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1/4. Benign vascular neoplasms of the spleen with myoid and angioendotheliomatous features.

    AIMS: To present the clinical light microscopic and immunophenotypic features of a distinctive vascular neoplasm of the spleen. methods AND RESULTS: Two of the splenic lesions arose in children, and one was found in an adult. They ranged from 19 to 40 mm diameter and histologically were quite similar. Sheets of large epithelioid cells with a spectrum of nuclear configurations ranging from oval and vesicular to twisted and hyperchromatic were noted in each case. Distinct or prominent nucleoli were present in many cells, and occasional cells had nuclear pseudoinclusions. In two cases, bands of basophilic, fibroblast-rich stroma with scattered chronic inflammatory cells were present. The mitotic rate ranged from 0/10 high-power fields (HPF) to 0.5/10 HPF in these epithelioid cells. The vascular nature of these tumours was manifested as a sieve-like array of round, erythrocyte-filled spaces, most with attenuated and cytologically bland lining cells. The polygonal, epithelioid cells exhibited the following phenotype: smooth muscle actin (SMA) , muscle specific actin (MSA) , vimentin , CD31-, CD34-, CD21-, CD8-, CD68- (2/3 cases), S100-, while the lining cells were CD34 , vimentin and SMA-, with variable CD31 and factor viii related antigen expression. Elongated SMA , MSA cell processes were evident in one case, reminiscent of previously characterized myoid elements of the normal spleen. An uneventful follow-up was noted for all three patients. CONCLUSIONS: The histology and immunophenotype set these neoplasms apart from classic hamartomas, haemangiomas and previously characterized (haem)angioendotheliomas of the spleen, and may represent proliferations of myoid elements native to the spleen.
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2/4. Giant fetal hepatic hemangioma. Case report and literature review.

    The purpose of this case report is to demonstrate the importance of prenatal imaging for treatment management of fetal giant hepatic hemangiomas. Prenatal ultrasound revealed an abdominal mass with several cystic areas and punctate calcifications in a fetus at 29 weeks' gestation. Doppler scans confirmed the highly vascular nature of the mass. In this case, ultrasound diagnosed the mass was of hepatic origin, while magnetic resonance imaging at 32 weeks' gestation was more equivocal with respect to the anatomy source of the lesion. Imminent hydrops caused by a rapidly enlarged liver tumor was sonographically demonstrated at 34 weeks' gestation. An elective C-section and immediate tumor resection was performed. At the age of 20 months the infant is thriving. This case supports the notion that the survival rates for giant hepatic hemangiomas improve when fetal hydrops is averted and specific pre- and postnatal treatment is applied based on correct prenatal imaging diagnostics.
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3/4. Primary undifferentiated sarcoma of the thoracic aorta.

    A case of a primary undifferentiated sarcoma of the descending thoracic aorta in a 75-year-old man is reported. Intraluminal growth and occlusion produced congestive heart failure and renal failure. The thoracic aorta was relatively normal on chest radiography so that the lesion was undiscovered until the autopsy. To our knowledge, it is the first case of a primary undifferentiated sarcoma of the thoracic aorta, and the first undifferentiated aortic sarcoma to be examined with immunohistochemistry and electron microscopy. Electron microscopy and immunohistochemistry verified the sarcomatous nature of the undifferentiated neoplasm.
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4/4. An immunohistochemical study on "neoplastic angioendotheliosis": demonstration of B lymphocyte markers in the neoplastic cells.

    Frozen cerebral and renal tissue sections of an autopsied "neoplastic angioendotheliosis (NAE)" case were investigated immunohistochemically using monoclonal and heterologous antibodies to lymphocyte, monocyte, endothelial, epithelial and histiocytic antigens. In both tissues, positive stainings for surface immunoglobulin (sIg) mu and chi, but not lambda, were observed in most of the neoplastic cells. These cells were also positive for other B cell markers (BA-1, Leu-12 and HLA-DR). No distinct staining was observed in the neoplastic cells with antibodies to T lymphocyte (OKT-11 and Leu-1) or monocyte (OKM-1) markers. Positive stainings were observed only in some small round lymphoid cells which were distributed sporadically in and around blood vessels and were considered to be reactive. No positive staining was observed in the neoplastic cells with antibodies to endothelial (factor viii), epithelial (cytokeratin) or histiocytic (lysozyme) antigens. Thus, our NAE case was shown to be of monoclonal B cell lymphoma in nature.
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