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)

Clinical and demographic data as well as the course of illness were analyzed in a retrospective study of 50 patients with a primary tumor or a malformation of the calvarial bones. The most frequent histological diagnosis was "eosinophilic granuloma of bone" (found in 42% of cases), followed by hemangioma, osteoma, dermoid, epidermoid and malignant tumors, and fibrous dysplasia. Local recurrence was observed in 1 patient with ossifying fibroma and 1 with a dermoid tumor, while further dissemination of illness was observed in 3 patients with "eosinophilic granuloma". In these cases, systemic drug therapy is needed. The necessity of an intensive search for further signs of illness and long follow-up periods in patients with primary localized histiocytosis X is stressed.
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PMID:[Tumors and space-occupying abnormalities of the skull calotte]. 325 4

Neoplasms and pseudotumors of the temporomandibular joint (TMJ) are very uncommon. Early recognition of such will prevent therapeutic delay and may have a dramatic impact on patient morbidity and mortality. Included in rare TMJ lesions are the following: 1) synovial chondromatosis, 2) osteochondroma, 3) osteoma, 4) osteoblastoma, 5) pigmented villonodular synovitis, 6) ganglion, 7) synovial cyst, 8) simple bone cyst, 9) aneurysmal bone cyst, 10) epidermal inclusion cyst, 11) hemangioma, 12) nonossifying fibroma, 13) Langerhans cell histiocytosis, 14) plasma cell myeloma, 15) sarcoma.
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PMID:Temporomandibular joint neoplasms and pseudotumors. 1107 60

Congenital self-healing reticulohistiocytosis is a variant of Langerhans cell histiocytosis. It is present at birth or appears in the neonatal period and involutes spontaneously within 3 to 4 months. Although the skin eruptions in most cases consist of papulonodules, several patients with vesicular or bullous lesions have been reported. We describe a case of congenital self-healing reticulohistiocytosis presenting hemorrhagic bullae that mimicked hemangioma.
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PMID:Congenital self-healing reticulohistiocytosis presenting with hemorrhagic bullae. 1273 83

Congenital self-healing reticulohistiocytosis (CSRH), a rare benign variant of Langerhans cell histiocytosis (LCH), is characterized by (a). congenital skin lesions, (b). a healthy infant with no systemic involvement, (c). the histopathologic finding of a Langerhans cell infiltrate and (d). spontaneous involution within the first year of life without sequelae. We report a Taiwanese girl born with widespread hemangioma-like lesions. The diagnosis of LCH was confirmed by finding a diffuse dermal infiltrate of S-100-protein- and OKT6 (CD1a)-positive mononuclear cells and the presence of Birbeck granules in 10% of the mononuclear cells ultrastructurally. The diagnosis of CSRH was further established by rapid and complete involution of the lesions in 3 months. No recurrence was noted for 7 years. Our case illustrates that CSRH can mimic diffuse neonatal hemangiomatosis clinically; thus, it is important to include CSRH in the differential diagnosis of congenital or neonatal hemangiomatosis.
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PMID:Congenital self-healing reticulohistiocytosis mimicking diffuse neonatal hemangiomatosis. 1505 4

CD163, a hemoglobin scavenger receptor, is expressed in monocytes and macrophages. We tested the expression of the CD163 protein in 1,105 human malignancies and normal tissues using tissue microarrays and conventional paraffin-embedded tissue sections. Besides staining nonneoplastic monocytes and histiocytes (tissue macrophages), membranous/cytoplasmic staining for CD163 was primarily limited to neoplasms with monocytic/histiocytic differentiation. CD163 reactivity was not observed in normal tissues, lymphomas, carcinomas, and in a majority of mesenchymal neoplasms, including follicular dendritic cell tumors (0 of 4), although it stained admixed histiocytes. Staining for CD163 was seen in Rosai-Dorfman disease (5 of 6), histiocytic sarcoma (3 of 4), littoral cell angioma (6 of 6), and Langerhans cell histiocytosis (3 of 5). A subset of atypical fibrous histiocytomas (9 of 16), benign fibrous histiocytomas (6 of 9), and atypical fibroxanthomas (1 of 3) also showed CD163 staining. Our studies also confirm earlier work showing that CD163 is expressed in acute myeloid leukemia with monocytic differentiation (AML, FAB subtype M5) (2 of 6), as well as a majority of giant cell tenosynovial tumors (7 of 8). Its limited range of expression and tissue specificity indicate that CD163 may have significant diagnostic utility in separating specific tumors with monocytic and histiocytic derivation from other entities in their differential diagnosis.
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PMID:Expression of CD163 (hemoglobin scavenger receptor) in normal tissues, lymphomas, carcinomas, and sarcomas is largely restricted to the monocyte/macrophage lineage. 1583 85

