Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:3.2.1.17 (lysozyme)
21,489 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pleuropulmonary blastoma (PPB) is a rare and highly aggressive tumor in children and is distinct from ordinary pulmonary blastoma or carcinosarcoma of the lung. This report describes the phenotypical characteristics of seven PPB tumors. The patients were five females and two males, all diagnosed in their third year of life. Five tumors were located within the pulmonary parenchyma, one in the mediastinum, and one involved both lung and mediastinum. Two children are alive and five have died of disease. Histologically, PPB showed a diffuse proliferation of undifferentiated blastemal cells with additional areas of chondroblastic foci (six tumors), storiform pattern (three tumors), alveolar pattern (one tumor), and lipoblastic differentiation (one tumor). Immunohistochemically, tumor cells were positive for vimentin in five of five tumors, histiocytic markers (A1AT and A1ACT, four of six cases; lysozyme, two of six; KP1, three of five) and myogenic markers (desmin, four of six; HHF35, five of six). S-100 was expressed in the chondroblastic areas (in three of four cases). Epithelial membrane antigen and cytokeratin were positive only in epithelial or mesothelial cells entrapped in the tumor. Ultrastructural examination demonstrated a similarity between the proliferating cells in PPB and those seen in malignant fibrous histiocytoma; that is, PPB tumor was mainly composed of a mixture of primitive, fibroblastic, myofibroblastic, histiocytoid, and fibrohistiocytoid cells. Also identified were cells with rhabdomyoblastic differentiation (two tumors) showing thick and thin filaments with Z-discs. In conclusion, PPB showed phenotypical diversity and was composed of MFH-type cells and cells with more specific mesenchymal differentiation.
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PMID:Pleuropulmonary blastoma in childhood. A tumor of divergent differentiation. 768 3

The clinical and pathological features of 15 infants with malignant rhabdoid tumors of kidney are presented. These tumors were identified among 391 primary renal neoplasms in this hospital. The male/female ratio was 2.8:1.0, mean age at diagnosis was 18 months with a range from 4 to 55 months. Of the 10 patients with follow-up records, 8 have died, 2 were alive and free of disease 15 and 55 months after the diagnosis respectively. A wide histologic spectrum was encountered. All tumors exhibited classical morphology in at least some areas, characterized by solid proliferation of monotonous tumor cells with vesicular nuclei and prominent nucleoli, abundant cytoplasm and intracytoplasmic inclusions. Immunohistochemical studies were performed in all 15 cases. Vimentin was positive in all tumors, ENA in 12, cytokeratin in 8, desmin in 2 and myoglobin in one. All cases were negative for presence of lysozyme, NSE and neurofilament. Our results show that this tumor is a distinctive and highly malignant neoplasm of the infant kidney with considerable morphological and immunohistochemical diverse phenotypes.
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PMID:[Malignant rhabdoid tumor of kidney: a clinicopathologic and immunohistochemical study of 15 patients]. 778 29

Giant cell myocarditis (GCM) is a rare condition whose histologic hallmark, the multinucleate giant cell, is of debated origin (monocytic v myogenic). We report the case of a 46-year-old woman with a previous diagnosis of ulcerative colitis who rapidly deteriorated and died as the result of refractory ventricular tachyarrhythmias. Postmortem examination showed a diffuse infiltration of the myocardium by round cells and multinucleate giant cells. Immunohistochemically, round cells were demonstrated to be T lymphocytes admixed with monocytes. Multinucleate giant cells expressed monocytic markers (MAC 387, lysozyme) and were negative for muscle markers (actin, desmin, myoglobin). This case illustrates the monocytic and macrophagic nature of multinucleate giant cells and lends support to the autoimmune hypothesis of GCM by the concurrence of the latter with ulcerative colitis.
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PMID:Giant cell myocarditis: monocytic immunophenotype of giant cells in a case associated with ulcerative colitis. 782 9

