Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.
 ...
PMID:Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. 631 Feb 27

The diagnostic significance of grooved nuclei in the cytology of extrathyroid lesions is not well-described in the literature. Isolated cases of metastatic adult granulosa cell tumor, reactive mesothelial hyperplasia, Langerhans' cell histiocytosis, recurrent renal cell carcinoma, and round cell liposarcoma were reviewed, and their cytologic features were discussed. While nuclear grooving can be present to a different extent in all these conditions, the significance of this feature in diagnostic cytology varies. The integration of clinical information, radiologic findings, different morphologic features, and immunohistochemistry is necessary before a final conclusion is reached.
 ...
PMID:Diagnostic significance and possible pitfalls of nuclear grooves in extrathyroid cytology. 1057 79

Fat-containing tumors of the liver are a heterogeneous group of tumors with characteristic histologic features, variable biologic profiles, and variable imaging findings. Benign liver lesions that contain fat include focal or geographic fatty change (steatosis), pseudolesions due to postoperative packing material (omentum), adenoma, focal nodular hyperplasia, lipoma, angiomyolipoma, cystic teratoma, hepatic adrenal rest tumor, pseudolipoma of the Glisson capsule, and xanthomatous lesions in Langerhans cell histiocytosis. Malignant liver lesions that can contain fat include hepatocellular carcinoma, primary and metastatic liposarcoma, and hepatic metastases. Identification of fat within a liver lesion can be critical in characterization of the lesion. The imaging characteristics of a lesion coupled with the pattern of intratumoral fatty change are helpful in narrowing the differential diagnosis. Although the presence of fat can be demonstrated with computed tomography or ultrasound, magnetic resonance imaging is the most specific imaging technique for demonstration of both microscopic and macroscopic fat.
 ...
PMID:Fat-containing lesions of the liver: radiologic-pathologic correlation. 1579 52

Bone and soft-tissue sarcomas comprise a rare, complex, and heterogeneous group of tumors for which it is difficult for even experienced pathologists to provide a conclusive diagnosis. The number of diagnoses made using genetic analysis has increased since the detection of fusion genes in several soft-tissue tumors in the 1990s. Moreover, other specific genetic aberrations have been reported in various bone and soft-tissue tumors. In addition, molecular therapeutic targets have been sought in advanced cases of soft-tissue and bone tumors similar to other organ malignancies. To enable the pathological diagnosis of bone and soft-tissue tumors, it is necessary to combine histological diagnosis with immunohistochemistry and gene analysis findings including fusion gene or other genetic aberrations. In this review, we describe the fusion genes recently reported in bone and soft-tissue tumors such as solitary fibrous tumor, aneurysmal bone cyst, nodular fasciitis, CIC-DUX4 fusion gene-positive small round cell tumors, or BCOR-CCNB3-positive sarcoma as well as other genetic aberrations in dedifferentiated liposarcoma, malignant rhabdoid tumor, cartilaginous tumor, Langerhans cell histiocytosis chondroblastoma, or giant cell tumor of the bone. We also demonstrate their association with pathological diagnosis.
 ...
PMID:[Genetic Aberration and Pathological Diagnosis in Bone and Soft-Tissue Tumors]. 2706 46