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)

Infantile myofibromatosis is a mesenchymal disorder of early childhood characterized by the formation of tumors in the skin, muscle, viscera, bone, and subcutaneous tissue. Although relatively rare overall, it represents the most common fibrous tumor of infancy. The etiology of this disorder is unknown. Infantile myofibromatosis can present as a solitary or multicentric form. With the multicentric form, bone is often involved, but solitary bone lesions account for only 10% of the cases. Imaging findings are not pathognomonic, and the differential diagnosis usually includes eosinophilic granuloma (Langerhans cell histiocytosis), osteomyelitis, metastasis, osteoblastoma, epidermoid cyst, hemangioma, fibrous dysplasia, fibrosarcoma, and meningioma. A histological pattern is typical, but there are no histopathological differences between the solitary and multicentric forms. Solitary lesions generally have a favorable prognosis if totally removed, with a 10% recurrence rate; incompletely resected lesions recur. We report the case of a 9-year-old boy who came to our attention with a solitary infantile myofibroma of the calvarium, appearing as a tight-elastic, lightly tender mass in the left frontal area, eroding both the inner and the outer tables. Histopathologically, the specimens showed a spindle-cell tumor with dense reticulin fiber network and expression of smooth muscle actin. Fifty-eight months MR follow-up after total removal showed no residual or relapse.
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PMID:Solitary infantile myofibromatosis of the cranial vault: case report. 2127 63

Langerhans cell histiocytosis (LCH) encompasses a range of clinical presentations. Pure osseous involvement is referred to as eosinophilic granuloma (EG), whereas systemic involvement can either be classified as Hand-Schuller-Christian disease or Letterer-Siwe syndrome. It is estimated that of the total incidence of LCH (0.5 per 100,000 children per year in the United States), nearly 70% are categorized as EG (1). We describe a case of clinically occult calvarial eosinophilic granuloma brought to medical attention only after a traumatic event led to scalp and epidural hemorrhage at the site of the lesion. Osseous EG initially presents as a painful or tender mass, at times even mimicking osteomyelitis. Computed tomography (CT) reveals a lytic lesion with beveled edges. Magnetic resonance (MR) imaging is often non-specific, but usually demonstrates a marrow-replacing process that is T1 hypointense, T2 hyperintense, with homogenous enhancement. The complex MR imaging findings in our case provided a unique perspective regarding the presentation of EG. Once pathology established eosinophilic granuloma, skeletal survey confirmed the lesion was solitary, despite a compelling history of present illness.
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PMID:Eosinophilic granuloma presenting as post-traumatic scalp hematoma with epidural hemorrhage. A case report. 2405 74

Vertebral collapse is a significant event in the paediatric patient with a real potential for associated deformity and morbidity. While in adults the causes tend towards the malignant, particularly metastatic and metabolic disease, the paediatric population demonstrates a different range of diagnoses. This article reviews the typical imaging findings of the more common underlying acquired pathological causes of vertebral collapse in children, including Langerhans cell histiocytosis, chronic recurrent multifocal osteomyelitis, osteogenesis imperfecta. Other causes include pyogenic osteomyelitis and tuberculosis and neoplastic lesions, either primary, metastatic or of haematological origin.
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PMID:Paediatric acquired pathological vertebral collapse. 2440 44

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory disease in children. Pathological vertebral fracture may be the first symptom revealing this disease. We describe the case of a 14-year-old boy, with no significant past medical history, who had a sudden dorsal pain after carrying a friend on his back. Plain radiographs and MRI showed fractures of the superior endplate of T5 and T6 associated with a mild degree of kyphosis. MRI allowed ruling out discitis. The diagnostic hypotheses raised were cancer (lymphoma, leukemia), Langerhans cell histiocytosis, osteogenesis imperfecta, and CRMO. A whole-body MRI (wbMRI) was performed and disclosed several clinically silent signal abnormalities in key sites of CRMO (pelvic bone and tibial metaphyses). We point out that CRMO should be systematically added to the list of possible diseases in case of vertebral fracture. In this perspective, wbMRI is a major noninvasive tool to assess the diagnosis of CRMO, and allows avoiding a bone biopsy in most cases.
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PMID:[Value of whole-body MRI in vertebral fractures]. 2565 82

Nonbacterial osteitis is a rare autoinflammatory disease. Often it is mistaken for a tumor or osteomyelitis. We present a case of a twelve-year-old girl referred to our hospital because of a lesion of the right clavicle. The differential diagnoses were sarcoma, osteitis, and Langerhans cell histiocytosis. After biopsy the diagnosis nonbacterial osteitis (NBO) was established. Treatment of choice is a nonsteroidal anti-inflammatory drug. This case report gives a complete follow-up of the disease, showing the pitfalls of the diagnosis.
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PMID:Nonbacterial osteitis of the clavicle: longitudinal imaging series from initial diagnosis to clinical improvement. 2569 64

This article presents the use of bone cytology for diagnosis of bone tumors. It discusses critical factors and considerations of fine-needle aspiration and bone cytology and presents diagnostic options and differential diagnosis for benign and malignant bone lesions. Osteomyelitis, chrondroblastoma, Langerhans cell histiocytosis, chondromyxoid fibromas, enchondromas, giant cell tumor of bone, osteosarcoma, chondrosarcoma and variants, Ewing sarcoma, chordoma, plasmacytoma, multiple myeloma, lymphoma, and metastatic bone disease are presented.
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PMID:Bone Cytology: A Realistic Approach for Clinical Use. 2683 16

