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

A case of histiocytosis X granuloma localized in the pituitary stalk is reported. Coronal and sagittal magnetic resonance imaging views were useful to determine the precise size and location of the mass lesion. The diagnosis was established immunohistochemically and the patient was treated with low-dose irradiation therapy. After irradiation, the patient improved well without endocrine replacement treatment. The pituitary stalk recovered its normal size with no evidence of recurrence on MRI at 7-year follow-up. We emphasize the importance of MRI before initiating therapy to evaluate the pituitary mass lesion and the effectiveness of low-dose irradiation for isolated histiocytosis X.
 ...
PMID:Isolated histiocytosis X of the pituitary stalk. 1078 58

An 18 year old girl presented with acute visual loss. T2 weighted brain MRI showed areas of hyperintensities in the thalamic nuclei, internal capsule, lentiform nuclei, the subarachnoidal spaces, and a retrobulbar infiltration. Analysis of CSF showed numerous foamy histiocytes without malignant cells, raised protein, and depressed glucose concentration. Biopsy of the right thalamus demonstrated aggregates of histiocytes with immunohistological and ultrastructural characteristics of non-Langerhans cell histiocytosis. The patient improved with chemotherapy and corticosteroids. After 3 months of treatment, CSF analysis showed no more histiocytes. Cytological examination of CSF can be helpful for the management of patients with extensive histiocytic infiltration.
 ...
PMID:Atypical form of non-Langerhans histiocytosis with disseminated brain and leptomeningeal lesions. 1103 28

Eosinophilic granuloma is a well-recognized form of Langerhans cell histiocytosis, most commonly involving the skull bones, usually with an excellent prognosis. Recurrent and difficult to recognize osteolytic lesions of the skull are encountered only rarely. A patient with recurrent eosinophilic granuloma of the skull is reported. In spite of appropriate multimodality treatment, there were several recurrences, most recently with involvement of the mastoid process. Imaging studies revealed extensive involvement of surrounding structures with expansion of the tumor into the middle cranial fossa and slight pressure on the antero-medial portion of the temporal lobe of the brain. Despite extensive involvement, the patient had no complaints. Because of the rarity of such silent and unpredictable lesions, a systematic approach with regular CT and MRI follow-up is suggested.
 ...
PMID:Unusual presentation of mastoid eosinophilic granuloma in a young patient. 1140 Jun 54

We report an unusual case of isolated Langerhans cell histiocytosis of the central nervous system. A 19-year-old man presented with an incomplete ocular palsy. MRI revealed a solitary mass in the pineal gland with marked contrast enhancement. Complete microsurgical excision was followed by local radiotherapy. Histological examination revealed histiocytosis. Unifocal brain involvement by histiocytosis X is rare with few cases in the literature; the most commonly involved areas are the hypothalamus and the pituitary gland.
 ...
PMID:Histiocytosis mimicking a pineal gland tumour. 1154 71

Langerhans' cell histiocytosis (LCH) is a disorder of histiocytic proliferation that primarily affects infants. Imaging findings of a rare case of lung and liver involvement in an adult are presented. High-resolution computed tomography (HRCT) of the lungs showed confluent thin-walled cystic air spaces compatible with advanced LCH. Liver CT and MRI revealed unspecific signs of fatty infiltration. Irregular widening of peripheral bile ducts was displayed in breath-hold MR cholangiography. This pattern is considered characteristic for sclerosing cholangitis and should support the diagnosis of LCH in case of concomitant cystic pulmonary disease, even in adult patients.
 ...
PMID:MR cholangiography in the diagnosis of sclerosing cholangitis in Langerhans' cell histiocytosis. 1173 51

PURPOSE. The study was performed to evaluate the efficacy of fine needle aspiration biopsy (FNAB) in orbital lesions. METHODS. Seventeen patients with orbital masses who had been fully investigated by non-invasive techniques participated in this study. FNAB was performed by standard technique, as an outpatient procedure, with ultrasound guidance in lesions posterior to the equator. A trained cytologist analysed all the smears. RESULTS. Specific results were obtained in 14 of the 17 (82%) patients studied by FNAB. Ten cases were neoplastic (8 malignant and 2 benign), 3 were inflammatory and 1 was a case of histiocytosis X. In 3/17 cases the results were non-specific. These were treated as pseudotumours and responded well to systemic steroids. In 7 cases the clinical and radiological diagnosis was confirmed by FNAB. Non-invasive investigations like USG, CT and MRI, however, failed to provide accurate diagnosis in the other 7 (41%) cases. In these patients, FNAB yielded a pathological diagnosis (histiocytosis X, cryptococcosis, non Hodgkin's lymphoma, adenocarcinoma, pleomorphic adenoma, Schwannoma and cysticercosis), helping us to modify treatment with an excellent response. No significant complications were encountered following the aspiration biopsies. CONCLUSION. FNAB proved to be a reliable method for distinguishing between malignant and non-malignant lesions. It was found to be rapid, accurate, cost-effective, safe and a valuable addition to ultrasound, CT scan and MRI in the diagnosis of orbital lesions. This tool may help in avoiding a traumatic surgical intervention.
...
PMID:Fine needle aspiration biopsy in orbital lesions. 1204 31

