Cases reported "Giant Cell Tumors"

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1/14. Giant cell angiofibroma of the orbit and eyelid.

    PURPOSE: To report the clinicopathologic features of a newly recognized tumor, giant cell angiofibroma. DESIGN: Observational case series. MAIN OUTCOME MEASURES: Clinical and histopathologic features of giant cell angiofibroma. methods: light and electron microscopy and immunohistochemistry of five cases of giant cell angiofibroma. RESULTS: A total of five patients (4 women and 1 man) are described: two presented with a painless mass in the eyelid, two with a mass in the orbit, and one presented with a conjunctival lesion. All lesions were well demarcated with no capsule and were composed of blood vessels, a patternless spindle-shaped cell proliferation with a solid and pseudovascular appearance, and multinucleated giant cells. Both spindle-shaped and giant tumor cells were intensely positive for CD34 and vimentin. CONCLUSION: Giant cell angiofibroma resembles solitary fibrous tumor and giant cell fibroblastoma and should be considered in the differential diagnosis of spindle-cell tumors in the eyelid, orbit, and conjunctiva.
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2/14. Giant cell angiofibroma of the inguinal region.

    Giant cell angiofibroma is a rare mesenchymal neoplasm most commonly arising in the soft tissues near the orbit. Recently, several cases of extraorbital giant cell angiofibroma have been reported. We report the light microscopic and immunohistochemical features of an additional case of extraorbital giant cell angiofibroma arising in the inguinal region that was clinically mistaken for an inguinal hernia. The patient was a 50-year-old woman who presented with a mobile, nonreducible, left inguinal mass. The tumor was 10.8 cm in greatest diameter, was well circumscribed, and appeared to be encapsulated. Histologically, the tumor was composed of a mixture of cytologically bland spindle-shaped cells and ovoid cells of varying cellularity with deposition in a variably collagenous and myxoid stroma. The tumor had prominent, various-sized blood vessels, often with perivascular hyalinization. In addition, scattered pseudovascular spaces filled with an amorphous eosinophilic material were present and lined by spindle-shaped and ovoid cells similar to those found throughout the neoplasm. Rare multinucleated floret-like giant cells were seen. Immunohistochemically, the tumor cells stained strongly and diffusely for both CD34 and bcl-2 while immunostains for S-100 protein, desmin, smooth muscle actin, and muscle-specific actin were negative. There is no evidence of local recurrence or metastasis 3 months following excision of the mass. This report emphasizes the recognition of this unusual tumor in extraorbital sites. We discuss the overlapping histologic and immunophenotypic features with giant cell fibroblastoma and solitary fibrous tumor and raise the possibility that these tumors could represent a histologic spectrum of CD34-positive dendritic interstitial cell neoplasms.
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3/14. 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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4/14. Giant cell angiofibroma of the nasolacrimal duct.

    PURPOSE: To describe clinical and histologic features of the first case, to our knowledge, of giant cell angiofibroma located in the nasolacrimal duct region in a 28-year-old woman. methods: Interventional case report. A left nasolacrimal duct tumor was excised en bloc by lateral rhinotomy. Histopathologic examination was performed with the use of light microscopy. Immunohistochemical staining included S-100 protein, muscle-specific actin, desmin, myoglobin, vimentin, and CD34. RESULTS: The lesion was characterized by haphazardly arranged oval to spindled cells, a myxoid and collagenous stroma, multinucleated giant cells, prominent blood vessels, and pseudovascular spaces. Tumor cells were strongly positive for vimentin and CD34 and were negative for other antigens. After excision, there has been no recurrence over 4 years of follow-up. CONCLUSIONS: Originally described as an orbital tumor, giant cell angiofibroma also may occur in the nasolacrimal duct and lacrimal sac region. This mesenchymal neoplasm should be included in the differential diagnosis of lacrimal drainage system tumors.
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5/14. Extraorbital giant cell angiofibroma.

    We describe a case of giant cell angiofibroma (GCA) in extraorbital location. A 47-year-old man developed a subcutaneous tumor on the forearm, which measured 6 cm in diameter and was well demarcated. Histologically, small-sized vessels similar to arterioles, sometimes with a hyalinized wall, were regularly distributed throughout the tumor tissue. Lesional cells were oval and spindle in shape with pale eosinophilic cytoplasm and plump nuclei with clumped chromatin. In addition, larger bizarre cells with lobulated hyperchromatic nucleus were randomly admixed. Areas with a sieve-like pattern contained small slit-like spaces or larger pseudocystic spaces lined by tumor cells with an admixture of bizarre cells. Both mononuclear and bizarre lesional cells expressed strongly and diffusely vimentin and CD34. Negative reactions were with cytokeratins, bcl-2, desmin, actin, VWF, S100, alpha-1-antitrypsin, CD99, lysozyme and FXIIIa. Our finding confirms that GCA is a benign neoplasm not necessarily confined to orbital location. The patient did well without recurrence after 13 months of follow-up.
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6/14. Osteoclast-like giant cell tumor in mucinous cystadenocarcinoma of the pancreas: an immunohistochemical and molecular analysis.

