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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-associated neurodegenerative syndrome is an enigmatic manifestation, most often localized in the cerebellum and the basal ganglia. Its pathophysiologic basis is poorly understood, and effective treatment strategies are currently missing. Modern imaging modalities offer the possibility of shedding further light on this puzzling disease in a noninvasive way. We report on a 12-year-old boy with a Langerhans' cell histiocytosis-associated neurodegenerative syndrome who underwent a thorough evaluation with different modern imaging methods in addition to routine brain magnetic resonance imaging (MRI) to analyze their informative value for this condition. Additional imaging included positron emission tomography using [18F]fluorodeoxyglucose (FDG-PET), single photon emission computed tomography using [123I]2beta-carbomethoxy-3beta-(4-iodophenyl)tropane and [123I]iodobenzamide, and magnetic resonance spectroscopy. The potential relevance of each method for neurodegenerative Langerhans' cell histiocytosis is discussed based on the results obtained, and a review of the literature is made. The case underlines the fact that MRI undoubtedly possesses the major role in the diagnostic evaluation and monitoring of Langerhans' cell histiocytosis-associated neurodegenerative syndrome. FDG-PET and magnetic resonance spectroscopy findings were in good correlation with the MRI results. In particular, magnetic resonance spectroscopy could provide a valuable diagnostic tool in addition to MRI in the early detection and evaluation of the neurodegenerative component of this disease.
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PMID:Modern imaging methods for the assessment of Langerhans' cell histiocytosis-associated neurodegenerative syndrome: case report. 1583 24

Our objective was to assess the utility of PET-CT in five pediatric patients with Langerhans cell histiocytosis (LCH) who underwent PET-CT imaging for clinical staging and determination of lesion activity at various stages of treatment and follow-up. PET-CT combines the anatomic detail of CT and the physiologic activity of (18)F-FDG imaging. We conclude that PET-CT information is clinically useful to evaluate disease activity and response to therapy and provides information that cannot be obtained from technetium 99m methylene diphosphonate bone scans or radiographs.
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PMID:PET-CT in pediatric Langerhans cell histiocytosis. 1756 38

Langerhans cell tumors are neoplastic proliferation of Langerhans cells and are classified into Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS). We report a case of LCH in an 89-year-old-woman with left axillary lymphadenopathy. A histologic examination demonstrated a proliferation of histiocytoid cells which were positive for CD1a, S-100 protein, and Lagerin (CD207). Initial diagnosis was LCS based on morphologic features, high MIB-1 index, and multi-system involvement detected by FDG-PET. However, the disease disappeared spontaneously without specific treatment in six months. The disease was considered to be spontaneously regressed LCH with multi-system involvement rather than LCS.
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PMID:Spontaneously regressed Langerhans cell histiocytosis of lymph nodes in an elderly patient. 1793 35

Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis characterized by progressive histiocytic proliferation with multiorgan involvement, typically of the kidney, skin, brain, and lung, and less frequently, the heart and retro-orbital tissue. Fluorine-18 fluorodeoxyglucose positron emission tomography (F-18 FDG PET) plays an important role in the management of this disease. It has been reported that FDG PET imaging allows accurate evaluation of the extent of the disease at baseline, as well as assessment of response to any specific therapy. In this case, a 57-year-old Chinese man presented with functional decline and a urinary tract infection. He had a prior history of xanthogranulomas of bilateral canthal masses. On imaging, he was found to have left hydronephrosis, diffuse urothelial thickening, increased density of the perinephric fat, mural thickening of the descending aorta and soft tissue masses along the posterior wall of the right atrium extending into the region of the interatrial septum and involving the right atrioventricular groove. Histopathology revealed retroperitoneal fibrosis. An IV contrast-enhanced FDG PET scan showed increased activity in a previously unidentified brain stem mass and the shafts of bilateral femora. Varying levels of FDG uptake were seen in the other lesions.
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PMID:Intensely hypermetabolic extra-axial brainstem tumor in Erdheim-Chester disease. 1969 24