We used positron emission tomography (PET) to characterize cerebral metabolism in 7 patients (serial examinations in 2 patients) with central nervous system disease in Langerhans cell histiocytosis (CNS-LCH) who had neuroendocrine abnormalities (n = 7), neuropsychiatric disabilities (n = 3), and CNS degenerative disease (n = 1). The PET scan alterations occurred at localizations with known CNS-LCH disease. The PET scans revealed areas where the metabolism and function were altered in 6 of the 7 patients studied, with either an increased or a decreased metabolism (uptake of glucose). Serial examinations may indicate alterations in the degree of ongoing disease activity, but further studies on functional imaging are desired. The additional information of PET compared with MRI is the ability to detect alterations in CNS metabolic activity in certain patients with CNS-LCH. PET may also provide a tool for longitudinal follow-up of therapeutic measures in selected patients.
 ...
PMID:Brain 18-FDG PET scan in central nervous system langerhans cell histiocytosis. 1221 70

We report the case of a child presented by her parents to the ENT outpatient service for swelling of the right temporal bone. The child had a history of recurrent bilateral inflammation of the middle ear. Tympanometry revealed a reduced compliance. Due to conductive hearing loss it was impossible to measure otoacustic emissions. Otherwise a normal ENT status was found. Imaging (MRI/CT) demonstrated bitemporal soft-tissue changes with extensive osseous destruction, but no typical imaging signs of an inflammatory, dysplastic or expansive process. The tentative diagnosis of Langerhans' cell histiocytosis (LCH) made on the basis of the clinical and imaging findings was confirmed by biopsy. After exclusion of disseminated LCH, chemotherapy was initiated, and the child underwent follow-up imaging after 3 months. CT showed clear signs of bitemporal reossification. The case reported here illustrates the problems encountered in diagnosing LCH which may present with unspecific clinical symptoms despite advanced osseous destruction. ENT specialists should be familiar with this very heterogeneous entity and think of LCH especially in children presenting with therapy-refractory otitis media, otitis externa, or mastoiditis in order to ensure a timely diagnosis and to thus improve the chances of successful therapy. Imaging modalities (CT, MRI) have a role in the early diagnosis and follow-up of this disorder.
 ...
PMID:[Langerhans cell histiocytosis: petrosal remodelling after chemotherapy--case report and review of the literature]. 1267 14

We present a 2-year-old girl with an unusual presentation of Langerhans' cell histiocytosis (LCH). Five months prior to admission to our hospital, she received IV steroids for bronchial obstruction. On admission, clinical signs of SVC obstruction were evident and a mediastinal mass was evident on the chest radiograph and MRI. Biopsy revealed fibrosing mediastinitis. Five months later, osteolysis was present on a skull radiograph. Surgical biopsy of the skull lesion revealed LCH. This case is unique because it demonstrates a rare initial manifestation of LCH that has not been previously reported. Furthermore, the primary, solitary mediastinal manifestation without calcifications was histologically interpreted as fibrosing mediastinitis, and the final diagnosis of LCH was only made after identifying the skull lesion.
 ...
PMID:Fibrosing mediastinitis with superior vena cava obstruction as the initial presentation of Langerhans' cell histiocytosis in a young child. 1271 69

A 37 year old man presented with recurrent secondarily generalized seizures from right partial onset since December 1999, MRI scan of brain (contrast study) revealed multiple enhancing lesions predominantly involving frontal, parietal and temporal regions. Left frontotemporal lesion was biopsied and histopathology confirmed it to be rare case of adult Langerhans cell histiocytosis. He was given short course of oral corticosteroids. Two years postoperative course on antiepileptic therapy alone is uneventful and repeat MRI brain and MR spectroscopy showed significant resolution of lesions.
 ...
PMID:Langerhans cell histiocytosis presenting as adult onset epilepsy. 1272 59


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>