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)

Two disorders involving histologically benign proliferations of fibrous tissue or of histiocytes occur preferentially in children and often require combined management by an orthopedic surgeon and a pediatric oncologist. Treatment of young people with aggressive fibromatosis usually begins with wide local excision of the lesion. However, some tumors cannot be completely removed either because of their location or because of the risk of subsequent serious dysfunction. Not infrequently, local recurrence supervenes despite previous wide local excision, and sometimes multiple tumors are present. In these situations a trial of multiple-agent chemotherapy incorporating vincristine, actinomycin D, and cyclophosphamide may be indicated in an attempt to control the disease. Radiation therapy may also be useful, but the relatively high dose (5000 cGy or more) needed in a growing child is at times a less attractive alternative. Biopsy of a lytic bone lesion in young patients with Langerhans' cell histiocytosis, formerly known as histiocytosis X, is also indicated for initial diagnosis. Biopsy and curettage are usually curative in the patient with an isolated lesion. Patients with multiple simultaneous or recurrent lesions need chemotherapy if dysfunction of the liver, spleen, or lungs is present. Drug therapy may also be beneficial for children with systemic symptoms. This article outlines suggestions for chemotherapeutic treatment in both diseases.
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PMID:Chemotherapy for children with aggressive fibromatosis and Langerhans' cell histiocytosis. 198 32

Tumors of the pediatric facial skeleton represent a major challenge in clinical practice because they can lead to functional impairment, facial deformation, and long-term disfigurement. Their treatment often requires a multidisciplinary approach, and radiologists play a pivotal role in the diagnosis and management of these lesions. Although rare, pediatric tumors arising in the facial bones comprise a wide spectrum of benign and malignant lesions of osteogenic, fibrogenic, hematopoietic, neurogenic, or epithelial origin. The more common lesions include Langerhans cell histiocytosis and osteoma, while rare lesions include inflammatory myofibroblastic and desmoid tumors; juvenile ossifying fibroma; primary intraosseous lymphoma; Ewing sarcoma; and metastases to the facial bones from neuroblastoma, Ewing sarcoma, or retinoblastoma. This article provides a comprehensive approach for the evaluation of children with non-odontogenic tumors of the facial skeleton. Typical findings are discussed with emphasis on the added value of multimodality multiparametric imaging with computed tomography (CT), magnetic resonance imaging (MRI) with diffusion-weighted imaging (DWI), positron emission tomography CT (PET CT), and PET MRI. Key imaging findings and characteristic histologic features of benign and malignant lesions are reviewed and the respective role of each modality for pretherapeutic assessment and post-treatment follow-up. Pitfalls of image interpretation are addressed and how to avoid them.
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PMID:Non-odontogenic tumors of the facial bones in children and adolescents: role of multiparametric imaging. 2828 10