Cases reported "Fibroma"

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1/92. Bilateral elastofibroma dorsi. Two case reports.

    Elastofibroma dorsi is a benign fibroproliferative lesion of unknown pathogenesis. It is most commonly found in the periscapular region in elderly women. The majority of patients are asymptomatic. The lesions have a characteristic location and a specific magnetic resonance imaging (MRI) appearance allowing accurate prospective diagnosis. The importance of recognizing the benign nature of this lesion to avoid an unnecessary operation is apparent. We describe two cases of bilateral elastofibroma dorsi with characteristic findings on MRI and typical histological appearances.
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2/92. Collagenous fibroma (desmoplastic fibroblastoma): genetic link with fibroma of tendon sheath?

    We observed clonal chromosome abnormalities in two fibrous soft tissue tumors diagnosed as collagenous fibroma (desmoplastic fibroblastoma). The involvement of the same band of the long arm of chromosome 11, 11q12, was observed in both tumors. The presence of hitherto unreported similar chromosomal abnormalities in this tumor supports the neoplastic nature of this lesion. In addition, a possible relationship with fibroma of tendon sheath, which also shows rearrangement of 11q12, is suggested. 11q12 might be a common genetic denominator of benign fibroblastic lesions, such as collagenous fibroma and fibroma of tendon sheath.
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3/92. Acrochordons are not a component of the birt-hogg-dube syndrome: does this syndrome exist? case reports and review of the literature.

    Multiple fibrofolliculomas, trichodiscomas, and acrochordons compose the triad of cutaneous lesions characterizing the birt-hogg-dube syndrome, inherited in an autosomal dominant fashion. We report a case of a family who had the triad of tumors of the birt-hogg-dube syndrome. Two members were observed clinically and histologically. Biopsies of the facial papules disclosed features of the fibrofolliculoma/trichodiscoma spectrum. Lesions that were clinically acrochordon-like proved to correspond to the same histopathologic spectrum. The characterization of the nature of the acrochordon-like lesions in our patients and the review of the literature allow us to question if acrochordons (skin tags) should be maintained as a component of this association. In light of our conclusion that fibrofolliculoma, trichodiscoma, and the acrochordon-like lesions are histologic variations of a single lesion, we further question whether the term "syndrome" is valid.
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4/92. A cardiac papillary fibroelastoma with chordal location.

    Papillary fibroelastomas, which were initially incidental findings at autopsy and surgery, are now being recognized with increasing frequency with the widespread use of echocardiography. Because of their embolic potential, an aggressive treatment approach is generally accepted. We report a papillary fibroelastoma located at the chorda of the anterior mitral leaflet that underwent conservative follow-up for 8 years without any complications. Because most of the cardiac papillary fibroelastoma cases reported are incidental findings, the question of whether symptomless fibroelastomas must be removed or other factors contribute to the embolic nature of the selected cases remains a challenge for the future.
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5/92. Histopathologic diagnosis of superficial soft tissue tumors, related lesions, and simulators: an algorithmic approach based on colors: tumors with predominance of pink.

    An algorithmic approach based on colors for histopathologic diagnosis of soft tissue tumors and their simulators is proposed. At scanning magnification, in specimens stained with hematoxylin and eosin, mesenchymal tumors can be classified according to their color. The color of a tumor is basically determined by density, morphology, and distribution of neoplastic cells, and by density and quantity of the stroma. The basic colors that can be observed by neoplasms stained with hematoxylin and eosin are white, pink, red, and blue. Colors may be used as a first step in choosing the algorithm for specific diagnosis of a given mesenchymal neoplasm. Furthermore, colors may be helpful in understanding the histogenesis of a tumor, and this is especially important in soft tissue pathology, because criteria for benignancy and malignancy vary according to the nature of the neoplasm. In this article, tumors with a predominance of pink are analyzed. Pink tumors are composed of cells with abundant eosinophilic cytoplasm and sparse chromatin in their nuclei, such as leiomyoma. In other cases, the pink color in a tumor is determined by abundant fibrous stroma rather than by characteristics of neoplastic cells, such as sclerotic fibroma.
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6/92. Elastofibroma dorsi: elaboration of cytologic features and review of its pathogenesis.

