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1/69. Bellini duct (collecting duct) carcinoma of the kidney.

    carcinoma of the collecting ducts, or Bellini carcinoma, is a rare renal tumour and, unlike most renal cell carcinomas, it derives from distal tubules. It displays highly aggressive behaviour and has a poor prognosis. In this study, the authors present three cases which they observed over the past three years.
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2/69. High grade MALT-lymphoma of the breast.

    A 65-year-old woman presented with a rapidly growing breast tumor, initially diagnosed as a carcinoma. histology showed a breast lymphoma of high grade MALT-type. A lymphoma should always be considered in the differential diagnosis of a breast tumor, because it needs a different work-up and treatment. The subgroup of NHL of Mucosa-Associated-Lymphoid-Tissue origin has different clinical behaviour, as illustrated in this report.
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3/69. Chondromyxoid fibroma of skull base: a tumour prone to local recurrence.

    Chondromyxoid fibroma of the skull base is extremely uncommon. Sometimes involvement of the nasal cavity may occur and the patients may present with nasal symptoms. The biological behaviour of this tumour has not been well studied, primarily because of the limited number of reported cases and the short duration of follow-up. We report a histologically confirmed case of chondromyxoid fibroma of the skull base that recurred repeatedly over a 10-year period after the initial operation. Histologically it showed identical morphology to the original tumour with no evidence of histological progression or dedifferentiation. Ultrastructurally, the spindle tumour cells in the fibromyxoid area showed dual chondroblastic and fibroblastic differentiation, suggesting that these spindle fibroblastic cells and the better differentiated chondroid cells were of the same cell type with different histological morphology.
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4/69. Altered expression of p53 and its regulated proteins in phyllodes tumours of the breast.

    The histological characteristics of phyllodes tumours of the breast are often not related to their clinical outcome. Additional studies must therefore be performed to investigate the possible relationship of cell biological parameters to the biological behaviour of these tumours. The expression of Ki-67, p53, and its regulated proteins has been studied in 19 primary phyllodes tumours, from patients with known follow-up, using immunohistochemical and molecular biological techniques. Overexpression of the p53 protein was observed in four cases and mutation in two cases. In only one case, the sequence alteration, at codon 273, was associated with overexpression of p53 protein and with strong expression of Ki-67 (30 per cent). This alteration was found in the primary, the recurrent, and the metastatic tumour samples. Moreover, the same p53 gene mutation, Arg273Cys, was detected in all tumour samples. No mutation was found in adjacent normal breast tissue, indicating that this was an acquired mutation. Unexpectedly, strong BAX expression was observed in the primary tumour. The patient died during the follow-up period. It is concluded that p53 gene status and an accumulation of BAX, both involved in the same apoptosis-controlling pathway, may be of prognostic relevance in phyllodes tumours.
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5/69. Spinal melanotic schwannoma: a tumour with poor prognosis.

    AIM: To clarify the prognosis of melanotic schwannoma. This is a rare tumour which is generally considered as a benign lesion, reported in many cases with a short follow-up only. methods AND RESULTS: Five cases of spinal melanotic schwannoma were retrospectively studied. The tumours were examined using standard histological, immunohistochemical and ultrastructural methods. No features of malignancy (high mitotic count, atypia or necrosis) were found in the primary tumours. The follow-up period ranged from 3 to 7 years. Malignant clinical behaviour was clear-cut in four cases: three patients died from metastases to various sites and one presented several discrete spinal tumours of the same type seven years after the first operation. Only one patient presented no recurrence and was free of disease 6 years after initial diagnosis. The review of 57 cases of the literature (including our cases), showed that 15% of the cases had recurrences and 26.3% were complicated by metastasis. Only 53% of the cases followed for more than 5 years, were free of disease vs. 67.5% of the cases with shorter follow-up. Twenty additional cases had no follow-up. CONCLUSION: Appropriate long-term follow-up is required for all melanotic schwannomas, as it may recur or metastasize after more than 5 years, even in the absence of overt malignant histological features.
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6/69. Warthin-like tumor of the thyroid a case report.

    A case of Warthin-like tumor of the thyroid (WaLTT) with cervical lymph node metastasis is presented. The problems of the FNA diagnosis of this type of tumor is discussed as well as the histogenesis, nature and behaviour of this peculiar tumor.
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7/69. ameloblastoma of the jaws.

    ameloblastoma is a histologically benign tumor derived from odontogenic apparatus. The tumor can infiltrate into surrounding tissues. Although it is benign, it presents symptoms of a malignant tumor, such as infiltration into the lungs, pleura, regional and distant metastases, orbit, base of skull, brain and has resulted in death. It also has a high incidence of recurrences, the existence of regional or distant metastasis, showing a microscopic pattern of ameloblastic carcinoma with cytologic features of an increasing nuclear/cytoplastic ratio, nuclear hyperchromatism, and the presence of mitosis. We report a study of 12 patients of ameloblastoma of the jaws between January 1992 and December 1996 consisting of 8 affected in the mandible and 4 in the maxilla. One patient with a tumor in the maxilla was excluded from this study, due to a different histological and clinical behaviour of the ameloblastoma.
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8/69. 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.
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9/69. Rapid growth in a cavernoma.

    The availability of magnetic resonance imaging has greatly increased the detection of cavernous malformations of the central nervous system in both symptomatic and asymptomatic patients. These lesions may be responsible for previously unexplained neurological events or may even have been incorrectly diagnosed. We describe a patient presenting with focal neurological signs in whom an initial diagnosis of malignant glioma had been made. Following excision, the pathological diagnosis was cavernoma. This vascular lesion has continued to show rapid growth and aggressive behaviour despite multiple surgical resections. The indications for operative and non-operative intervention will be discussed.
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10/69. Recurrent cardiac myxoma: why it occurs. A case report with literature review.

    Two years after surgical removal of a right atrial myxoma, recurrences were found in both atria: multiple tumors in the right atrium and one tumor in the left atrium. All were subsequently removed. There was no further recurrence during the following 3 years. We report on an unusual case which, to our knowledge, is the first time that such a case appears in the literature. We also discuss the clinical symptoms, diagnostic aspects, as well as details of a literature review regarding recurring myxoma. We think the recurrence in our case was due to totipotent multicentricity of the tumor. Previous studies showed that the recurrence might be due to the following reasons: inadequate resection, or multifocal pattern behaviour of a benign myxoma, either in the same or in a different location as the primary tumor. Familial disposition may also play a role in recurrent development. The abnormal dna ploidy pattern of myxoma patients showed a high recurrence.
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