Cases reported "Myxoma"

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1/20. Corneal myxoma associated with keratoconus and Down's syndrome.

    PURPOSE: Primary myxoma of the cornea is extremely rare. Until now, only four primary corneal myxomas were reported in the literature, whereas secondary involvement of the cornea by conjunctival and limbal tumors is much more common. methods: We report an additional case in a 26-year-old woman with keratoconus and Down's syndrome. Excision of the corneal mass was performed by penetrating keratoplasty. Histochemical, immunohistochemistry, and ultrastructural studies were used to obtain a definitive diagnosis. RESULTS: The tumor exhibited the characteristic histologic features of myxoma. The tumor cells showed immunoreactivity for vimentin but not for S-100 protein, epithelial membrane antigen, CAM 5.2, HHF-35, or muscle-specific actin. Ultrastructural features were fibroblast-like or stellate cells with cytoplasm containing abundant, rough reticulum and dilated cisternae. No recurrence was observed 36 months after penetrating keratoplasty. CONCLUSION: This is only the fifth report of such an occurrence. Although the coexistence of myxoma in Down's syndrome with keratoconus is described here for the first time, the differential diagnosis of apparently evident acute hydrops on clinical inspection should not rule out the possibility of a corneal myxoma. Histologic analysis should therefore be performed.
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2/20. Tumorous deformity of mitral valve leaflet after chordal rupture in a child.

    A case with tumorous deformity of the posterior mitral valve leaflet after spontaneous chordal rupture in a child is described. A partial rupture in the chordae tendineae of the posterior mitral leaflet was found by echocardiography in a 9-year-old Japanese boy. Tumorous bulging was gradually developed in the leaflet and was surgically excised 5 years later. Multiple nodular tumors were found on the atrial surface of the posterior mitral leaflet. Histological examination revealed that the tumorous bulging consisted of myxomatous materials in which collagen fibrils and very fine elastic fibers were distributed loosely and irregularly. Normal-looking endothelial cells covered the luminal surface of the bulging lesion. vimentin-positive spindle-shaped mesenchymal cells were scattered in the bulge area. The labeling index of proliferating cell nuclear antigen (PCNA) in these cells was 29.3%. These spindle cells were positive for matrix metalloproteinase (MMP)-1 in the entire bulge area. The cells and matrix were positive for MMP-2 and tissue inhibitor of MMP (TIMP)-1 in the basal area of bulging, but were weakly positive or negative at the surface area. Reactivity for TIMP-2 in the cells in the bulge area was obviously weaker than that in the cells at the spongiosa of the anterior mitral leaflet, which was obtained from the patient at the valve replacement operation 9 months after the initial operation. These findings indicated that the tumorous deformity of the mitral valve was formed by the overgrowth of valve tissue under the stimulation of mitral regurgitation in this child, and the imbalance of MMP and TIMP might play an important role in the bulge formation.
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3/20. A case of perineurioma with prominent myxoid changes.

    A perineurioma with prominent myxoid changes is presented. The patient, a 41 year-old woman, presented with a small skin nodule on her back. light microscopy revealed subtle cellular whorls within a myxoid background. immunohistochemistry showed epithelial membrane antigen positivity. In contrast, S-100 protein, common leukocyte antigen, vimentin and AE1-AE3 cytokeratin were negative.
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4/20. Cardiac myxoma with a cytokeratin-immunoreactive glandular component.

    This study documents the expression of cytokeratin intermediate filaments (IFs) in a surgically excised glandular atrial myxoma. The glandular structures showed also positivity for carcinoembryonic antigen. Typical isolated or cordlike myxoma cells contained vimentin IFs. Positivity for von willebrand factor was detected in cells lining deep invaginations of surface papillae and vascular channels. Bundles of smooth muscle cells and myofibroblasts scattered throughout the myxoid matrix synthesized the isoform of alpha-actin specific for smooth muscle.
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5/20. Nerve sheath myxoma (neurothekeoma)--a case report.