The clinicopathological and immunohistochemical features of four patients with systemic multicentric reticulohistiocytosis (MR) were compared with five cases of solitary and one case of multiple reticulohistiocytoma (RH), which were confined to the skin only. The MR cases mostly affected the limbs of older women, while RH affected young male adults without preference to site. Characteristically, both entities consisted of oncocytic mononuclear histiocytes (with granular eosinophilic cytoplasm similar to oncocytic thyroid cells) and multinucleated histiocytes with a ground-glass appearance, which appeared to be much larger (> 200 microns) and bizarre in cases of RH compared with cases of MR (50-100 microns). In RH a variable number of vacuolated, spindle-shaped, and xanthomatized mononuclear histiocytes were also present. Immunohistochemical profiles showed positivity of mononuclear histiocytes with HHF35, factor XIIIa, and LN3 (HLA-DR), with a variable number of multinucleated histiocytes in RH showing binding with peanut agglutinin. In mono- and multinucleated histiocytes in both entities macrophage markers KP1 (CD68), KiM1P, HAM56, lysozyme, and alpha 1-antitrypsin were positive. However, macrophage markers MAC387 (L1 antigen) and Leu-M1 (CD15) were negative. Vimentin was universally positive in both conditions, with all other markers (S100, desmin, smooth muscle-specific actin, and QBEnd 10 [CD34]) negative. This study shows that histology supplemented by immunocytochemistry delineates MR from RH and immunohistochemical profiles indicate a cell lineage relationship between RH and adult xanthogranuloma.
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PMID:Reticulohistiocytoma and multicentric reticulohistiocytosis. Histopathologic and immunophenotypic distinct entities. 859 81

Six cases of hepatic sarcoma are reported: leiomyosarcoma in two, malignant fibrous histiocytoma in two malignant hemagiopericytoma in one and fibrosarcoma in one. In addition to the routine paraffin section and HE stain, immuno-histochemical studies with antibodies against vimentin, EMA, CK, S100, ACT, AAT, desmin, AFP, lysozyme and factor VIII and Masson trichrome staining and argyrophilia staining were done. AFP was negative in all 6 patients and the primary sarcoma was characterized by the absence of accompanying liver cirrhosis. The diagnosis, histogenesis and prognosis of primary liver sarcoma are discussed.
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PMID:[Primary sarcoma of the liver]. 795 5

Abrikosov's tumour was studied light- and electron-microscopically and immunohistochemically with the use of antibodies against desmin, myoglobin, vimentin, cytokeratin, glial fibrillar acid protein, neuron-specific enolase, S-100 protein, neurofilaments (68 kd), lysozyme, alpha-1-antichymotrypsin, alpha-1-antitrypsin, structural components of extracellular matrix (collagen I, II, III, IV, V types and fibronectin). The presence of neurofilaments, apart from vimentin, S-100 protein and small amount of neurospecific enolase, in the tumour cells is demonstrated for the first time. Neurofilament expression by tumour cells and the lack of the basal membrane collagen (type IV) in the extracellular matrix indicate the cell differentiation different from that of Schwann cells. A similar cell immunophenotype is typical for tumours of sympathetic ganglion and paraganglion structures. Both these results and literature data prove phenotypic heterogeneity of the granular cell tumours and their histogenetic link with the cells of nervous comb.
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PMID:[Neurofilaments in Abrikosov's tumor (an immunohistochemical study)]. 798 61

Few published reports describe patients with giant cell fibroblastoma, a rare, benign soft-tissue tumor that recurs locally and predominantly arises in children. A 4-year-old boy underwent surgery for removal of a giant cell fibroblastoma in the paranasal region, an unusual site. Six months after excision the tumor recurred locally. Immunohistochemical examination of the primary tumor and recurrence revealed vimentin positive staining in the cytoplasm of all the cells. The multinucleated giant cells and the flat cells bordering the vessel-like spaces were negative for Factor VIII-related antigen, S-100 protein, actin and desmin. Some histiocytes stained positively for alpha-1-antitrypsin, alpha-1-antichymotrypsin, antimacrophage and lysozyme antibodies. These immunoreactions indicate that giant cell fibroblastomas have a fibrohistiocytic origin.
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PMID:Paranasal giant cell fibroblastoma: case report and immunohistochemical findings. 804 95

