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

We present 23 cases of Langerhans cell histiocytosis with central nervous system (CNS) involvement. The major complaints were a mass on the cranial vault in fifteen (65%), visual disturbance in four (16%), polyurea-polydipsia in three (13%), and progressive weakness in all extremities in one patient (4%). Neurological examination revealed no abnormality in sixteen patients (70%), cranial nerve palsy, visual field defect and optic atrophy in six (26%) and paraparesis in one (4%). Tumoral mass was found to be located on the cranial vault (65%), in the suprasellar region (21%) and in the spinal column (8%). The cranium and spinal column were both involved in one patient. All patients underwent surgery; craniectomy with grossly total tumor excision plus cranioplasty (65%), craniotomy with subtotal tumor excision (26%), and vertebrectomy with grafting (13%) were performed. The clinical, radiological and histopathological features, as well as therapeutical considerations are discussed and the pertinent literature is reviewed.
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PMID:CNS involvement of Langerhans cell histiocytosis. Report of 23 surgically treated cases. 900 88

Langerhans cell histiocytosis (LCH) is an enigmatic disease, usually occurring in the young. Despite the fact that clinical entities of the disease have been known for some time, the pathogenesis and etiology remain obscure. A major stride toward understanding LCH was taken when ultrastructural and immunohistochemical studies identified the "histiocytic" cells in LCH lesions to be members of the Langerhans (dendritic) cell system. Another finding of importance was the discovery that LCH cells within the lesions are clonal. Clonality alone, however, is not enough to define LCH as a neoplasm. In this article, we review the recent developments in the pathogenesis and etiology of LCH and discuss the implications of these findings.
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PMID:New insights into the pathogenesis of Langerhans cell histiocytosis. 908 55

Among all patients with a pathologic diagnosis of juvenile xanthogranuloma (JXG) seen at our institution from 1983 to 1994, we identified five patients with an unusual histologic pattern that differed from the classic juvenile xanthogranuloma (CJXG) with foamy cells and Touton giant cells. Four of these five cases, which we termed nonlipidized juvenile xanthogranuloma (NJXG), were seen in infants. The histologic features include a monomorphic infiltrate with absent or few foam cells and Touton giant cells. There is little inflammation, and mitotic figures are easily found. Four cases exhibit a diffuse sheetlike pattern while one is trabecular. Immunoperoxidase staining was done. All lesions are consistently positive for factor XIIIa as opposed to only focally positive or negative in CJXG and negative in Langerhans cell histiocytosis (LCH). The S-100 was negative. NJXG represents an atypical histologic variant of JXG, which may suggest a malignant or aggressive tumor. The follow-up, however, indicates that these lesions behave in a fashion similar to those of CJXG. The differential diagnosis should be made with LCH, intradermal nevus, and reticulohistiocytosis. The immunoperoxidase findings help to differentiate NJXG from these entities.
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PMID:Nonlipidized juvenile xanthogranuloma: a histologic and immunohistochemical study. 914 93

Dendritic cells are antigen-presenting cells derived from the hematopoietic stem cell. The dendritic cell family includes Langerhans' cells (CD1a-positive dendritic cells of the skin), and antigen-presenting cells that are found in the lymphoreticular system and throughout the organ parenchyme. Dendritic cells play a key role in both the primary and secondary immune responses. Several studies indicate that these cells participate in antitumor immunity, tumor surveillance, graft-versus-host disease, and in the pathogenesis of clinical syndromes of unknown origin or those induced by viruses, such as the human immunodeficiency virus. Different disorders are characterized by an abnormal proliferation and accumulation of dendritic cells; for example, the Langerhans' histiocytes, which accumulate in Langerhans' cell histiocytosis. In this review the immunophenotypic, morphological, and functional characteristics of the dendritic cell family is described. The clinical and laboratory studies suggesting a unique role for these cells in various syndromes and diseases are reviewed. The Langerhans' cell histiocytoses and the malignant disorders associated with transformation of cells belonging to the dendritic cell family, are discussed.
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PMID:Physiology and pathophysiology of dendritic cells. 949 Feb 85

Juvenile xanthogranuloma of parotid gland is reported in a 9-year-old boy. This kind of tumor is thought to be very rare in salivary glands. Histological, immunohistochemical and ultrastructural data showed characteristic features and excluded a Langerhans cell histiocytosis. Follow-up was uneventful after 18 months.
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PMID:[Juvenile xanthogranuloma localized in the parotid gland]. 922 Oct

