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)

In this study fresh frozen tissue samples of benign osseous tumors (five non-osteogenic fibromas, one fibrous dysplasia, one chondromyxoidfibroma), tumors of uncertain biological behaviour (eight cases of histiocytosis X, two giant-cell tumors), and of malignant intraosseous tumors (two malignant fibrous histiocytomas, two malignant histiocytosis, four osteosarcomas, one chondrosarcoma and two Ewing sarcomas) were studied with a panel of monoclonal antibodies reactive with monocyte/macrophages and various types of dendritic cells. In addition, tumors were further defined with a broad spectrum of antibodies against filamentous proteins and lymphocyte differentiation antigens. The specimens were stained with a triple-layer immunoalkaline phosphatase protocol. Tumors stained with these antibodies could be roughly divided into two groups. The first group comprised tumors with one predominant cell population reactive with one particular monoclonal antibody. In this group, cases of histiocytosis X were found to be consistently labelled with CD-1 antibodies. The giant-cell tumors showed a very homogeneous staining with certain monocyte/macrophage antibodies (Ki-M8). Nevertheless, even in these tumors, heterogeneity was demonstrated by the occurrence of cells with monocytic differentiation in histiocytosis X and conversely by the occurrence of cells with differentiation antigens of the dendritic cell system in giant-cell tumors. An exception has to be made for the two cases of malignant histiocytosis examined. These tumors were selectively labelled with antibodies against monocyte/macrophages (Ki-M8, IOM-1). The second group comprised tumors showing a high degree of heterogeneity demonstrated by the varying amounts of tumor cells reacting with the applied markers of the monocyte/macrophage and dendritic cell systems. In most cases it was difficult to ascribe labelled cells to the tumor cell population as opposed to an "innocent bystander" inflammatory cell population. This distinction was especially difficult in malignant fibrous histiocytomas underlining the current concept that these tumors are of primitive mesenchymal rather than true histiocytic origin.
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PMID:Histiocytic differentiation in benign and malignant bone tumors. 246 63

The authors report on 21 cases of "primary" xanthoma of bone. Twenty of the patients were older than 20 years old. The male-female ratio was 2:1. The presenting symptom was pain in 13 patients and neurologic symptoms in 2; in 6 patients, the lesion was an incidental finding. All but one of the lesions in this series were solitary, and the flat bones (pelvis, rib, skull) were the most frequently involved sites. Radiographically, a well-defined, sometimes expansile lytic lesion, with either a small area of surrounding reactive bone or a distinct sclerotic margin, was seen. Microscopically, foam cells, giant cells, cholesterol clefts, and fibrosis were present in varying degrees. In none of these cases was there an identifiable underlying lesion. The differential diagnosis includes Erdheim-Chester disease (a multisystemic granulomatosis) and bone involvement in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). More important is the differential diagnosis with metastatic clear cell carcinoma. Xanthoma of bone is a benign lesion, and complete or even partial removal is effective. Xanthomas may represent a "burnt-out" benign condition such as fibrous dysplasia or histiocytosis X.
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PMID:Xanthoma of bone. 314 Jun 52

Clinical and demographic data as well as the course of illness were analyzed in a retrospective study of 50 patients with a primary tumor or a malformation of the calvarial bones. The most frequent histological diagnosis was "eosinophilic granuloma of bone" (found in 42% of cases), followed by hemangioma, osteoma, dermoid, epidermoid and malignant tumors, and fibrous dysplasia. Local recurrence was observed in 1 patient with ossifying fibroma and 1 with a dermoid tumor, while further dissemination of illness was observed in 3 patients with "eosinophilic granuloma". In these cases, systemic drug therapy is needed. The necessity of an intensive search for further signs of illness and long follow-up periods in patients with primary localized histiocytosis X is stressed.
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PMID:[Tumors and space-occupying abnormalities of the skull calotte]. 325 4

Viruses and other possible causative agents should be sought light and electron microscopically in all cases of ill-defined diseases including "sarcoid." Ideally, tissue should be prepared for electron microscopic examination as soon as a specimen is obtained; however, when this has not been done, tissue preserved in formalin solution can be used. Viruses, some bacteria, and other agents suspected on the basis of light microscopic findings can be verified electron microscopically by reprocessing paraffin-embedded tissue from areas that show smudge cells, focal necrosis with atypical cellular proliferation, and nuclear inclusions. Electron microscopically, all dying cells show swelling and rupture of cellular organelles and membranes; reactive changes include proliferation of branching tubules and paracrystalline and other types of proteinaceous precipitates (inclusions) in both the nucleus and cytoplasm. Qualitative and quantitative changes of cellular organelles, fibrils, microvilli, and intercellular junctions reflect hyperplasia, metaplasia, or dysplasia of the cell and may enable identification of the diseases, e.g., desquamative interstitial pneumonia. In various conditions, basal laminae become irregular, disruptive, or reduplicated following epithelial necrosis and regeneration. Electron microscopic evidence of immunologic damage to basal lamina and cells and immuno-electron-microscopic features of the lung in general require further studies. Electron microscopic features of transbronchial biopsy specimens may be diagnostic in cases of alveolar proteinosis, histiocytosis X, and amyloidosis. Ultrastructural abnormalities of cilia are common; primary ciliary defects are rare. Finally, light microscopic, scanning electron microscopic, and x-ray energy-dispersive spectrometric examinations of paraffin-embedded sections appear most practical for the pathologic evaluation of cases of pneumoconiosis.
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PMID:Applications of electron microscopy to diagnostic pulmonary pathology. 635 60