Erdheim-Chester disease (ECD), first described by Jakob Erdheim and William Chester in 1930, is a rare form of non-Langerhan's cell histiocytosis with unknown aetiology, is charaterized by systemic xanthogranulomatous infiltrative disease. To date, about 350 cases of ECD have been described in the medical literature. The typical ECD diagnostic triad is bone pain, diabetes insipidus and bilateral exophthalmos. A 24 years old man came at our attention for polydipsia with nocturnal and diurnal polyuria, anorexia, febrile episodes (38(o)C), and arthromyalgia especially in the knees. Physical examination showed bilateral periorbital xanthelasma. Blood exams showed increase of plasma osmolarity, haematocrit, sodium and urea and decrease of potassium. Urine exams showed just decreased urine specific gravity, (1.001;normal range: 1.010-1.030) suggestive for central diabetes insipidus (CDI). Brain magnetic resonance with gadolinium enhancement showed the presence of multiple hyperintense lesions expecially in neurohypophysis (swollen and with markedly contrast enhancement). All these data raised the suspision of neurosarcoidosis, so a chest and abdomen contrast enhancement computed tomography was performed, which didn't show abnormalities, making less possible the diagnosis of sarcoidosis. Two weeks later, whole-body (from head to pelvis) plus lower limbs 18-fluorine-labelled 2-deoxy-2-fluoro-D-glucose positron emission tomography/computed tomography ((18)F-FDG PET/CT) was performed. Uptake of (18)F-FDG was observed in the upper portion of the midbrain area (SUV(max) 7.1) and the pituitary gland (SUV(max) 7.3), and diffuse bone marrow uptake of (18)F-FDG in the proximal epiphysis and metaphysis of both humeri and thigh bones (SUV(max) 6.5), shoulder blades, pelvis bones and the L2 vertebral body (SUV(max) 3.9). This (18)F-FDG PET/CT confirmed the presence of brain lesion seen in MRI , the absence of visceral lesions, but also showed the presence of an atypical bone uptake of (18)F-FDG, leading to the suspision of ECD. A technetium-99m-methyl-diphosphonate skeletal scintigraphy ((99m)Tc-MDP) scan showed diffuse uptake of the radiopharmaceutical, in the diaphysis of long bones and in the left portion of the body and the spinous process of L2. Considering the difficulties of an osteomedullary or brain biopsy, biopsy was performed on a right anterior thoracic cutaneous xanthelasma. Histology showed lipid-laden histiocytes (CD1a-, CD68+, S-100 protein -) with small nuclei, Touton giant, lymphocytic infiltrates, eosinophils and fibrosis, ECD gold standard patterns as reported in literature. The patient was discharged with the diagnosis of ECD with central nervous system (CNS) manifestations, and treatment started. The diagnosis can be lead by the most charateristic bone findings of symmetrical osteosclerosis of the long bones, especially the lower limbs (tibia and fibula), involving metaphyses and diaphyses but sparing epiphyses. The typical pattern of osteoscerosis of the long bones reflects increased osteoblastic activity. About half of all ECD patients may experience extraskeletal manifestations, including CNS. Visceral involvement in ECD is not specific, and this enforces the diagnostic value of skeletal imaging findings. Furthermore xanthomas can be found at any location on the skin, especially the eyelids as in our patient. For visceral involvement, CT is most useful, while MRI is more sensitive for CNS lesions. Involvement of CNS may be frequently revealed clinically by diabetes insipidus. Few case reports have shown that (18)F-FDG PET/CT scanning could be useful in assessing the extension of ECD lesions. Both radiography and (99m)Tc-MDP skeletal scintigraphy may reveal osteosclerosis of the long bones, which is a typical finding in ECD. The typical bone pattern of (18)F-FDG PET/CT scan is specific for ECD and (99m)Tc-MDP skeletal scintigraphy may be performed in patients in whom initial (18)F-FDG PET/CT scans present the possibility of ECD diagnosis. Others reported that (18)F-FDG PET/CT scans had good sensitivity (66.7%) and specificity (92.3%) as compared with MRI of the CNS involvement or lesions. In conclusion, the (18)F-FDG PET/CT scan and the (99m)Tc-MDP scan depicted many of the most relevant lesions of ECD for the initial assessment of ECD in our patient.
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PMID:(18)F-FDG positron emission tomography/computed tomography and (99m)Tc-MDP skeletal scintigraphy in a case of Erdheim-Chester disease. 2208 57

