1/88. Giant central ossifying fibroma of the mandible: report of case.A case is presented of a patient with a giant ossifying fibroma of the mandible that had been diagnosed earlier as fibrous dysplasia. Surgical resection of the tumor and involved bone was chosen as the mode of treatment because of the extent of the lesion and continued infection. Surgeons should be aware that ossifying fibroma is a very distinct possibility in large growing lesions in age groups older than is deemed compatible with fibrous dysplasia.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/88. Non-ossifying fibromas and giant cell reparative granulomas in a child with ocular-ectodermal syndrome.We report on a patient with ocular-ectodermal syndrome who was previously described in 1993 [Am J Med Genet (1993) 45:764-766]. This boy has now developed additional manifestations, including giant cell granulomas and non-ossifying fibromas. This adds to the list of phenotypic manifestations of this condition.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
3/88. An intrasylvian "fibroma" in a child with cystic fibrosis: case report.OBJECTIVE AND IMPORTANCE: Intracranial fibrous tumors are uncommon during childhood. An unusual case of benign intrasylvian "fibroma" that has remained clinically and radiographically stable more than 3 years after a subtotal resection is described. CLINICAL PRESENTATION: A 9-year-old girl with cystic fibrosis presented with new-onset focal seizures referable to a large calcified left sylvian fissure mass. INTERVENTION: An open biopsy with subtotal resection of the lesion revealed a benign process characterized by exuberant fibrocollagenous tissue intermeshed with chronic inflammatory cells and foreign body giant cells, encompassing islands of gliotic brain tissue. Immunohistochemical analysis showed staining for epithelial membrane antigen and reticulin within some of the spindle cells, although the majority were nonreactive. The majority of tumor cells exhibited staining for laminin; CD34 staining was absent. Ultrastructural studies were also suggestive of a fibroblastic rather than a meningothelial origin of the lesion, with elongated cells separated by abundant extracellular collagen. Although dense adherence of the mass to the pial surface and the middle cerebral artery vessels precluded a complete resection, the patient remains seizure-free without anticonvulsant therapy more than 3 years postoperatively with no evidence of growth of the lesion. CONCLUSION: The lesion in this patient bears morphological similarity to a rare group of tumors referred to as "intracerebral fibromas," although a variety of other rare mesenchymal neoplasms were also considered within the differential diagnosis. However, the absence of any definite neoplastic features, the finding of chronic inflammatory changes, and the lack of growth of the residual tumor during an extended follow-up interval indicate that the mass may represent either an extremely indolent neoplasm or a nonneoplastic process. The differential diagnosis of intracranial fibrous tumors is contrasted with that of the reported case.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
4/88. Huge malignant localized fibrous tumor of the pleura.Localized fibrous tumor is an unfrequent mesenchymal neoplasm. The malignant variant of the pleura is exceptional and differential diagnosis with the more frequent benign type or with other neoplasms such as soft tissue sarcoma and mesothelioma is rarely possible in a preoperative setting. The best treatment of this disease is radical surgical resection. No definitive data exist about the role of chemotherapy. We report a case of a giant right intrathoracic mass whose preoperative diagnosis, from an open biopsy, was consistent with sarcoma and, in a second review, with fibrous tumor of the pleura without any indication about malignancy. A right pleuropneumonectomy and pericardial resection was performed through a right hemiclam-shell approach. histology demonstrated an aggressive behaviour: high mitosis rate, Ki 67 of 34% and diffuse necrosis were present. In consideration of the apparent local radicality we did not perform any adjuvant treatment. Six months after the operation a wide local recurrence was evident and a systemic treatment with ifosfamide and Adriamicina is still in progress. So far a good response has been documented. Preoperative diagnosis of malignancy has an important role as a therapeutic strategy in management of fibrous tumours of the pleura. When there is suspicion of a malignant form neoadjuvant chemotherapy can represent a further tool to control poorly differentiated and large tumors, and a wide surgical resection of the lesion must be performed.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/88. Natural skin reduction and breast recovery using a tissue expander after enucleation of a giant breast tumour.We report a new use of the tissue expander for reshaping a breast after resection of a giant tumour. After resection of giant fibroadenomas, two patients had expanders inserted into the tissue defect and gradually reduced in size over five months. This facilitated healing and natural skin shrinkage and resulted in a natural shape and size.- - - - - - - - - - ranking = 6keywords = giant (Clic here for more details about this article) |
