Primary tumors of the heart and pericardium.
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Calcification of the heart and great vessels is secondary to those diseases which cause a significant alteration of tissue matrix. Metabolic derangements can cause microscopic calcification of heart muscle, but this has not been demonstrated conclusively roentgenographically. The deposition of calcium may follow a single event or be the result of a longstanding process. The etiology and roentgen appearance of calcium deposition in the various tissues which comprise the heart and great vessels are reveiwed. The common as well as the rare cases are considered; if possible, each entity is demonstrated roentgenographically. In addition, an attempt is made to reciew various points of view, which at times are conflicting. The prognostic importance of cardiac, aortic, or pulmonary arterial calcification is underscored whenever possible.
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Epithelioid sarcoma (ES) and malignant rhabdoid tumor (MRT) have heretofore been regarded as two separate clinicopathologic entities. However, they have some histologic similarities, and both represent histogenetic and phenotypic enigmas. This study reports the pathologic and immunohistochemical findings of four vulvar neoplasms occurring in young women that represented diagnostic dilemmas because of their similarity to both ES and MRT. Only one case had the classic histologic features of ES, whereas, in our opinion, the other three cases fulfilled the histologic criteria of MRT, despite the fact that two of the three cases were reported earlier as examples of ES. Neither electron microscopy nor immunohistochemistry has been found to be helpful in separating ES from MRT, mainly because they share several ultrastructural and immunophenotypic features. The behavior of these vulvar tumors--ours and the few published examples of ES--is generally aggressive, more in keeping with MRT than classic ES. We believe that some, if not most, putative ES of the vulva are in fact MRT, a neoplasm with an unfavorable prognosis.
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Report of two cases of Fetal Rhabdomyomatous Nephroblastoma, a cytodifferentiated variant of Wilms' tumor. The incidence of this rare variant is of 2.10% in our material (88 nephroblastomas in a sixteen years period). The patients were ten months and two years old. Bilateral tumors were discovered in both patients. Preoperative radiotherapy was given but no reduction of the tumors size was obtained. Microscopically, benign looking striated muscle is the predominant component of the tumors, with scare foci of epithelial elements. The low age of presentation, high incidence of bilaterality and the peculiar intrapelvic renal growth are three relevant characteristics in the 35 cases previously reported as well as in these two new cases, that distinguish Fetal Rhabdomyomatous Nephroblastoma from conventional Wilms' tumor.
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A wide variety of benign tumors present in the oral cavity. These tumors are for the most part rare and are classified by the tissue of origin. Although benign oral cavity tumors are not life-threatening, they can result in extensive loss of soft tissue and/or bone. Furthermore, many patients are subject to the threat of recurrence, multiple surgical procedures, and the possibility of malignant degeneration. Because many tumors vary little clinically, an adequate biopsy specimen must be obtained for diagnosis. Radiographs are, in general, nondiagnostic. Collaboration with an experienced pathologist is necessary to determine the tumor's probable clinical behavior. Therapy, which is dictated by tumor type, is almost always surgical.
A case of neonatal hypertrophic cardiomyopathy (HCM) without obstruction is reported. Von Recklinghausen neurofibromatosis in the mother and several relatives and the occurrence of café-au-lait spots in the child at 6 weeks of life led to the association of HCM with this phacomatosis. The spontaneous regression of the myocardial hypertrophy after 6 weeks makes this first report of neonatal HCM in the course of Von Recklinghausen neurofibromatosis peculiar. Several reports in the literature mention the possibility of such a spontaneous regression of HCM or diffuse cardiac tumors, especially in the course of phacomatosis.
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A variety of soft tissue tumors can occur in the head and neck area. Because, with the possible exception of rhabdomyosarcoma, they are seen less often in these sites than in the trunk and extremities, they can pose special problems in diagnosis, classification, and management.
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This is the first description in Russian literature of histological and electron-microscopic structure of an exophytic skin mesenchymoma with melanocytic and rhabdomyopoietic differentiation. The presence in the tumour of cells-hybrids possessing the two differentiations allows one to suppose a histogenetic link of this tumours with pluripotential cells of neuroectoderm.
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