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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 still of unknown origin. Its clinical patterns are various and it is above all a bone disease of children, mostly boys. It is a tumoral condition basically benign but with a strong tendency toward dissemination and destruction. Its natural history is unpredictable. The authors have reviewed 37 cases after a 44 months mean follow-up for bringing up a better understanding of the role of orthopedic surgery. The diagnosis was always based on biopsies looking for at least two basic signs out of four possible ones. Scintigraphy was used to detect other locations. MRI was used to evaluate the effect of treatment. In single eosinophilic granuloma, curettage was associated with biopsy. Local injections of corticoids may help in spinal locations. In disseminated forms, surgical treatment should be little aggressive, limited to a biopsy of the most superficial lesion. These forms should be treated by radiotherapy corticotherapy and chemotherapy, as well as the aggressive lesions, the extensive ones, the threatening ones and those which are not accessible.
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PMID:[Localizations of histiocytosis X in bone]. 141 Jul 17

Five cases of central diabetes insipidus demonstrate the potential of MRI to explore the hypothalamohypophysial axis. The diversity of the detected pathologies are illustrated: abnormal signal of the posterior pituitary in an idiopathic diabetes insipidus, lobar prosencephaly, malignant teratoma (embryonal carcinoma), histiocytosis X, craniopharyngioma.
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PMID:[Contribution of MRI in 5 cases of central diabetes insipidus]. 217 Sep 28

Multifocal eosinophilic granuloma is part of the spectrum of histiocytosis X, in which the unifying feature is the proliferation and infiltration of histiocytes. Central nervous system (CNS) involvement has a predilection for the hypothalamic nuclei, and these infiltrates appear as high signal foci on T2-weighted magnetic resonance (MR) images that completely resolve after effective chemotherapy. Intradiploic skull lesions are well delineated with MRI, and the relation to underlying brain may be confidently assessed. MRI is the procedure of choice for staging multifocal eosinophilic granuloma in the skull and CNS, as well as for monitoring response to therapy.
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PMID:MRI in multifocal eosinophilic granuloma: staging disease and monitoring response to therapy. 275 Oct 73

Langerhans cell histiocytosis is a systemic disorder consisting of abnormal histiocyte proliferation, in the form of focal deposits. Central nervous system involvement is most common in the hypothalamus, although other sites have been described, such as the cerebellum and the meninges. We present a case with presumed involvement of the corpus callosum and cerebellum, demonstrating gadolinium enhancement on MRI.
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PMID:Langerhans cell histiocytosis involving the corpus callosum and cerebellum: gadolinium-enhanced MRI. 766 62

We report on 3 patients with intracranial manifestations of Langerhans cell histiocytosis (LH). The results are correlated with histological, clinical and radiological reports on some 70 patients described in the literature as suffering from intracranial LH. Two different morphological pictures can be differentiated. First, typical infiltrates can be seen by microscopy; these can be located in every part of the brain, but are seen mainly in the region of the hypothalamus. These infiltrations are shown as space-occupying lesions with Gd-DTPA enhancement. The other manifestation is a demyelinized lesion with a sparse infiltration of Langerhans cells. These lesions, which are located mainly in the region of the nucleus dentatus of the cerebellum and the brain stem, show increased signal intensity in the T2 sequence and no Gd-DTPA enhancement on MRI.
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PMID:[Intracranial manifestations of Langerhans-cell histiocytosis. Nuclear magnetic resonance findings]. 784 76

We report the radiological findings and more specifically the MRI features in three typical cases of Langerhans' cell histiocytosis of the head and neck. All three cases were of solitary eosinophilic granuloma of bone: two mandibular and one temporal bone lesion. Reports on the MRI features of head and neck eosinophilic granulomas are rare.
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PMID:Eosinophilic granuloma of the head and neck: CT and MRI features in three cases. 800 91

Two cases of histiocytosis X in young adults with involvement of only the petrous bone are reported. In the first symptoms and signs consisted of a seventh nerve palsy of gradual onset over 3 years, and in the second, of pulsatile tinnitus associated with otorrhoea of clear fluid and impaired vestibular function. CT revealed large lytic lesions of the petrous bone. T1-weighted MRI before and after gadolinium confirmed the presence of a mixed tumour of the petrous bone with marked uptake of contrast medium. Spin echo images demonstrated the absence of extradural extension, and the patency of the arterial and venous network, therefore enabling differential diagnosis from extensive glomus jugulare tumours.
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PMID:Histiocytosis X of the petrous bone in the adult: MRI. 806 84

Langerhans cell histiocytosis (LCH) is a disease of children characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system. Intracranial LCH involving the brain is uncommon. We present a case of LCH involving the dural venous sinuses and choroid plexus. Contrast MRI provided an excellent means of identifying the extent of tumor involvement and showed that the patient was at risk for venous sinus thrombosis.
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PMID:MR of intracranial Langerhans cell histiocytosis. 812 86

In up to 25% of cases of children with central diabetes insipidus no organic cause can be documented. We present three boys (age 2.2, 2.3 and 6 years at diagnosis) with acute onset central diabetes insipidus, in whom the only pathological finding using MRI was a thickened central part of the pituitary stalk (> 2.5 mm). Recent reports demonstrate similar MRI findings in adults with Langerhans cell histiocytosis (LCH), sarcoidosis, or tuberculosis, and in children with proven LCH and diabetes insipidus. In those adults with LCH, the pituitary stalk lesion has been histologically verified as a sequela of LCH. In contrast, in two of our three patients pituitary stalk thickening preceded the typical peripheral lesions of LCH by several months, whereas in the third patient there is as yet no evidence of systemic disease. We conclude that thickening of the central part of the pituitary stalk might represent the first manifestations of LCH clinically presenting with diabetes insipidus. MRI investigation of the pituitary stalk in children with unexplained central diabetes insipidus and accurate follow up in patients with thickening of the pituitary stalk in necessary to avoid missing other manifestations of a systemic disease.
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PMID:Pituitary stalk thickening with diabetes insipidus preceding typical manifestations of Langerhans cell histiocytosis in children. 831 4

A 31-year-old overweight man, suffering from high-blood pressure, was hospitalized for transient fits and hemiparesis. MRI disclosed a large irregular mass affecting the vault, meninges and invading the parietal lobe. At neurosurgery, the lesion was necrotic, hemorrhagic and poorly demarcated from the surrounding brain. Histopathology revealed a benign Langerhans cell histiocytosis. No other systemic nor organic lesions could be discovered. After additional local radiotherapy, the patient recovered completely and regained normal activities 6 months later.
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PMID:Focal brain invasion as the first manifestation of Langerhans cell histiocytosis in an adult. Case report. 840 25


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