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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Langerhans cell histiocytosis (LCH) is a rare disease that may show as a solitary or multifocal lesion of bone, soft tissue or viscera. Involvement of the temporal bone has been described in 15-61% of patients with LCH, usually in association with multisystemic involvement. We report the case of a 30-year-old man presenting with vertigo and fluctuating hearing loss caused by monosystemic LCH of the left petrous bone. The patient was treated with radiosurgery (covering dose 10 Gy at 85% isodose, maximum dose 11.76 Gy). Two years after treatment, no evidence of recurrent disease was found in the CT scan or MRI. We discuss the treatment of temporal bone LCH, traditionally based on surgery, low-dose radiation therapy and intralesional steroids. To our knowledge, this is the first reported case of LCH of the petrous bone successfully treated with radiosurgery. This approach may be interesting in cases of LCH located on nonaccessible areas of the temporal bone.
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PMID:Petrous bone Langerhans cell histiocytosis treated with radiosurgery. 1722 79

A 44-year-old woman with progressive cerebellar ataxia and spastic paraparesis was referred to our hospital. Brain MRI showed bilateral high signals in superior, middle, and inferior cerebellar peduncles on the T2 weighted images. After 3 years, her symptoms progressively worsened in spite of various therapies including whole brain irradiation and high dose oral prednisone. No evidence of diabetes insipidus was noted. In MRI, brainstem lesions expanded to both hemispheres of the cerebellum without enhancement by contrast medium. We confirmed diagnosis of LCH by skin biopsy of intractable truncal rash which emerged after neurological symptoms.
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PMID:[Adult onset Langerhans cell histiocytosis with progressive cerebellar ataxia and spastic paraparesis]. 1732 85

The authors present a rare case of C-6 vertebral involvement in a 12-year-old boy with histiocytosis X. The patient presented with limitation in movements of neck and upper extremities. Computed tomography (CT) and magnetic resonance (MRI) imaging were used in the preoperative workup. Surgery was performed via an anterior cervical approach along with stabilization using a fibula strut graft and plate fixation. Pathological assessment showed infiltration of Langerhans cells accompanied by a mixture of many eosinophils, giant cells, neutrophils and foamy cells. The patient went on to make a full recovery with complete resolution of his motor weakness. In an extensive review of the literature there are very few cases of cervical histiocytosis X reported. In addition, surgical management of this type of lesion has rarely been discussed.
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PMID:Rare C-6 vertebral involvement in a child with histiocytosis X: case report. 1759

Central nervous system (CNS) involvement in Langerhans' cell histiocytosis (LCH) has been described as a progressive neurological disorder marked by motor and cognitive decline. Detailed analysis of ocular motor abnormalities is lacking. We report on a 60-year-old male with histologically confirmed LCH who developed oscillopsia and gait ataxia over a 1-year period. Eye movements recorded with infrared oculography revealed a high rate of square-wave jerks (SWJ) with frequencies of 41 min(-1) on average and amplitudes between 1 degrees and 7 degrees , as well as marked impairment of smooth tracking of sinusoidally moving targets. Furthermore, static posturography disclosed increased body sway, with an abnormally high sway path. The initial brain MRI was unremarkable. Due to the presumed cerebellar dysfunction we performed a second MRI 1 year later that disclosed deep cerebellar lesions compatible with LCH relapse within the CNS. The abnormal high SWJ rate and the impaired smooth pursuit performance correctly heralded later involvement of the cerebellum anticipating lesion appearance in the MRI.
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PMID:Square-wave jerks and smooth pursuit impairment as subtle early signs of brain involvement in Langerhans' cell histiocytosis. 1807 8

Erdheim-Chester disease (ECD) is a granulomatous and infiltrative disorder of unknown etiology with proliferation of cholesterol-containing histiocytes and peculiar bone involvement. It is very similar to Langerhans cell histiocytosis (LCH) on histology but with a different immunohistochemical profile. This is the first report of intraocular involvement in this disease. MPSG, a 46 y.o. woman, presented with proptosis of the OD. She referred ulcerated lesions on the hard palate, symmetrical and bilateral osteosclerosis of the fibulae and tibiae and a nodule in the right breast (biopsy: xantomatous histiocytic infiltrate CD68+, S-100 and CD1a negative on immunohistochemistry compatible with ECD). MRI studies demonstrated an extraconal tumor in the juxta-bulbar temporal portion of the right orbit close to the lacrimal gland and hyperintense on T1. Vision was 20/20 OU, with numerous drusen in the posterior pole, similar to basal laminar drusen. Two regions of orange subretinal infiltrates that showed progressive staining on the angiogram were seen in the peripapillary region and also close to the fovea in the OD. Choroidal neovascular membranes were seen 2 years later in OU leading to severe visual loss in the OS and to a slight visual field loss in the OD, which retained 20/20 vision. This pioneer report depicts in vivo characteristics of histiocytic granulomas in ECD. Caution should be taken with patients with ECD as potentially blinding intraocular complications may arise.
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PMID:[Intraocular involvement in Erdheim-Chester disease--first report in the literature: case report]. 1815 16

