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

Langerhans cell histiocytosis (LCH) is a granulomatous disease which can involve multiples sites of the body. Diagnostic imaging is of utmost importance in the management of these patients. Up to now radiographic skeletal survey and bone scintigraphy (BS) have been used to assess bone involvement (both with low specificity). Magnetic resonance imaging (MRI) and CT have been used to assess visceral involvement but with the limitation that they cannot give information about the functional status. Recently somatostatin receptor scintigraphy (SSRS) has been proposed to detect active lesions and to monitor response to treatment. The aim of this study is to assess bone and somatostatin receptor scintigraphy in the detection of bone involvement in LCH in children. Twenty scintigraphies (12 SSRS and 8 BS) were performed in seven patients (3 girls and 4 boys) aged at diagnosis: 18 month-12 years (mean age 6 years). The findings obtained in the scintigraphies were compared with clinical evolution and other imaging techniques. Bone scintigraphy detected all the LCH bone lesions, and discovered one unknown lesion. SSRS scintigraphy visualised the active lesions in 3 patients (clinical and other imaging techniques were also positive). SSRS was negative in one patient classified as disease free and another in clinical remission. SSRS detected 2 new unknown bone lesions, but could not detect LCH bone lesions confirmed in other imaging techniques in 2 patients. Somatostatin receptor and Bone scintigraphy can be used to detect active LCH bone lesions in children and can help to monitor response to treatment. Further studies with more patients are needed to confirm the diagnostic usefulness of these techniques.
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PMID:[Bone scintigraphy and somatostatin receptor scintigraphy in pediatric patients with bone involvement in Langerhans cell histiocytosis]. 1458 29

We make a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We report a case of Erdheim-Chester disease and review 60 cases from the literature. These cases are consider to have Erdheim-Chester disease when they have either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration with distinctive immunohistochemical phenotype of the non-Langerhans cell histiocytes with positive staining for CD68 and negative staining for S-100 protein and CD1a. Our patient undergoes chemiotherapy according to the LCH-II stratification and therapy plan (Vinblastine, Etoposide and Prednisone) and thereafter receives Carboplatin and Etoposide, and Somatostatin. She is alive and clinically well 33 months after onset of symptoms and the lesions don't appear to progress at imaging examinations. In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, the characteristics radiological pattern of Erdheim-Chester disease together the immunohistochemical phenotype of hystiocytic infiltration supports the theory that Erdheim-Chester disease is a unique disease entity distinct.
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PMID:[Erdheim-Chester disease: a non-Langerhans cell histiocytosis. A clinical-case and review of the literature]. 1534 69