At the time children with suspected hypopituitarism are seen with a subnormal growth rate, it is expected that testing will demonstrate growth hormone (hGH) deficiency. Seven patients with organic CNS lesions--three with histiocytosis X, one with septo-optic dysplasia, one with neonatal meningitis, one with an anterior encephalocele and meningitis, and one with neurofibromatosis who had normal growth hormone concentrations (greater than 7 ng/mL) despite a subnormal growth rate--were studied. Subsequent retesting 0.5 to 4.6 years later demonstrated the development of growth hormone deficiency. Four of the patients had evidence of other pituitary hormone deficiencies at the time of initial testing whereas two subsequently developed other deficiencies. The initial subnormal growth rate in these children may be secondary to their organic CNS lesion or an evolving hypopituitarism, perhaps with deficient somatomedin generation. These studies point out the need for continued observation and retesting in such patients.
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PMID:Evolving hypopituitarism in children with central nervous system lesions. 640 95

The skull may mirror many systemic diseases or conditions. In fact, when gross and microscopic pathologic findings are confusing, proper analysis of the skull radiograph may lead to a more accurate and complete diagnosis. The article discusses some of the most common systemic diseases with skull or calvarial manifestations (fibrous dysplasia, hyperparathyroidism, histiocytosis X, Paget's disease, and anemias), with special attention to pathophysiology.
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PMID:Some skeletogenic lesions with common calvarial manifestations. 697 86

CT and MR imaging findings of 57 sphenoidal masses were retrospectively reviewed to assess the possibility of differential diagnosis between them. Various kinds of masses such as pituitary adenoma, epipharyngeal cancer, mucocele, chordoma, chondroma, chondrosarcoma, distant metastasis, multiple myeloma, fibrous dysplasia, craniopharyngioma, hemangiopericytoma, giant cell tumor, primary sphenoidal cancer, malignant melanoma, leukemia, histiocytosis X, and giant cell tumor were included in this series. CT scanning was performed in all cases, while MR images were obtained in 48 cases using a spin-echo pulse sequence. The relative density of the masses, bony changes and calcification were evaluated on CT, and on MR images, signal intensity of the masses relative to the normal gray matter, contrast enhancement and extension/contour were evaluated. Although no single feature appeared to be specific to the masses, detection of calcification on CT, identification of the normal pituitary gland as deformed or displaced on T1-weighted images, signal intensity on T2-weighted images, and extension of the masses seemed to be useful and should be examined in terms of their ability to assist in differential diagnosis. Finally, accommodative classification of sphenoidal masses primarily based on presumed origin or mode of extension was attempted.
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PMID:[CT and MR imaging findings of sphenoidal masses]. 807 65

There are several tumors and tumorlike entities of bone that do not easily fit into the specific classifications provided elsewhere in this issue. In this article, several seemingly unrelated entities are discussed, including cystic diseases of bone (simple bone cysts and aneurysmal bone cysts), Langerhans' cell histiocytosis, benign and malignant vascular tumors of bone, and adamantinoma of long bone and its relationship to osteofibrous dysplasia.
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PMID:Miscellaneous lesions of bone. 844 53

Bone scintigraphy of the hands and wrists represents an important adjunct imaging technique that complements plain film radiographic examination. The use of the three-phase bone scan provides clinical information not only regarding osseous uptake but the blood flow and extravascular distribution of the radiotracer as well. Scintigraphic evaluation of the hands and wrists is employed in acute and chronic conditions. In the event of an equivocal or negative plain film, the bone scan can identify occult fractures. Of particular concern is the identification of scaphoid fractures due to the higher incidence of osteonecrosis. Work related injuries represent a significant health issue. The bone scan can be a part of the algorithm for evaluating chronic pain syndromes including reflex sympathetic dystrophy. The complimentary roles of bone scanning and imaging with gallium-67 citrate or radiolabeled leukocytes has proven useful in the evaluation of acute or chronic osteomyelitis. In addition, the diphosphonates are useful in identifying solitary and multiple primary bone tumors. In the case of primary bone tumor, thallium-201 can be used to evaluate response to therapy. Although uncommon in the hand and wrist, the bone scan can identify metastatic tumors or tumor-related conditions such as hypertrophic osteoarthropathy. Finally, bone scintigraphy may be useful in identifying location and extent in a variety of conditions such as fibrous dysplasia, histiocytosis X, and Paget's disease.
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PMID:Upper extremity radionuclide bone imaging: the wrist and hand. 946 90

A variety of normal variants or pathologic conditions of the ribs may be overlooked at chest radiography if the ribs are not evaluated carefully. Rib lesions may simulate pulmonary disease as well. Normal rib variants include cervical, intrathoracic, and pelvic ribs; forked rib; fusion and bridging; and pseudarthrosis of the first rib. Trauma-related lesions are common and usually occur in isolation but can alert the radiologist to other injuries. Metastases may appear as vague areas of increased opacity overlying the lung if seen en face and typically have a smooth interface with the lung on oblique images. Chondroid lesions nearly always arise at or near the anterior end of the rib. Osteochondroma (exostosis) typically manifests as a deformity or expansion of the rib with calcification of the cartilaginous cap. Acute rib infections are seen as focal areas of bone destruction, whereas chronic infections may manifest as periosteal reaction or a bone sequestrum. Inferior rib notching may be seen in a wide variety of pathologic conditions. Rib abnormalities may also be seen in fibrous dysplasia, Langerhans cell histiocytosis, Paget disease, and various hemoglobinopathies. In most cases, radiography is sufficient for the identification and diagnosis of normal variants and pathologic conditions of the ribs.
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PMID:Keep your eyes on the ribs: the spectrum of normal variants and diseases that involve the ribs. 1048 69


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