    Osteoclast-like giant cell tumors (OLGT) are rare neoplasms of the pancreas and mostly associated with ductal adenocarcinomas. In this report, we present the rare case of OLGT associated with mucinous cystadenocarcinoma (MCC). We investigated the expression profile of both tumors by methods of molecular biology and immunohistochemistry. The panel of markers included osteopontin, her2/neu, mismatch repair genes, K-ras, p53, E-cadherin, VEGF-C, and podoplanin. osteopontin was expressed by the osteoclast-like giant cells but not by the mononuclear tumor cells of the OLGT. We detected an amplification and overexpression of her2/neu in the MCC but not in the OLGT. Although we observed an immunohistochemical expression of hMSH2 and hMLH1 in the OLGT, we were not able to confirm this result by western blot analysis. We also did not find any microsatellite instability (D2S123, BAT26). While mutation of K-ras codon 12 was found in both tumor components, there was wild-type dna of p53. E-cadherin was expressed in MCC but not in OLGT. VEGF-C was only positive in osteoclast-like giant cells and some of the mononuclear cells of OLGT. The vessel-rich stroma of OLGT did not present any podoplanin-positive lymphatic vessel. The observation of our case and others in the published literature may indicate separating OLGT with undifferentiated carcinoma from OLGT with MCC for the better clinical outcome of the latter.
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7/14. Application of the free vascularized bone graft in the treatment of malignant or aggressive bone tumors.

    Although recent advances in clinical microsurgery have made free tissue transfer a clinical reality, the value of free bone transfers in the reconstructive tumor surgery of long bones has not been reported to our knowledge. Two patients with malignant or aggressive bone tumors who underwent en bloc resection of their tumors and reconstruction with free vascularized bone grafts are presented. In the first case, a giant cell tumor of the distal radius was resected, and a 10 cm free vascularized fibular graft was performed as a reconstructive procedure; in the second case a chondrosarcoma of the femur was excised and replaced by a 20 cm segment of free vascularized fibula. Postoperatively, these patients were followed with arteriograms and sequential bone scans. The indications for the procedure and the operative technique are discussed. A comparison of the merits of free rib versus free fibular transfers is presented with emphasis on bony architecture, vessel characteristics, dissection required and the options available for reconstructive surgery. The advantages and disadvantages of free vascularized bone grafts with respect to conventional methods and the potential applicability of this technique are discussed.
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8/14. Surgical treatment of giant cell tumors of the pelvis.

    There are many problems concerning management of giant cell tumors in the pelvis: diagnosis, surgical approach, adequacy of treatment, reconstruction, and prognosis. Of 58 cases of giant cell tumor of bone from 1962 to 1985, five cases arose in the pelvis, two in the ilium, two in the ischium, and one in the pubis. Surgical procedures included en bloc excision in four cases and intralesional excision in one. Reconstruction included sacroiliac fusion in one, iliofemoral fusion in one, and pelvic prosthetic replacement in another. recurrence and metastasis did not occur in any of the cases. All patients were able to walk without crutches. A good surgical approach isolates major vessels and nerves, and permits excision to prevent recurrence and control bleeding. Reconstructive surgery may be required for treatment of a pelvic giant cell tumor.
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9/14. Free vascularized bone grafts in surgery of the upper extremity.

    Free vascularized fibular grafts were employed in five patients with segmental bone defects following trauma or resection of tumors of the upper extremity with excellent results in three patients and satisfactory results in two. No donor site morbidity was experienced. A comparison with rib and iliac crest grafts indicates that the fibula is more suitable for reconstruction of long bone defects. The advantages of this technique are stability without sacrificing viability and a shorter immobilization period with more rapid incorporation and hypertrophy of the graft. The disadvantages are prolonged operating time, difficulty in assessing patency of anastamoses in the immediate postoperative period, and sacrifice of a major vessel in the lower extremity.
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10/14. Facilitation of removal of a hypervascularized cervical vertebral body tumour by intraoperative temporary occlusion of the tumour-feeding vertebral artery, using intraarterial catheterization.

    The surgical treatment of a highly vascularized tumour that destroyed the fourth cervical vertebral body in a young patient, without neurological deficits, is reported. After posterior stabilization an anterior approach was used in order to replace the affected vertebral body with an acrylic prosthesis. The tumour-feeding vessels originated largely from the right vertebral artery. In order to maintain optimal visibility, intraoperative haemorrhage was kept at a minimum by temporarily occluding the tumour-feeding vertebral artery with an intraarterial catheter.
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