Forty two cases (46 lesions) of inflammatory fibroid polyp (IFP) of the stomach were reviewed histologically and studied immunohistochemically. The paraffin sections were stained with a panel of antibodies against alpha-smooth-muscle actin, HHF-35, desmin, vimentin, lysozyme, alpha-1-antitrypsin, alpha-1-antichymotrypsin, KP1, Mac 387, S-100 protein, neuron-specific enolase, factor VIII RAg, and with Ulex europaeus agglutinin I. The lesions ranged in size from 0.3 to 3.5 cm with a mean of 1.2 cm. Forty (87.0%) IFPs occurred in the antrum, five in the gastric body, and one in the cardia. In all but two lesions, the mucosal layer was involved, and 20 (43.5%) lesions were entirely restricted to the mucosa. All 46 IFPs tested diffusely positive with vimentin. Positive reactions for alpha-smooth-muscle actin and HHF-35 were observed in 12 (26.1%) and 10 (21.7%) IFPs, respectively. Two lesions with alpha-smooth-muscle actin and one lesion with HHF-35 showed a diffuse staining. Seventeen (37.0%) lesions were focally positive for KP1, and seven (15.2%) IFPs were focally positive for Mac 387. All other antibodies and agglutinin were negative with the proliferating cells. The results of this study confirmed (a) the presence of myofibroblastic and histiocytic lines of differentiation in addition to the main fibroblastic features for the main cellular component in IFP, and (b) the heterogeneity of the immunoprofile of IFP.
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PMID:Inflammatory fibroid polyp of the stomach. A special reference to an immunohistochemical profile of 42 cases. 821 61

Thirteen cases of juvenile xanthogranuloma (JXG) and 13 cases of adult-type xanthogranuloma (AXG) were compared at the light and immunohistochemical levels. Histologically, four main cell types (vacuolated, xanthomatized, spindle-shaped, and "oncocytic") were seen in variable proportions (from monomorphous to mixed variants) with different types of giant cells (nonspecific, foreign body, Touton, and "ground-glass"). Giant cells were more prominent in AXG than in JXG; oncocytic cells (characterized by an eosinophilic, slightly granular cytoplasm similar to thyroid oncocytic cells) and mostly periodic acid-Schiff (PAS) negative giant cells with a ground-glass appearance (6 of 26) were not observed in classic JXG (i.e., occurring in children < 2 years old). Immunohistochemically, JXG and AXG gave similar results: most xanthogranuloma cells labeled strongly with KiM1P and vimentin, while HHF35 and HAM56 stained less intensively. Factor-XIIIa (FXIIIa), KP1 (CD68), and HAM56 stained mostly in the periphery of the lesions. Some markers gave variable results: peanut agglutinin (PA), 60%; alpha-1-antitrypsin, 50%; lysozyme, 25%; LN3 (HLA-DR), < 10% of cells positive. Others were negative: S-100, MAC387 (L1 antigen), LeuM1 (CD15), desmin, smooth muscle-specific actin, and QBEND10 (CD34). This profile helps to delineate xanthogranuloma from histological stimulants such as dermatofibroma (which is FXIIIa+, LN3+, KP1-, and PA-) and multicentric reticulohistiocytosis (which is FXIIIa-, KP1+, PA-, and HHF35-).
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PMID:Juvenile and adult xanthogranuloma. A histological and immunohistochemical comparison. 829 51

A prospective clinicopathological study including 100 consecutive hysterectomies and 100 consecutive cone specimens to investigate the frequency and pathogenesis of multinucleated stromal giant cells (SGCs) in the uterine cervix is presented. Sections containing SGCs were analyzed by immunohistochemical methods for the presence of cytokeratin, S-100 protein, factor VIII, desmin, muramidase, alpha 1-antichymotrypsin, and vimentin. Tissue for electron microscopy was punched out of four paraffin blocks from four different cases. SGCs were not found in patients < 30 years old, but their incidence increased with age until the sixth decade, at which time they were found in 42% of patients. We found at the same time that the incidence of SGCs declines in the seventh decade. SGCs were not related to any inflammatory or granulomatous lesion, and their presence was independent of other clinical parameters, such as hormonal therapy, history of trauma on the uterine cervix, pregnancy, or parity in general. Immunohistochemical and ultrastructural analyses support the concept of a fibroblastic-histiocytic origin of SGCs. SGCs in the uterine cervix may reflect a physiological tissue reaction during the transition from the fertile to the postmenopausal state.
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PMID:Multinucleated stromal giant cells of the uterine cervix. 834 59


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