    Elastofibroma is a slow-growing soft tissue lesion characteristically found between the inferior scapula and chest wall. Because it behaves clinically in a benign manner, fine-needle aspiration (FNA) represents the simplest and quickest method of obtaining a definitive diagnosis, thus obviating more invasive means of obtaining a tissue diagnosis. However, due to the nature of this lesion a correct diagnosis can inadvertently be missed. Herein we describe the findings of a recent FNA that obtained abundant diagnostic material and elaborate upon the spectrum of cytologic features of the elastic fibers that can be identified. These features should be recognized, since aspiration biopsy in elastofibromas can lead to hypocellular smears. In addition, we discuss recent developments in the pathophysiology of elastic fibers and their application toward understanding the generation of an elastofibroma.
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7/92. Giant-cell fibroblastoma: a case report emphasising the presence of hyperplastic subplasmalemmal linear densities in continuity with granular matrices in the extracellular space.

    The histological, immunohistochemical and ultrastructural features of a case of giant-cell fibroblastoma from the soft tissues of the chest wall in a 48-year-old female are described with special reference to the cell surface and matrix. Subplasmalemmal linear densities (SLDs) characterised cell surfaces, and exhibited excessive development of the dense external component: foci of identical dense material were present in the matrix. The nature of these dense foci, both the external component of the SLD and those free in the extracellular space, was investigated by light microscope immunostaining for fibronectin, laminin and collagen IV. All three proteins stained vessels. There was weaker but positive staining for tumour cell surfaces and matrix, consistent with the widely dispersed nature of the dense foci. Given their fine structural appearance, these dense foci can be referred to as granular matrices. Given also that the matrix protein immunostaining pattern is consistent with the distribution of these granular matrices as observed by electron microscopy, they may be provisionally interpreted as a kind of basement-membrane-related granular matrix. The presence of these proteins emphasises the point that, while giant-cell fibroblastoma fibroblasts lack a lamina, they nevertheless bear basement-membrane-related proteins organised, however, in a non-laminate fashion. The observations reinforce the need to qualify immunostaining results by ultrastructural investigation in order to understand the organisation of immuno-detected proteins and are discussed in terms of their diagnostic and possible biological significance.
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8/92. Pedunculated malignant melanoma.

    A woman had a primary pedunculated malignant melanoma. This is a rare form of presentation and may result in clinical confusion with seborrheic keratosis, fibroepithelial papilloma, or granuloma pyogenicum. The aggressive nature of the lesion was indicated by the presence of erosion and bleeding. Though the tumor cells were present only in the pedunculated mass, it had metastasized to regional lymph nodes at the time of surgery.
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9/92. Conservative treatment of recurrent ovarian fibromas in a young patient affected by Gorlin syndrome.

    The case of recurrent bilateral ovarian fibromas occurring in a 22 year old Italian girl affected by Gorlin syndrome is reported. Ovarian fibromas occur in 75% of female patients with Gorlin syndrome and their recurrence has rarely been reported in the literature. Management is guided by the benign nature of the lesion and consists of surgical removal of the fibroma. Preservation of the normal ovarian tissue is recommended even though there is risk of recurrence of the fibroma.
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10/92. Cytogenetic and molecular characterization of a congenital mesoblastic nephroma.

    A newborn baby boy was diagnosed with the mixed form of congenital mesoblastic nephroma (CMN) representing both classic and cellular histology features in the renal tumor. Additionally, the patient had skin and bone lesions consistent with multifocal involvement of a generalized infantile fibromatosis (IFS). Both skin and bone lesions were distinctly different from CMN and did not represent metastasis. The primary tumor cell line (MCH-MN-1), established from the resected right kidney tumor, had a diploid dna content. Cytogenetic studies revealed deletion on the long arm of chromosome 3 (q21q24) and duplication on the short arm of chromosome 11 (p15). MCH-MN-1 cells expressed ETV6-NTRK3 gene fusion transcripts, characteristic of cellular and mixed forms of CMNs. The cells had high p21 and low Bax mRNA expression in the reverse transcriptase-polymerase chain reaction (RT-PCR) assay. The high level of proliferative marker (Ki67) mRNA expression correlated well with the pluripotent nature of MCH-MN-1 in tissue culture (cell doubling time = 12.4 h). Our results showed that MCH-MN-1 might be a good model cell line for investigations on mesoblastic nephroma.
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