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

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

The authors present an immunohistochemical study of 11 cases of maxillo-facial primitive sarcomas. Specimens from demoliti maxillary resections were prepared and stained with alpha-1-antichymotrypsin, lysozyme and CD68. Alpha-1-antichymotrypsin confirmed in this study its lack of specificity as a tumor marker being relevated both in fibroblasts and in osteoblasts and even in chondrosarcomatous tissue. The results of lysozyme and CD68 stainings were interesting especially in malignant fibrous histiocytoma (MFH), fibrosarcoma and osteosarcoma. The authors showed, once more, that while in osteosarcoma the markers were noted in osteoclasts or pre-osteoclasts alone and not in the neoplastic stroma; all fibroblastic elements were marked in MFH. Immunohistochemical research of histiocyte-macrophage lineage confirmed its utility in osteosarcoma versus MFH differential diagnosis. In fibrosarcoma, furthermore, the authors obtained a positive staining of CD68 and lysozyme in fibroblastic elements morphologically similar to the other neoplastic cells. This datum induced the authors to formulate the interesting hypothesis that MFH and fibrosarcoma represent the opposite ends of a wide spectrum of differentiation of a single neoplasm of fibrohistiocytic origin.
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PMID:[Histological and immunohistochemical studies in cases of malignant mesenchymal neoplasms of the oromaxillofacial area]. 807 67

This paper reports 5 patients with MFH in the soft tissues and 10 in the jaws. Fourteen patients (5 in soft tissues and 9 in jaws) underwent surgery, 5 and 3 of the 9 bone cases received additional irradiation and chemotherapy respectively. Fourteen patients had been followed up for 6 months to 4 and 1/2 years. Two patients of jaw had local recurrences 1 to 8 months following surgery; 6 of 9 ones with follow-up died, 2 of whom were associated with pulmonary metastases. MFH of bone showed significantly worse prognosis than those localized in soft tissues, particularly in maxilla. Pathologically, the tumors of this series were divided into 5 types. The relation between the mitosis and the malignancy of MFH in the jaws was significant. Immunohistochemical stains for lysozyme, A, AT and ACT showed that ACT was the most sensitive marker for MFH.
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PMID:[Clinical and pathological studies of malignant fibrous histiocytoma (MFH) in the oral and maxillofacial regions with report of 15 cases]. 819 19

By clinical, pathologic and immunohistochemical study on aniomatoid malignant fibrous histiocytoma, which is a relatively uncommon soft tissue tumor described by Enzinger in 1979, and often misdiagnosed, we are reporting 32 cases of this lesion. The distinctive histopathology were: (1) Cystic change filled with hemorrhagic fluid or blood, (2) surrounded by nests of fibroblastlike and histocyte-like cells and (3) intermingled with chronic inflammatory cells, (4) often surrounded by a fibrous pseudocapsule. Immunohistochemical staining done in 4 cases showed all to be positive in lysozyme, three positive in Mac 387 and CD 68. These results support their histiocytic origin. Follow-up information was available in 25 of 32 cases. All the 25 patients were alive, 4 with recurrence (16%), 1 with metastasis (4%). These results concur with the opinion that intrinsically, this is a low grade tumor.
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PMID:[Angiomatoid malignant fibrous histiocytoma]. 876 38

An extremely rare case of malignant fibrous histiocytoma in the pancreas is reported. A 70-year-old man complained of upper abdominal discomfort. A tumor in the head of the pancreas was demonstrated by ultrasonography and computed tomography. The surgical specimen revealed a relatively well demarcated tumor, 9 x 7 x 6.5 cm in size. Microscopically, fibroblastic, histiocytic, and multinucleated giant tumor cells were observed in the myxoid area, but some tumor cells had proliferated in a storiform-pleomorphic pattern. Immunohistochemically, some tumor cells were positive for lysozyme, alpha-1-antitrypsin, alpha-1-antichymotrypsin, and vimentin. Electron microscopically, tumor cells showed a combination of fibroblastic and histiocytic features. The patient is currently well with no evidence of recurrence or metastasis 22 months after operation.
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PMID:Malignant fibrous histiocytoma of the pancreas. 887 8

A histologic, immunohistochemical, and DNA ploidy analyses were performed on two cases of angiomatoid malignant fibrous histiocytoma to ascertain the histogenesis and relationship of endothelial, histiocytic, and fibroblastic elements. Both cases were slowly growing, grossly encapsulated. Subcutaneous masses resected from pediatric patients. Microscopically, the tumors were composed of solid masses of epithelioid and spindle cells with abnormal endothelial-lined and blood-filled cystic spaces surrounded by normal vascular structures and aggregates of lymphocytes occasionally forming germinal follicles. The tumor cells stained exclusively with CD34 and vimentin antibodies. Tumor-associated vessels stained for CD31, CD34, vimentin, and Ulex europaeus. Occasional cells within germinal follicles stained for lysozyme, CD68, and HAM56. Ploidy analysis of tumor cells showed intermediate aneuploidy with a DNA index of 1.14. Blood vessels within and surrounding the tumor as well as inflammatory cells were DNA euploid. These studies suggest that the tumor--though comprised of histologically and immunohistochemically benign-appearing euploid endothelial, fibroblastic, and inflammatory elements--contains an aneuploid population of undifferentiated mesenchymal cells.
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PMID:Angiomatoid malignant fibrous histiocytoma revisited. An immunohistochemical and DNA ploidy analysis. 965 Jul 10

