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

A 41-month-old black child with a symtomatic diaphyseal destructive lesion of the femur, and a corresponding area of increased uptake on a technetium99m bone scan, had an upper respiratory tract infection. An open biopsy was performed because of an initial clinical diagnosis of osteomyelitis, histiocytosis X or a round cell sarcoma. The biopsy showed numerous blast cells compatible with acute lymphocytic leukemia. Acute leukemia should be included in the differential diagnosis of symptomatic diaphyseal destructive lesions in children. A peripheral blood smear should be carefully interpreted prior to any other invasive diagnostic tests.
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PMID:Childhood leukemia presenting as a diaphyseal radiolucency. 28 Apr 25

Pathological or spontaneous fractures in childhood are rare and are mostly caused by benign bone diseases; the fractures must be treated on an individual basis, as there are no constant rules. Since the new method of osteosynthesis called "elastic stable intramedullary nailing" (ESIN) or "embrochage centro-medullaire elastique stable" (ECMES) has demonstrated the best results in the treatment of normal fractures in childhood, this method is rapidly being used in the treatment of spontaneous or pathological fractures. We report our experience in the treatment of spontaneous fractures in childhood with "elastic stable intramedullary nailing", in nine patients with ten fractures. The pathological diagnosis was in 5 cases a juvenile bone cyst; in addition, there were cases of histiocytosis X, lymphangiomatosis, hemangiomatosis and osteoporosis (one each). The location was the femur (two cases) and humerus (seven cases). All fractures healed completely without pseudarthrosis; as complications we saw one incidence of osteomyelitis, one of a second fracture after "elastic stable intramedullary nailing" and one coxa vara in a child with histiocytosis X of the proximal femur. In the 5 children with juvenile bone cysts the nails are still in situ; in two cases the nails had to be changed.
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PMID:[Treatment of pathological fractures of long tubular bones in childhood using elastic stable intramedullary nailing]. 876 36

Among the potential sites of involvement by Langerhans cell histiocytosis (LCH), the head and neck region is the most commonly cited. Though principally a pediatric disease, LCH can affect any age group. It can be unifocal (skeletal) or multifocal (skeletal and/or visceral); it appears as though the presence of visceral lesions is more common in the youngest patients, and may be associated in some with a rapidly progressive course resulting in death. Head and neck manifestations may mimic such varied entities as eczema, otitis media, osteomyelitis, and cholesteatoma. Current approaches to therapy are less aggressive than they were in the past, and are particularly intended to monitor for and treat any complicating secondary infections (which may develop in the youngest patients with multifocal disease including visceral involvement). The prognosis is very good for unifocal skeletal system disease, and poor for multifocal disease with involvement of tissues other than bone.
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PMID:Head and neck Langerhans cell histiocytosis. 919 16

The spectrum of histopathologic changes in four cases of chronic recurrent multifocal osteomyelitis encountered in our orthopedic outpatient clinic in the past 3 years was studied in conjunction with clinical and radiologic findings. All presented with pain with or without swelling in the affected region. Radiographically, the appearance of the lesions varied from a mixed picture of bone lysis and sclerosis with expansion to sclerosis alone to bone collapse. Bone scintigraphy demonstrated asymptomatic and separate foci of activity in all cases. Prior to biopsy, the clinical and radiologic differential diagnoses included Ewing's sarcoma, metastatic neuroblastoma, hematolymphoid malignancy, Langerhans cell histiocytosis and chronic infection, notably tuberculosis. The spectrum of histopathologic changes ranged from acute (acute inflammatory infiltration, active bone resorption and necrosis, reactive bone formation) to subacute (predominantly lymphocytic and plasma cell infiltration) to chronic inflammation (fibroblastic organization and bony sclerosis). Histologic changes correlated poorly with clinical features, but relatively well with radiologic findings. Lesional excision was performed in one case, cortical saucerization in another, while the final two cases received supportive treatment. All remained well 18-21 months post-therapy. Chronic recurrent multifocal osteomyelitis is a great clinical and radiologic mimic, which merits recognition by the pathologist. Awareness of the spectrum of histologic features encountered enables a correct diagnosis to be made in the appropriate clinical setting. The patient can thus be reassured of a favorable prognosis.
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PMID:Chronic recurrent multifocal osteomyelitis: a great clinical and radiologic mimic in need of recognition by the pathologist. 1023 Jun 89