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

A group of 50 pediatric patients affected with tumors or pseudotumors of the spine were studied with the purpose of determining the interval between the onset of symptoms and definitive diagnosis, the incidence of various symptoms, the statistical frequency based on age, sex, histologic type, localization, site. Also studied were diagnostic procedures adopted, therapy, recurrence, complications. The child affected with benign tumor pathology of the spine is rarely submitted early to appropriate diagnostic testing. Tumors are more frequently localized in the lumbar and thoracic spine and there is predilection for the vertebral arch. The most frequent histologic types are in decreasing order: histiocytosis X, osteoid osteoma, and aneurysmal cyst. Treatment is constituted by simple curettage in histiocytosis X, complete resection of the neoplasm in osteoid osteoma and osteoblastoma, partial resection associated with radiotherapy or selective embolization in aneurysmal bone cyst.
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PMID:Benign tumors of the pediatric spine: statistical notes. 971 11

We treated a 46-year-old Japanese man with Langerhans cell histiocytosis (LCH) localized to the eyelid alone. He was cured successfully by local and complete resection. Results of pathological examinations of the excised tumor demonstrated diffuse infiltration by atypical histiocytic cells with eosinophilic cytoplasm and convoluted nuclei, S100 immunoreactivity, and tennis-racket-shaped Birbeck granules. Based on these pathological findings, we diagnosed LCH. Clinical examination revealed no LCH involvement in other parts of the body. To our knowledge, there has been only one report of LCH occurring as an isolated tumor in the eyelid. Generally LCH has been reported in children or young people. This is an unusual case of LCH isolated to the eyelid of an older patient.
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PMID:Langerhans cell histiocytosis localized to the eyelid. 979 Jun 43

The use of fine-needle aspiration biopsy (FNAB) in the initial evaluation of pediatric bone and soft tissue tumors is controversial, especially for those patients being considered for histiogenetic-specific therapeutic protocols, e.g., the Intergroup Rhabdomyosarcoma Study Group, the Pediatric Oncology Group. We retrospectively reviewed 33 consecutive FNAB specimens (28 primary tumors, 5 metastases) from 32 pediatric patients (< or = 19 yr of age), none of whom had a previously established tumor diagnosis. In one patient, FNAB of the primary tumor and a presumed axillary metastasis were obtained concomitantly. The cytomorphologic analysis included osteosarcoma, eight patients; rhabdomyosarcoma, five; neuroblastoma, five; Ewing's sarcoma/primitive neuroectodermal tumor, four; Langerhans' cell histiocytosis, three; and one each synovial sarcoma, undifferentiated sarcoma, infantile myofibromatosis, fibroma, chondroblastoma, chondromyxoid fibroma, and desmoplastic small round-cell tumor. Ancillary studies, e.g., immunocytochemical analysis, were used in 13 cases. Cytogenetic analysis helped to confirm one Ewing's sarcoma [t (11;22) (q24;q12)] and one synovial sarcoma [t(X;18) (p11;q11)]. With adequate FNAB specimens, a histogenetic-specific diagnosis was rendered in 27 (93%) of 29 cases, and all were correctly recognized as either benign or malignant. One case each of Langerhans' cell histiocytosis, chondroblastoma, and infantile myofibromatosis yielded unsatisfactory specimens. Fibroma and desmoplastic small round-cell tumor were initially misclassified as nodular fasciitis and rhabdomyosarcoma, respectively. Of 18 patients clinically eligible for histogenetic-specific therapy protocols, an accurate diagnosis was obtained in 17 patients. With a multidisciplinary approach and judicious use of ancillary studies, FNAB represents a highly accurate and cost-effective technique for the diagnosis of pediatric bone and soft tissue tumors, especially sarcomas, and should be considered as a viable diagnostic technique for pediatric therapeutic protocols.
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PMID:The role of fine-needle aspiration biopsy in the initial diagnosis of pediatric bone and soft tissue tumors: an institutional experience. 979 16

Nine cases of Langerhans' cell histiocytosis (LCH) of the liver are presented. Five of the patients had liver involvement only. Other organ systems, notably the lymph nodes and skin, were involved in the other four patients. Four of the patients had sclerosing biliary disease with infiltration of the bile ducts by Langerhans' cells, whereas in two other patients, the biliary sclerosis was not associated with direct hepatic involvement by Langerhans' cells. Histologically, the lesions were composed of focal aggregates of Langerhans' cells in a polymorphous background of mature eosinophils, lymphocytes, neutrophils, and plasma cells. LCH encompasses a syndrome that has a broad range of clinical presentations and that might involve the liver solely as tumor-like lesions or cystic lesions, or as part of systemic disease. Even when Langerhans' cells are not demonstrable, sclerosing cholangitis can be seen in LCH.
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PMID:Liver involvement in Langerhans' cell histiocytosis: a study of nine cases. 1022 1


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