A wide variety of pathologies arise from the petrous apex. Such lesions may present with symptoms caused by mass effect or cranial nerve palsies, or may be detected during an investigation for an unrelated disease. CT and MRI are complementary in providing an appropriate differential diagnosis and in aiding surgical planning. This pictorial review appraises the anatomy and contents of the petrous apex. Benign (e.g. cholesterol granuloma, cholesteatoma), inflammatory (e.g.apical petrositis) and dysplastic (e.g. Pagets disease) lesions of the petrous apex are discussed and illustrated. Whilst it is more frequent for neoplastic lesions to extend from adjacent structures, we demonstrate a variety of aggressive tumours and tumour-like conditions (e.g. metastasis, rhabdomyosarcoma, Langerhan's cell histiocytosis, endolymphatic sac tumour) that directly involve the petrous apex. A range of normal radiological appearances are seen, some of which may be mistaken for significant pathology (e.g. asymmetric marrow space development, simple effusions, cephaloceles). An imaging algorithm to aid the formulation of a differential diagnosis is also presented.
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PMID:Imaging of the petrous apex: a pictorial review. 1820 55

Bone marrow oedema is associated with a wide variety of pathological processes including both benign and malignant bone tumours. This imaging finding in relation to intraosseous tumours can aid in providing a more focused differential diagnosis. In this review, we will discuss the MR imaging of bone marrow oedema surrounding intraosseous neoplasms. The different pulse sequences used in differentiating underlying tumour from surrounding oedema are discussed along with the role of dynamic contrast enhanced MRI. Benign lesions commonly associated with bone marrow oedema include osteoid osteoma, osteoblastoma, chondroblastoma and Langerhan's cell histiocytosis. Metastases and malignant primary bone tumours such as osteosarcoma, Ewing's sarcoma and chondrosarcoma may also be surrounded by bone marrow oedema. The imaging findings of these conditions are reviewed and illustrated. Finally, the importance of bone marrow oedema in assessment of post chemotherapeutic response is addressed.
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PMID:Bone marrow oedema associated with benign and malignant bone tumours. 1835 60

A 60-year-old man with progressive gait ataxia and mild pyramidal signs showed at MRI a pontine lesion with post-contrast enhancement in the left middle cerebellar peduncle. Diagnosis of Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis, was suggested, further supported by a previously diagnosed retroperitoneal fibrosis. X-ray films demonstrated characteristic bilateral and symmetric osteosclerosis of the long bones of the lower limbs, which at radionuclide studies exhibited a marked increase in technetium-99 uptake. A cerebral 18FDG-PET showed a relevant pontine uptake of the tracer. Re-evaluation of a past retroperitoneal biopsy showed an intense CD68+, CD1a-, and S100- infiltrate of histiocytes with foamy cytoplasm, thus confirming the diagnosis. ECD should be regarded as a rare cause of adult-onset sporadic ataxia, especially when pontine lesions and extraneurological manifestations are present.
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PMID:Late-onset sporadic ataxia, pontine lesion, and retroperitoneal fibrosis: a case of Erdheim-Chester disease. 1881 Jun 2

Langerhans cell histiocytosis (LCH) is a multisystemic disease, which may present with neurological involvement. We report the case of a 20-month-old girl with initial liver and skin involvement. Initial symptoms were recurrent episodes of trunk dystonia, lasting approximately 2 months prior to the diagnosis of LCH. No brain MRI abnormality was demonstrated at initial work-up and over 7 years of follow-up, except for a postpituitary involvement noted after 3 years of follow-up. These episodes of dystonia subsided during the first week of specific LCH chemotherapy (vinblastine and steroid), suggesting that they may have resulted from hepatalgia related to the histiocytic infiltration of the liver.
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PMID:[Inaugural trunk dystonia revealing Langerhans cell histiocytosis]. 1942 24

Intrinsic bony lesions of the skull base are diseases which arise within the bones forming the skull base. Mainly they are bone tumours and tumour-like lesions. With the exception of osteomas of the paranasal sinuses and exostoses of the external auditory canal, these lesions occur rarely. This article gives an overview of the appearance of the most common primary bony skull base masses in CT and MRI. From the authors' point of view these are fibrous dysplasia, chordomas, chondrosarcomas, Langerhans cell histiocytosis and multiple myelomas, which must be differentiated from pseudolesions. The possibilities of CT and MRI in making a specific diagnosis, differential diagnosis and the kind of making the final diagnosis are described.
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PMID:[CT and MRI of intrinsic space-occupying lesions of the bony skull base]. 1943 84


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