A benign bone lesion may have a typical appearance on plain radiographs. This is the case with benign cortical defects and osteochondroma. With most other lesions, cross-sectional imaging is needed to complete the study of the tumor. The nidus of osteoid osteoma is well demonstrated on computed tomography, but magnetic resonance imaging also will show the nidus in most cases. Magnetic resonance imaging is considered the modality of choice for evaluation of other benign musculoskeletal lesions because it is highly sensitive to changes in the signal intensity of bone marrow and adjacent soft tissues. It provides useful information for diagnosis of the lesion as in primary or secondary aneurysmal bone cyst, chondroblastoma, osteoblastoma, fibrous dysplasia, and osteofibrous dysplasia, and it helps differentiate these lesions from osteomyelitis, Langerhans' cell histiocytosis, and stress fracture. Bone scanning is most useful for depicting multiple silent lesions as may be seen in multiple osteochondromatosis, nonossifying fibromas, and polyostotic fibrous dysplasia.
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PMID:Magnetic resonance imaging of benign bone lesions: cysts and tumors. 1240 90

Langerhans cell histiocytosis of bone causes bone destruction and frequent bone reactions that mimic benign and malignant neoplasms as well as osteomyelitis. The bony lesion of Langerhans cell histiocytosis consists of histiocytes with variable numbers of eosinophils, lymphocytes, and neutrophils. We present a rare case of Langerhans cell histiocytosis of bone, including imaging studies and pathology. In addition, we discuss the classification and pathology of Langerhans cell histiocytosis.
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PMID:Langerhans cell histiocytosis of bone--a case report. 1251 71

A 13-year-old Ethiopian female patient who initially presented with localized pain and minimal soft tissue swelling later developed pathological fracture of the left femnur. The patient was initially treated as a case of osteomyelitis until the diagnosis of Langerhans cell histiocytosis (histiocytosis X, eosiniphilic granuloma) was made by bone biopsy. The clinical presentation of the case and brief review of the literature is discussed.
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PMID:Unifocal langerhans cell histiocytosis presenting with pathological fracture. 1764 63

We report a case of primary multifocal osseous lymphoma in a 6-year-old girl presenting with multifocal osteolytic lesions without systemic symptoms or identifiable non-osseous primary tumor. The differential diagnoses for such a presentation include histiocytosis X, chronic recurrent multifocal osteomyelitis, acute lymphoblastic leukemia, metastatic disease, and primary bone lymphoma. Although non-Hodgkin lymphoma is common in the pediatric population, its presentation as a primary bone tumor, especially with multifocal disease, is extremely rare and is frequently misdiagnosed. We hope that awareness of this entity will help radiologists achieve timely diagnosis and intervention.
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PMID:Primary multifocal osseous lymphoma in a child. 1877 59

Bone pain in children is common. The cause may be as benign as growing pains or as life-threatening as a malignancy. When a cause cannot be established by laboratory tests, physical examination or patient history, imaging of the affected body part is often obtained. Distinguishing benign from malignant processes involving the bones of children, based on imaging findings, can be challenging. The most common benign conditions that mimic pediatric bone tumors on imaging are Langerhan's cell histiocytosis and osteomyelitis. In this review, the current literature regarding the pathology and imaging of these conditions is reviewed. Benign conditions are compared with the most common pediatric bone tumors, Ewing sarcoma and osteosarcoma, with an emphasis on clinical and imaging features that may aid in diagnosis.
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PMID:The child with bone pain: malignancies and mimickers. 1996 1

Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is a rare, proliferative disorder in which the accumulation of pathologic Langerhans cells leads to local tissue infiltration and destruction. The incidence of LCH is estimated to be one case per 200,000 children per year. The role of the dentist is important in early and accurate evaluation, staging and diagnosis of LCH, because it may mimic more common diseases, such as juvenile periodontitis and osteomyelitis. There are multiple treatment options, but the response is unpredictable. The aim of this paper is to give a short, introductory overview on current diagnostic and treatment strategies for LCH in the oral and maxillofacial region and to present a case of LCH that mimicked juvenile periodontitis and was resolved following extraction of affected teeth. The history, radiological appearance, differential diagnosis, histopathology and treatment options for the patient are discussed.
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PMID:Resolution of eosinophilic granuloma after minimal intervention. Case report and review of literature. 2044 Oct 47


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