    A case of nerve sheath myxoma also called as neurothekeoma in a 33-year-old woman is described. The lesion appeared as a painful, elevated nodule on the scalp for several months, without an appreciable increase in size. Microscopically, it showed typical histologic characteristics of nerve sheath myxoma, and tumor cells revealed strong, positive reaction for S-100 protein and negativity for epithelial membrane antigen (EMA) on immunohistochemical staining. These immunohistochemical findings of this case support the view that the origin cells of this tumor may be schwann cells rather than perineurial cells. The histogenesis and differential diagnosis of this tumor are discussed.
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6/20. Mobilization of the posterior leaflet of the mitral valve for resection of a left ventricular tumor producing carbohydrate antigen 19-9.

    We report an extremely rare case of a left ventricular tumor producing carbohydrate antigen 19-9 (CA19-9). A 43-year-old man with an episode of right hemiplegia underwent an emergent operation for a left ventricular tumor just beneath the posterior leaflet of the mitral valve. The tumor was successfully resected by mobilization of the posterior leaflet of the mitral valve. The resected specimen was immunoreactive for CA19-9.
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7/20. Primary corneal myxoma.

    PURPOSE: To present the clinical and histopathological characteristics of a corneal myxoma. methods: A 36-year-old, white woman presented with blurred vision and a whitish opacity on the left cornea. Clinical examination revealed a whitish, non-tender, 5 x 9 mm superficial tumour on the cornea. The tumour was excised and examined microscopically. RESULTS: The tumour was composed of spindle-shaped cells in a myxomatous ground substance. Immunohistochemically, the tumour cells were positive for vimentin, muscle-specific antigen and smooth muscle antigen. The tumour was classified as a myxoma of the cornea. The origin of the tumour is discussed. CONCLUSION: This is the second reported case of a corneal myxoma without prior corneal disease.
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8/20. thymoma arising within cardiac myxoma.

    Hematopoietic, glandular, and mesenchymal elements can be found within cardiac myxomas; ectopic endocrine tissues and "thymic rests" have also rarely been described. Atrial tumors (one right and one left) from 2 patients (a 69-year-old man and a 77-year-old woman) were encountered among the atrial myxoma cases in one of the author's consultation files. Both tumors were comprised of classic cardiac myxoma (with characteristic rings and syncytial chains of myxoma cells in a loose myxoid matrix) and cellular thymoma-like elements (characterized by a lobulated sheet-like growth of epithelioid spindle cells admixed with small lymphocytes punctuated by vessels with prominent perivascular spaces). Neither patient had evidence of thymoma elsewhere. Immunophenotypically, the thymoma-like component reacted strongly with antibodies to keratins (AE1/AE3, Cam 5.2, wide spectrum, CK19, CK7) and CD57 and weakly with antibodies to CD31, CD34, and calretinin. This intermediate phenotypic expression of both epithelial and vascular antigens likely reflects the multipotential nature of the cells comprising this lesion. The most likely explanation for this extremely unusual finding is neoplastic transformation of thymic rests within a myxoma.
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9/20. Congenital dermatofibrosarcoma protuberans with fibrosarcomatous and myxoid change.

    This report describes a case of congenital dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous (FS) and myxoid areas. Immunohistochemical results showed that tumour cells in ordinary DFSP areas were diffusely positive for CD34, whereas in the FS and myxoid areas, few tumour cells were positive for this antigen. Ki-67 positive tumour cell numbers were greater in the FS (11.8%) and myxoid areas (19.8%) relative to ordinary DFSP areas (2.2%). reverse transcription polymerase chain reaction and sequence analysis showed the presence of an identical COL1A1-PDGFB fusion transcript in ordinary DFSP (plaque-like area), FS, and myxoid areas of DFSP. These results indicate that the three components of DFSP have a common histogenesis. This study documents the first application of gene analysis involving the myxoid area of DFSP.
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10/20. Angiomyxofibromatous tumor of the falx cerebri.

    We report a distinctive angiomyxofibromatous lesion arising from the falx cerebri of a 48-year-old woman. The tumor was composed of bland-appearing, spindle, and stellate cells in a myxoid matrix with prominent vascularity. The tumor cells were immunopositive diffusely for vimentin and focally for S-100 protein, but were immunonegative for epithelial membrane antigen, CD34, MIC2, Bcl-2, glial fibrillary acidic protein, cytokeratin CAM 5.2, desmin, and smooth muscle actin. This lesion could not be categorized according to the current world health organization classification of tumors of the nervous system, thus underscoring a need to enhance our understanding of myxoid mesenchymal neoplasms and reassess their nosology.
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