A case of Erdheim-Chester disorder, a rare non-Langerhans' cell histiocytosis, was referred for restaging by F-18 FDG PET/CT more than 10 years after initial diagnosis. The patient presented diabetes insipidus, hypergondotropic hypogonadism, and osteosclerotic lesions. Previous bone scintigraphy documented pathognomonic long bones' involvement. Chronic steroid and hormone replacement therapy was administered, and the patient was asymptomatic. F-18 FDG PET/CT was useful for disease restaging at cardiac and soft tissues level.
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PMID:F-18 FDG PET/CT detects muscle involvement in Erdheim-Chester disease. 2222 52

Langerhans cell histiocytosis (LCH) is a disorder of clonal proliferation of Langerhans-type cells. The imaging findings of LCH are not specific. A 27-year-old woman was admitted to our hospital because of liver enzyme elevation without other hepatic signs. Radiological studies were originally interpreted as possible metastatic disease to the liver. Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT) images demonstrated a diffuse pattern of nodules in the liver with hypermetabolic activity. LCH was diagnosed histopathologically with an ultrasound-guided liver biopsy. This case illustrates the importance of considering proliferative/benign conditions of the liver when interpreting PET/CT. Failure to do so could result in patient mismanagement.
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PMID:F-18 FDG PET/CT imaging of solitary liver Langerhans cell histiocytosis: preliminary findings. 2239 42

A 24-year-old woman presented with recurrent bilateral ear infections since childhood and a more recent history of partial hearing loss, discharge, and ear pain. Biopsy of the left external auditory canal revealed Langerhans cell histiocytosis. An F-FDG PET/CT was done to look for additional sites of disease. Increased metabolic activity was seen within both external ear canals.
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PMID:Langerhans cell histiocytosis of the auditory canal detected by 18F-FDG PET/CT. 2288 88

We report the case of a 63-year-old woman with Erdheim-Chester disease (ECD) and histologic features of Langerhans cell histiocytosis, both extremely rare histiocytic proliferations responsible of skeletal and extraskeletal involvement. 18F-Fluoride PET/CT revealed multiple intense focal uptake scattered throughout the skeleton. We also performed an 18F-FDG PET/CT which point out visceral and vascular involvement. This case illustrates the interest of PET/CT in ECD, a rare polymorphus and systemic disease, and in our knowledge, this is the first reported illustration of 18F-fluoride PET/CT findings in this pathology.
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PMID:18F-fluoride PET/CT aspect of an unusual case of Erdheim-Chester disease with histologic features of Langerhans cell histiocytosis. 2360 79

Objective. The default window setting on PET/CT workstations is soft tissue. This study investigates whether bone windowing and hybrid FDG PET/CT can help differentiate between malignant and benign primary bone tumors. Materials and methods. A database review included 98 patients with malignant (n=64) or benign primary bone (n=34) tumors. The reference standard was biopsy for malignancies and biopsy or >1 year imaging follow-up of benign tumors. Three radiologists and/or nuclear medicine physicians blinded to diagnosis and other imaging viewed the lesions on CT with bone windows (CT-BW) without and then with PET (PET/CT-BW), and separate PET-only images for malignancy or benignity. Three weeks later the tumors were viewed on CT with soft tissue windows (CT-STW) without and then with PET (PET/CT-STW). Results. Mean sensitivity and specificity for identifying malignancies included: CT-BW: 96%, 90%; CT-STW: 90%, 90%; PET/CT-BW: 95%, 85%, PET/CT-STW: 95%, 86% and PET-only: 96%, 75%, respectively. CT-BW demonstrated higher specificity than PET-only and PET/CT-BW (p=0.0005 and p=0.0103, respectively) and trended toward higher sensitivity than CT-STW (p=0.0759). Malignant primary bone tumors were more avid than benign lesions overall (p<0.0001) but the avidity of benign aggressive lesions (giant cell tumors and Langerhans Cell Histiocytosis) trended higher than the malignancies (p=0.08). Conclusion. Bone windows provided high specificity for distinguishing between malignant and benign primary bone tumors and are recommended when viewing FDG PET/CT.
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PMID:Bone Windows for Distinguishing Malignant from Benign Primary Bone Tumors on FDG PET/CT. 2398 16


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