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)

We herein describe two unusual neoplasms showing histopathologic features consistent with those of giant cell angiofibroma, which was originally described as a neoplasm arising in the orbit in adults: one of them arose in the right submandibular region of a 48-year-old woman and the other in the right parascapular region of a 49-year-old woman. Macroscopically, although the latter was characterized by a lymphangioma-like cystic appearance, both tumors were well circumscribed and encapsulated. Microscopically, in both cases, pseudovascular spaces lined by a discontinuous row of multinucleated cells were seen against a background of spindle-shaped fibroblastic cell proliferation. In the second case, the tumor presented increased cellularity and plump and somewhat atypical nuclei of proliferating fibroblastic cells, compared with the tumor in the first case. Immunohistochemically, the mononuclear and multinucleated cells within these tumors were positive for vimentin and CD 34 but negative for any other antigens, including Factor VIII-related antigen, desmin, alpha smooth muscle actin, myoglobin, S-100 protein, LeuM1, lysozyme, alpha-1-antitrypsin, and cytokeratins (AE1/AE3 and CAM5.2). The features in these cases indicate that giant cell angiofibroma can arise in an extraorbital site in middle-aged patients and presents some histopathologic diversity.
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PMID:Extraorbital giant cell angiofibromas. 938 57

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.
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PMID:Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases. 1100 38