Encephalocraniocutaneous lipomatosis and Proteus syndrome.
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A patient with severe isolated right-sided cardiac failure of unknown origin is presented in whom three right ventricular endomyocardial biopsies obtained in different bioptome positions disclosed a yellowish material which was confirmed at histological examination as fat. At operation, a complete replacement of atrial and ventricular myocardium by fat was noted. this previously unreported use of endomyocardial biopsy is emphasized, especially when the differential diagnosis of isolated right-sided heart failure is considered.
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An unusual case of multiple polypoid, benign lipomatous tumors in the ileum of a 53-year-old female with vague gastrointestinal symptoms is described. The pertinent literature is reviewed, and a technique which could have allowed definitive preoperative diagnosis is suggested.
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