1/14. Fibromatosis.Fibromatosis represents a diverse group of fibroproliferative tumours. Their behaviour and pathological qualities are situated in an intermediate position between benign and malignant disease. The following represents the pathological and radiological presentation of a 29-year-old female with fibromatosis.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
2/14. 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 = behaviour (Clic here for more details about this article) |
3/14. Peripheral myxoma of maxilla. A case report.The intra-oral soft tissue myxoma or peripheral myxoma is a rare, slowly growing, benign mesenchymal tumor. Pathologically, it may be difficult to differentiate from other tumors with myoxid stroma and is occasionally misinterpreted as malignant. Though its counterpart, central odontogenic myxoma shows aggressive local behaviour and high recurrence rate following conservative excision, no much detail is available regarding peripheral myxoma of the oral cavity in the published English literature. In this paper, a rare case of peripheral myxoma of maxilla in a 35 years old female is presented with emphasis on review of relevant literature, histological aspects in differential diagnosis and 'semi-radical approach' in its management.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
4/14. Desmoid tumours treated with triphenylethylenes.Desmoids are uncommon mesenchymal tumours that occur at single or multiple anatomical sites, occasionally in association with polyposis coli. This paper describes the use of the triphenylethylene tamoxifen, and a new chlorinated analogue, toremifene, in 20 patients with progressive desmoid disease. Clinical responses ranging from stabilisation of disease to complete resolution were observed in 65% of cases. The antitumour activity of this group of drugs has been attributed to their anti-oestrogenic behaviour. However, desmoids provide a clinical model of a purely mesenchymal tumour which appears to respond despite having generally low levels of hormone receptor. This emphasises the significance of stroma within breast (and other) tumours, in particular how the stroma may regulate the response to these drugs regardless of receptor status.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
5/14. Retroperitoneal solitary fibrous tumour arising from the pelvis in women--a case report and review of literature.A solitary fibrous tumour is an unusual spindle cell neoplasm that most frequently occurs in the pleura based intrathoracic region. In recent years attention has been drawn towards solitary fibrous tumours arising in extrathoracic sites. They are usually benign but malignant solitary fibrous tumours have also been reported (Nielson et al. 1997). There is far less information about the clinical behaviour of an extra thoracic solitary fibrous tumour unlike intrathoracic tumours which is well reported in many case series (england et al. 1989). Although solitary fibrous tumours are well described lesions, the occurrence of similar tumours in the pelvic retroperitoneum of women and presenting as pelvic mass have been reported only sporadically. Because of the rarity, unpredictable behaviour; lack of information available about the clinical behaviour (recurrence and metastasis) and lack of follow up protocol, we are reporting this case which we encountered along with the review of previously reported cases.- - - - - - - - - - ranking = 3keywords = behaviour (Clic here for more details about this article) |
6/14. A rare case of solitary fibrous tumour of the pre-sacral space: morphological and immunohistochemical features.A 28-year-old woman presented with abdominal pain. Ultrasonograhic examination showed a pre-sacral mass, with complex structure and well delimitated cystss with thick walls. The resected specimen was 7.5 x 6 x 4 cm in size, well circumscribed and yellow in colour, with cysstic change containing mucoid-like material. Histologically, the lesion was composed of spindle cells with high cellularity and rich vascularization with a haemangiopericytoma-like pattern. The diagnosis of solitary fibrous tumour (SFT) was made. The differential diagnosis for SFT of the pre-sacral spaace involves haemangiopericytoma, GIST, malignant mesothelioma, synovial sarcoma, leiomyomatous tumours and granulosa cell tumour. Immunohistochemical studies revealed reactivity for CD34, CD99 and Bcl-2, but no staining for desmin, inhibin, c-kit, EMA, CK, SMA, S-100 and CD31, confirming a diagnosis of SFT. Although SFT is usually associated with a favourable prognosis, close follow-up is recommended because of the limited information on its long-term behaviour.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
7/14. Intracranial gliofibroma mimicking a meningioma: a case report and review of literature.Gliofibromas are rare glio-mesenchymal tumors composed of astrocytic and benign mesenchymal components, which commonly occur in the first two decades of life. They are not listed as a distinct entity in the current WHO classification of CNS tumors. Their biological behaviour is unknown, and histogenesis is debatable. We describe a case of histopathologically proven gliofibroma in the region of the left quadrigeminal plate in a 15-year-old child that mimicked a tentorial meningioma both at imaging and surgery. Post-operatively, the residual tumor regrew to a size similar to the initial mass (4 cm x 3.6 cm x 3.5 cm), within one and a half years after the first surgery. The histopathology of the tumor was similar on both occasions. Although a poor prognosis has been characteristically noted in gliofibromas with high-grade glial component, but the present case had recurrence despite being of low-grade, thus highlighting the uncertain behaviour of this rare tumor.- - - - - - - - - - ranking = 2keywords = behaviour (Clic here for more details about this article) |
8/14. Fibromyxoma of bone: a case report and review of the literature.In this study, a case of fibromyxoma of the proximal femur in a 59-year old woman is reported. The classification of this rare bone tumour is still a matter of debate and some investigators have suggested that these lesions represent a degenerative form of fibrous dysplasia. Some authors make a further distinction between fibromyxoma and myxoma of bone. In a review of 23 cases of fibromyxoma and five cases of myxoma, no differences in clinical, radiographic and biologic behaviour between fibromyxoma and myxoma were found. Apart from the age at diagnosis, the most important difference between fibromyxoma and myxoma was the degree of myxoid matrix. Therefore, we suggest that extragnathic myxoma is a regressive variant of extragnathic fibromyxoma and should be termed as the same entity. In contrast to monostotic fibrous dysplasia fibromyxoma / myxoma often causes pain and presents as a Lodwick IC lesion with a soft tissue mass. Therefore, fibromyxoma / myxoma should be distinguished from fibrous dysplasia because of its different clinical and radiographic features.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
9/14. Central odontogenic fibroma interesting the maxillary sinus. A case report and literature survey.Odontogenic fibroma (OF) is a rare benign odontogenic tumor deriving from the dental mesenchymal tissue and accounting for less than 5% of all odontogenic tumors. This paper presents an aggressive histologically diagnosed central odontogenic fibroma (COF) in a 17-year-old girl characterized by asymptomatic rapid growth with massive replenishment of the left maxillary sinus. We carried out a review of the literature to retrieve all published cases of COF especially focused on radiographic aspects and surgical treatment of cases characterized by clinical aggressive behaviour, as we observed in our patient. Search strategy included retrieval of English language papers, published from 1966 to today, in dental journals on medline/pubmed and EMBASE, and hand-searching of the bibliography of retrieved papers. Sixty-nine cases of COF were identified from 1954 to 2003 and a new one was added. We have compared characteristics of COFs according to age, gender, location, clinical and radiographic findings of aggressive development, and histology. We discuss clinical and radiographic aspects of our case compared with COFs previously published. We give suggestions for surgical treatment of COF in case of aggression to important anatomical structures.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
10/14. Mesenteric fibromatosis in familial polyposis. A case report and a review of Japanese cases.A 30-year-old female with mesenteric fibromatosis (MF) associated with familial colonic polyposis (FCP) was reported together with a review of Japanese cases of simple MF. Intestinal surgery can contribute to the pathogenesis of MF more intensely in cases associated with FCP than in simple MF cases. Furthermore, the biological behaviour of MF seems to be more aggressive in the former than in the latter.- - - - - - - - - - ranking = 1keywords = behaviour (Clic here for more details about this article) |
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