were both less than 3 percentile. Fluid restriction and vasopressin test were performed to differentiate nephrogenic from neurogenic diabetes insipidus. Skin biopsy revealed picture of LCH and LCH with complete central diabetes insipidus was diagnosed. Brain MRI and other laboratory examinations were all within normal limits. She received nasal DDAVP treatment and chemotherapy with TPOG-H 94 protocol. After 3 months treatment, her skin lesions disappeared and daily urine amount returned to normal range.
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PMID:Diabetes insipidus in Langerhans' cell histiocytosis: report of a case. 1037 74

Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination.
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PMID:Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis. 1045 Aug 54

We report an exceptional case of aspecific inflammatory lesion of the thoracic spinal cord simulating an intramedullary glial tumor. Patient history was characterized by progressive spastic paraparesis with urinary incontinence; MR imaging (T4-T5) showed an enhancing intrinsic mass lesion. Myelotomy enabled partial resection of grayish astrocytoma-like tissue. Only light microscope examination was possible and disclosed aspecific inflammatory tissue composed of eosinophils, lymphocytes and histiocytes. After the operation, the patient improved promptly and no further therapy was administered. Control MRI after four months, one year and two years showed complete disappearance of the intramedullary mass lesion and the patient remained clinically stable with no other signs of disease. Since electron microscopic as well as immunohistochemical studies were not available, a definitive histological diagnosis was not possible. However on the basis of some clinical similarities with cases of isolated histiocytosis X of the CNS reported in the literature, we suggest that a diagnosis of isolated intramedullary eosinophilic granuloma could be reasonable.
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PMID:Aspecific inflammatory lesion (histiocytosis?) simulating intramedullary astrocytoma. Case report. 1049 69

The natural course and optimal treatment for isolated hypothalamic Langerhans cell histiocytosis (LCH) are unknown. We describe an adult female in whom total resection of a hypothalamic LCH granuloma was performed 12 years after transphenoidal resection of a pituitary adenoma. A retrospective review of the histological specimen of the first operation revealed CD1a positive cells characteristic of LCH along with a plurihormonal adenoma 12 years earlier. No other manifestations of LCH were found and MRI of the brain at the last follow-up 4 years after surgery did not show any recurrent or additional lesion. The diagnosis of isolated hypothalamic LCH is only possible by biopsy and our case demonstrates the feasability of a gross total resection in certain cases.
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PMID:Resection of a Langerhans cell histiocytosis granuloma of the hypothalamus: case report. 1061 92


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