Although "giant cell tumor of soft parts" has traditionally been considered a single entity as reflected in the original term "malignant giant cell tumor of soft parts (MGCT)" and later by the term "malignant fibrous histiocytoma, giant cell type" the degree of atypia and mitotic activity varies in this group, suggesting biologic heterogeneity. The clinicopathologic features of 31 tumors meeting the traditional criteria of MGCT but having only mild to moderate nuclear atypia are presented. Patients with these tumors (19 females; 12 males) ranged in age from 14 to 84 years (mean, 40 years) and presented with masses of involving either superficial (n = 16) or deep (n = 13) soft tissue. Most occurred on the arm or hand (n = 16) and ranged in size from 0.7 to 6.5 cm (mean, 2.1 cm). The tumors consisted of sheets and nodules of rounded mononuclear cells that blended with spindled cells and benign osteoclastic giant cells. Pleomorphic giant cells were absent. Osteoid was noted in 10 cases, but features typically associated with tenosynovial giant cell tumors (such as dense stromal hyaline, siderophages, and xanthoma cells) were nearly always absent. Mitotic figures ranged from 1-10/10 HPF (mean, 2-3/10 high-powered field), and angiolymphatic invasion was present in 10 cases. Necrosis was absent, however. The mononuclear cells expressed CD68, tartrate-resistant acid phosphatase, and smooth muscle actin, but lacked CD45, S100 protein, desmin, and lysozyme, an immunophenotypic profile identical to that of giant cell tumor of bone. Follow-up information in 19 patients (mean, 3 yrs; median, 1-7 yrs) indicated recurrences in four patients, but none developed metastasis. This behavior contrasts significantly with the high-grade behavior traditionally associated with MGCT of soft parts. These giant cell tumors can be consistently recognized by the lack of cytologic atypia even in the face of mitotic activity and vascular invasion. Although their long term metastatic risk is not fully defined, we propose they be termed "giant cell tumors of low malignant potential" and regarded as the soft tissue analogue of giant cell tumor of bone. The term "malignant giant cell tumor of soft parts" or giant cell malignant fibrous histiocytoma should be restricted to histologically high-grade lesions.
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PMID:Soft tissue giant cell tumor of low malignant potential: a proposal for the reclassification of malignant giant cell tumor of soft parts. 1049 98

Malignant histiocytosis was diagnosed in 4 cows. In all cases the tumor tissues were composed of cytologically atypical histiocytes with evidence of erythrophagocytosis. The tumor in case 1 appeared highly anaplastic with marked nuclear pleomorphism, and had areas of spindle cell differentiation, but had no relation to malignant fibrous histiocytoma. The neoplastic tissue in case 2, characterized by cohesive growth of tumor cells, was distinguishable from anaplastic carcinoma cells by cytokeratin immunostaining. There were many hemosiderin-laden neoplastic cells suggestive of high phagocytic activity in a lymph node of case 3. The neoplastic cells in case 4, frequently multinucleated, were less atypical than in the other cases. All cases expressed histiocyte-associated markers (lysozyme and HAM56), and were negative for cytokeratin, S100, and T- and B-cell lineage-specific markers (CD3 and CD79a). The most frequent HAM56 immunoreactivity was detected in case 4, and the giant, multinucleated forms, reminiscent of epithelioid cell differentiation. seemed not to indicate cytological pleomorphism as a result of neoplastic transformation.
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PMID:Malignant histiocytosis in cattle. 1119 37

Although malignant fibrous histiocytoma (MFH) is one of the most common soft tissue sarcomas, its pathogenesis remains unclear. In this study, a cell line derived from human MFH, TNMY1, was established from a metastatic chest-wall lesion of a 60-year-old woman with MFH. The TNMY1 cell line was passaged 95 times, and it still retained the biological characteristics of the original tumor. TNMY1 consists of spindle-shaped cells and pleomorphic cells associated with multinucleated giant cells. Immunohistochemical studies showed that the spindle-shaped and pleomorphic cells were positive for vimentin, CD68 and alpha-smooth muscle actin, but negative for epithelial membrane antigen, desmin, muscle actin, alpha-sarcomeric actin, myoglobin, lysozyme and S-100 protein. The cells expressed collagen types I, III and V. These results indicate that MFH may originate from mesenchymal stem cells with the potential to differentiate into either fibroblasts or histiocytes. An elevated level of collagen type V mRNA expression is considered to support a diagnosis of MFH.
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PMID:Establishment and characterization of cell line TNMY1 derived from human malignant fibrous histiocytoma. 1156 13


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