6/88. Giant-cell fibroblastoma and dermato fibro sarcoma protuberans: the same tumoral spectrum? Report of two cases of association in children.We describe two cases of giant-cell fibroblastoma (GCF) with dermato fibro sarcoma protuberans (DFSP) component, occurring in two children in a chest wall localization. One case recurred 1 year later. The two patients were tumor-free 12 and 8 years later. GCF is a rare mesenchymal cutaneous and subcutaneous tumor reported mostly in the first two decades of life. Dermato fibro sarcoma protuberans, occurring preferentially in adults, is a rare skin tumor with a pronounced tendency to local recurrence. Some cases of association of recurrence of GFC under the form of DFSP have been reported, raising the question of a continuum between the two tumors. The treatment of choice of the two tumors is a wide local excision.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
7/88. chondrosarcoma of the proximal femur with myxoid degeneration mistaken for chondromyxoid fibroma in a young adult. A case report.BACKGROUND: Fine needle aspiration cytology (FNAC) is effective in the diagnosis of bone tumors when combined with careful radiologic and clinical evaluation. However, cases where clinical or radiologic findings are atypical or unusual may lead to an erroneous diagnosis. CASE: A 19-year-old male presented with a pain in the left hip area that had been slowly progressive over a 10-month period. Clinical and radiologic findings suggested either giant cell tumor or chondroblastoma. The smeared aspiration specimen showed loosely cohesive, oval to round cells with moderate amounts of pale pink cytoplasm admixed with pinkish-blue, chondromyxoid material. The individual cells contained a single nucleus with evenly distributed, fine chromatin. A few osteoclastic giant cells were scattered in the smears. A cytologic diagnosis of myxoid lesion with a few giant cells, suspicious for chondromyxoid fibroma, was made. The diagnosis of chondrosarcoma was made by subsequent histologic examination. CONCLUSION: Absence of the usual clinicoradiologic features of chondrosarcoma combined with an unusual cytologic presentation in this case led to a misdiagnosis. In most centers, FNAC has achieved undisputed status as a diagnostic tool, and cytologic diagnosis often forms the basis of the therapeutic protocol. However, at some sites FNAC diagnosis is more problematic. awareness of the limitations and pitfalls of FNAC is just as important as knowledge of the scope of FNAC in bone tumors. Tumors with chondromyxoid features provide particular difficulties.- - - - - - - - - - ranking = 3keywords = giant (Clic here for more details about this article) |
8/88. 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.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
9/88. Calcifying aponeurotic fibroma features seen on fine-needle aspiration biopsy: case report and brief review of the literature.Calcifying aponeurotic fibroma is a rare benign soft tissue proliferation, which occurs in the distal extremities in children. We describe a case of calcifying aponeurotic fibroma of the hand in a 9 year-old male diagnosed by FNA. Clinically and radiographically this mass was felt to be suspicious for sarcoma, likely alveolar rhabdomyosarcoma. Cytologic examination revealed benign appearing spindled cells, chondroid cells, multinucleated giant cells and calcific debris. These features recapitulate the classic histologic features of calcifying aponeurotic fibroma. Conservative excision was performed and histologic exam confirmed the diagnosis. To the authors' knowledge, this is the first description of the cytologic features of this entity.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
10/88. Composite tumor consisting of dermatofibrosarcoma protuberans and giant cell fibroblastoma associated with intratumoral endometriosis. Report of a case.We present a unique case of composite skin tumor of the vulva consisting of dermatofibrosarcoma protuberans (DFSP) and giant cell fibroblastoma (GCF) with an intratumoral focus of endometriosis. A 31-year-old female with a 10-year-history of a recurring subcutaneous tumor in the vulvar area underwent excision of the seventh recurrence of the tumor. Microscopic examination revealed a composite fibrohistiocytic tumor consisting of DFSP and GCF. Additionally, a focus of endometriosis within the tumor tissue was found. Malignant transformation of extragonadal endometriosis has already been described; we present, however, the occurrence of a focus of endometriosis within the tissue of a hormonally independent soft tissue tumor. There is a possible link to the occurrence of cutaneous endometriosis at previous surgery sites and in the scars. The presence of endometriosis within the soft tissue tumor represents, to the best of our knowledge, a previously undescribed collision phenomenon.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
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