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)

Eosinophilic granuloma of bone is a rare skeletal manifestation of one of the spectrum of diseases known as histiocytosis X. These lesions often simulate other pathophysiologic processes (Ewing sarcoma, chronic osteomyelitis, Brodie abscess, and chondroblastoma) and may present significant diagnostic problems. Three additional cases of eosinophilic granuloma in an epiphysis are reviewed in this report. Transphyseal extension was present in all our cases and in five of 10 cases documented in the literature. Diagnosis demands accurate biopsy and histopathologic evaluation. Treatment and prognosis are individualized.
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PMID:Eosinophilic granuloma of bone in the growing epiphysis. 398 15

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

Localized Langerhans cell histiocytosis (LCH) of bone often presents as a diagnostic challenge. Magnetic resonance imaging (MRI) is frequently used to better delineate most solitary bony lesions. The authors present two cases that illustrate and better define the role of MRI in the evaluation of solitary bone lesions of LCH. In a 3-year-old boy with left-sided hip pain, MRI showed a focal lesion involving the proximal left femur with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. A tumor was suspected because of the overall imaging characteristics and increased uptake on three-phase nuclear scintigraphy. In a 6-year-old boy with right thigh pain, MRI showed a fluid-containing lesion in the mid-diaphysis of the right femur, suggestive of chronic osteomyelitis and Brodie abscess. MRI was instrumental in showing the extent of the lesions in both cases; however, the final diagnosis of LCH was achieved only with histopathologic confirmation, illustrating the limited diagnostic power of this imaging tool.
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PMID:Defining the role of magnetic resonance imaging in unifocal bone lesions of langerhans cell histiocytosis. 1609 26

Eosinophilic granuloma (EG) is a benign tumor-like condition which is characterized by a clonal proliferation of Langerhans-type histiocytes and defined as a local form of Langerhans cell histiocytosis (LCH). The radiographic appearances of EG are quite different depending on the phase of the disease and the site of involvement. A status of EG in the bone is divided into acute and chronic phases. Radiologically acute phase of EG is difficult to differentiate from a malignant bone tumor such as Ewing's sarcoma or acute osteomyelitis. Chronic phase of EG may mimic a chronic osteomyelitis or a benign bone tumor. We report 3 children's cases of EG in the pelvis which showed quite different radiological features and clinical courses. A 6-year-old boy (Case 1) had an osteolytic lesion with slightly defined margins in the right acetabulum. A 4-year-old boy (Case 2) had a radiologically similar-looking lesion in the left acetabulum. These lesions resembled radiologically chronic osteomyelitis (Brodie's abscess) or a benign bone tumor and healed spontaneously after biopsy. A 2-year-old boy (Case 3) had an osteolytic lesion with ill-defined margin in the ilium. It was difficult to differentiate from a malignant tumor such as Ewing's sarcoma, or acute osteomyelitis. The lesion became enlarged after needle biopsy. In spite of an additional curettage, the osteolytic lesion remained in the left pelvis in 1 year. Treatment for EG is controversial. Curettage of the affected site and bone grafting is usually accomplished. However, some EG heal spontaneously. It is of great importance to understand the natural course of EG and this knowledge will give us the opportunity to avoid unnecessary treatment. EG with poor osteolytic margins may progress further after biopsy. EG with well-defined margins may heal spontaneously after biopsy only.
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PMID:Eosinophilic granuloma arising from the pelvis in children: A report of three cases. 1850 15

Small round cell lesions of the bone encompass a heterogeneous group of tumors and tumor-like lesions, including Ewing sarcoma, small cell osteosarcoma, mesenchymal chondrosarcoma, neuroblastoma, non-Hodgkin's lymphoma (NHL), "Ewing-like" undifferentiated round cell sarcomas, metastasizing small cell carcinoma, along with plasma cell dyscrasia and Langerhan's cell histiocytosis. At the same time, there are tumor mimics, for example, chronic osteomyelitis, which has overlapping radiologic features with Ewing sarcoma and a primary intraosseous NHL. An exact diagnosis necessitates integration of clinical, radiologic, pathologic, and ancillary test results, including immunohistochemical and molecular results. Currently, there are several immunohistochemical markers and specific molecular signatures, driving most of these tumors, available, for an exact diagnosis. This review focuses on a pragmatic approach towards uncovering specific small round cell lesions of the bone, emphasizing upon integration of traditional morphology with ancillary techniques, including immunohistochemical markers and molecular techniques, the latter, especially in cases of Ewing sarcoma, Ewing-like undifferentiated round cell sarcoma, mesenchymal chondrosarcoma, and neuroblastoma. Subsequent to the diagnostic approach, including an impact on treatment, individual intraosseous round cell lesions have been described in detail. The references include updated articles from PUBMED.
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PMID:Small round cell lesions of the bone: Diagnostic approach, differential diagnoses and impact on treatment. 3097 40

Osteoid osteoma is a benign bone tumor that frequently occurs between the ages of 10 and 25 years old; in about 80% of the patients, it is associated with intense pain. The present article describes the case of an 11-month-old infant with claudication, right lower limb shortening, and painless right leg volume increase. Image studies demonstrated an osteolytic lesion with small ossifications within, involved by cortical thickening of the right tibial diaphysis. The diagnostic hypotheses were osteoid osteoma, chronic osteomyelitis (Brodie abscess), Ewing sarcoma, and Langerhans cell histiocytosis. Microorganism cultures were negative and the histopathological exam demonstrated osteoid osteoma. The present report expands the knowledge on osteoid osteoma as a cause of painless limping and lower limb shortening in infancy. The early differential diagnosis is important, as surgical excision is curative and prevents further complications.
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PMID:Limb Length Discrepancy on an 11-Month-Old Boy with Osteoid Osteoma. 3136 70