Multiple lytic bone lesions in a child can be a manifestation of various diseases like Langerhans cell histiocytosis, metastatic neuroblastoma, leukemia, hyperparathyroidism, multifocal osteomyelitis and histoplasmosis. Disseminated histoplasmosis caused by Histoplasma capsulatum var. duboisii is well known to present with multiple osteolytic lesions in immunocompromised adults and is mostly restricted to the African subcontinent. Histoplasmosis seen in American and Asian countries is caused by Histoplasma capsulatum var. capsulatum, which presents with pulmonary and systemic manifestations and rarely bone involvement. We report a case of histoplasmosis, caused by H. capsulatum var. capsulatum with extensive lytic bone lesions in a 13 year old immunocompetent boy who presented with prolonged fever, weight loss and multiple boggy swellings. He responded to amphotericin and is currently on Itraconazole. This case is unique for extensive osteolytic lesions with H. capsulatum var. capsulatum infection in an immunocompetent child.
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PMID:Histoplasma capsulatum Infection with Extensive Lytic Bone Lesions Mimicking LCH. 2732 88

Background. The incidence of paediatric nontraumatic clavicle lesions is unknown and there is limited literature regarding the management of such patients. Methods. A review of a prospectively complied radiological database held at the study was conducted for a defined 10-year period. The study centre is the only paediatric service available for a defined catchment population. The case notes of all patients with nontraumatic lesions were reviewed, and the mode of presentation, the diagnostic dilemmas, and the management were recorded. Results. A total of 2133 clavicle radiographs were performed during the study period, with only five having a nontraumatic history. The overall incidence of paediatric nontraumatic clavicle lesions was 0.38 per 100,000 per year. Three patients were diagnosed with chronic recurrent osteomyelitis, one with chronic bifocal osteomyelitis, and one with Langerhans cell histiocytosis. All patients with osteomyelitis demonstrated a typical natural history of a chronic relapsing remitting infection. Three underwent bone biopsy; however, no organism was identified. Conclusion. This study demonstrated that the incidence of nontraumatic clavicle lesions is small, and those patients presenting with osteomyelitis should not routinely undergo a bone biopsy and close observation with the appropriate antibiotic therapy is advised.
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PMID:Nontraumatic Lesions of the Clavicle in a Paediatric Population: Incidence and Management. 2735 73

An important mandate when imaging pediatric patients is the reduction of radiation exposure to the lowest possible level consistent with good quality diagnostic imaging, so individual selection of radiopharmaceutical dosage and imaging technique is essential. Although the logistics of image acquisition and the approach to interpretation mirror the more common use in adults, the challenges of imaging young children typically require greater attention to patient preparation, positioning, and supervision during imaging, with the use of parental or family engagement in the procedure, gentle restraint, and occasionally the need for sedation. Because the normal bone scan appearance varies dramatically with age as osseous structures mature, it is critical that the interpreting physician be well versed in the range of normal variability at each developmental stage. Although routinely employed in the evaluation of malignancy, radionuclide bone imaging in children is used for a much wider range of benign disorders than it is typical in adults. In many pediatric nuclear medicine departments, the evaluation of benign bone disorders represents most imaging procedures. Common indications include the evaluation of sports injuries, osteomyelitis, trauma, including suspected child abuse, growth disorders, such as mandibular condyle asymmetry, and noninfectious processes such as Langerhans cell histiocytosis. Both planar scintigraphy and SPECT imaging are widely employed, although the SPECT acquisition time presents a barrier in some age groups, making shorter planar spot views desirable when feasible. PET bone imaging with 18F-sodium fluoride has been shown useful in a number of pediatric disorders, but may present issues in young children, as it can require the use of sedation. Despite this challenge, the higher resolution and resulting better anatomical depiction of bone anatomy by PET techniques can be very valuable in evaluating small structures, and current experience suggests a large future role for fluoride PET imaging.
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PMID:Nuclear Medicine Techniques in Pediatric Bone Imaging. 2841 51

Positron emission tomography with the radiotracer 18F-fluoro-2-deoxy-d-glucose (FDG) plays an important role in the evaluation of bone pathology. However, FDG is not a cancer-specific agent, and knowledge of the differential diagnosis of benign FDG-avid bone alterations that may resemble malignancy is important for correct patient management, including the avoidance of unnecessary additional invasive tests such as bone biopsy. This review summarizes and illustrates the spectrum of benign bone conditions that may be FDG-avid and mimic malignancy, including osteomyelitis, bone lesions due to benign systemic diseases (Brown tumor, Erdheim-Chester disease, Gaucher disease, gout and other types of arthritis, Langerhans cell histiocytosis, and sarcoidosis), benign primary bone lesions (bone cysts, chondroblastoma, chondromyxoid fibroma, desmoplastic fibroma, enchondroma, giant cell tumor and granuloma, hemangioma, nonossifying fibroma, and osteoid osteoma and osteoblastoma), and a group of miscellaneous benign bone conditions (post bone marrow biopsy or harvest status, bone marrow hyperplasia, fibrous dysplasia, fractures, osteonecrosis, Paget disease of bone, particle disease, and Schmorl nodes). Several ancillary clinical and imaging findings may be helpful in discriminating benign from malignant FDG-avid bone lesions. However, this distinction is sometimes difficult or even impossible, and tissue acquisition will be required to establish the final diagnosis.
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PMID:Benign Bone Conditions That May Be FDG-avid and Mimic Malignancy. 2858 74


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