Facial swelling is a common clinical problem in pediatric patients. The causes of swelling are diverse, and knowledge of the typical clinical and imaging manifestations and the most common sites of occurrence of these conditions is needed to formulate a differential diagnosis. The general clinical manifestations may be classified into the following four groups: (a) acute swelling with inflammation, (b) nonprogressive swelling, (c) slowly progressive swelling, and (d) rapidly progressive swelling. Conditions that may account for acute swelling accompanied by inflammation include lymphadenitis, sinusitis, odontogenic infection, and abscess. Contrast-enhanced computed tomography is the modality of choice for detection of abscesses requiring surgical drainage. Nonprogressive midfacial swelling is suggestive of a congenital anomaly (eg, a cephalocele, nasal glioma, or nasal dermoid or epidermoid cyst). Slowly progressive swelling may indicate the presence of a neurofibroma, hemangioma, lymphangioma, vascular malformation, or pseudocyst, or of fibrous dysplasia. The differential diagnosis for rapidly progressive facial swelling in association with cranial nerve deficits should include rhabdomyosarcoma, Langerhans cell histiocytosis, Ewing sarcoma, osteogenic sarcoma, and metastatic neuroblastoma.
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PMID:Causes of facial swelling in pediatric patients: correlation of clinical and radiologic findings. 1641 50

The use of contrast-enhanced ultrasound (CEUS) for the study of peripheral lung lesions has never been systematically investigated. We evaluated the CEUS patterns of 98 peripheral lung lesions before performing US-guided fine-needle biopsies (FNB). The examinations were done with an Esaote Technos MPX scanner in the harmonic mode with a mechanical index of 0.04 or less. Contrast enhancement was achieved with a 4.8 mL bolus of SonoVue (Bracco) administered via an antecubital vein. All FNBs performed under CEUS guidance were adequate for pathologic diagnosis. Seventy-eight lesions were malignant: 33 (41.9%) were adenocarcinomas, 29 (36.5%) were squamous-cell carcinomas, 13 (17.6%) were undifferentiated large-cell carcinomas and the remaining three (4.1%) were small-cell carcinomas. All presented intralesional enhancement consistent with tumor neovascularization. In some cases, there were unenhanced areas consistent with zones of necrosis and these areas were avoided during FNB. The other 20 lesions were benign (four lipomas, two fibrous lung tumor, two noncaseous granulomas, six abscesses, one rheumatoid nodule, one histiocytosis X, one chondroid hamartoma, one sclerosing hemangioma, two sarcoid nodules) and none presented intralesional enhancement. This initial and admittedly limited experience suggests that CEUS may provide diagnostically useful information on peripheral lung lesions and increase the diagnostic yield of transthoracic FNB by reducing the risk of inadequate tissue sampling.
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PMID:Contrast-enhanced ultrasound (CEUS) for the study of peripheral lung lesions: a preliminary study. 1704 65

A wide variety of bowel diseases, some of which are unique to or more prevalent in pediatric patients, may manifest with intestinal wall thickening at computed tomography (CT). Common causes of bowel wall thickening include edema, hemorrhage, infection, graft-versus-host disease, and inflammatory bowel disease; more unusual causes include immunodeficiencies, lymphoma, hemangioma, pseudotumor, and Langerhans cell histiocytosis. Radiologists must be familiar with the CT signs of bowel disease and should take careful note of the bowel characteristics (eg, extent and distribution of disease involvement, bowel dilatation, mural stratification, perienteric findings) to generate an adequate differential diagnosis. The study should be tailored and optimized in advance according to the clinical scenario to decrease radiation exposure due to repeated or delayed scanning. With spiral CT scanners, studies can be performed quickly, thereby eliminating the need for sedation, and multiple reconstructed images can be generated. CT is an invaluable diagnostic tool in the evaluation of pediatric diseases involving the bowel, in spite of the use of ionizing radiation.
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PMID:Bowel wall thickening in children: CT findings. 1848 Apr 81

We conducted a retrospective case-series review to identify the various diagnoses of neoplasms of the nasal cavity and paranasal sinuses in a pediatric population. Our study group was made up of 54 children-23 boys and 31 girls, aged 8 months to 16 years (mean: 9 yr). All patients had been diagnosed with a tumor of the nasal cavity or paranasal sinuses between Jan. 1, 1955, and Dec. 31, 1999, at one of four university-based, tertiary care referral centers. We compiled data on tumoral characteristics (location, size, and histopathology), morbidity and mortality, and rates of recurrence. Lesions included adnexal neoplasm, ameloblastic fibro-odontoma, basal cell carcinoma, benign fibrous histiocytoma, blue nevus, chondrosarcoma, compound nevus, epithelioma adenoides cysticum, esthesioneuroblastoma, Ewing sarcoma, fibrosarcoma, giant cell granuloma, granulocytic sarcoma, hemangioma, hemangiopericytoma, Langerhans cell histiocytosis, lymphangioma, lymphoma, melanoma, neuroblastoma, neurofibroma, ossifying osteofibroma, osteochondroma, osteosarcoma, port wine stain, rhabdomyosarcoma, Spitz nevus, and xanthogranuloma. To the best of our knowledge, this is the largest such study of its kind to date. We believe that the large size of this study and the data on disease incidence will allow clinicians to be better informed of the differential diagnosis of neoplasms of the nasal cavity and paranasal sinuses in the pediatric population.
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PMID:Differential diagnosis of pediatric tumors of the nasal cavity and paranasal sinuses: a 45-year multi-institutional review. 2108 77

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


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