[Proteus syndrome and encephalo-cranio-cutaneous lipomatosis, are they the same hamartoma? Apropos of a case].
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Biomedical subjects
Publications and source records attributed to F Contreras Rubio.
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OBJECTIVES: Since the initial report of renal hemangiopericytoma by Black and Heinemann in 1955, only 24 cases of this rare vascular neoplasm, involving the renal capsule, parenchyma or sinus have been described in the world literature. Herein we describe an additional case and review the literature. METHODS: We report on a 65-old woman in whom a 5 x 4 cm solid mass was incidentally found in the left sinus. The tumor compressed the renal pelvis and caused hydronephrosis and parenchymal atrophy. A radical left nephrectomy was performed. Histological and immunological studies revealed a hemangiopericytoma of renal sinus soft tissues. RESULTS: The patient did well postoperatively and remains disease-free at the present time. CONCLUSIONS: Although there are other histological and clinical criteria indicative of worse prognosis, the malignancy of hemangiopericytoma is determined by the presence of hematogenous metastases.
Report of the microscopic, ultrastructural and immunohistochemical findings of two malignant rhabdoid tumors of the kidney in two males aged 2 1/2 and 19 months. The incidence of this tumor in our files is 1.9% of all the renal tumors of infancy. Both cases were formally considered to be rhabdomyosarcomatous variants of nephroblastomas. Microscopically a slightly cohesive polygonal cell proliferation is shown. The cells have intracytoplasmatic hyaline inclusions ultrastructurally identifiable as intermediate filament bundles, showing positivity to vimentin in the immunohistochemical technique. Malignant rhabdoid tumors are referred to as very aggressive tumors with a poor response to treatment and a high mortality rate. Association with central nervous tumors and hypercalcemia has been reported. The clinical and pathologic features make it possible to differentiate them from nephroblastomas, although the histogenesis is not definitely established.
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