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1/20. Hybrid carcinoma of the salivary gland: salivary duct adenocarcinoma adenoid cystic carcinoma.

    AIMS: Hybrid tumours of the salivary gland are rare neoplasms that have been described only in the parotid and palate. Their recognition is important particularly when the component tumours have different biological behaviours. The occurrence of a submandibular hybrid tumour has not been reported. methods AND RESULTS: We describe a case of a 36-year-old woman with a hybrid carcinoma composed of salivary duct adenocarcinoma and adenoid cystic carcinoma of the right submandibular gland. There was no evidence of a pre-existing or concurrent pleomorphic adenoma. The presence of the two components was verified by differential immunohistochemical staining using a panel of cytokeratin, vimentin, smooth muscle actin and S100. The patient subsequently developed metastases to the pelvis, lumbar, vertebra and wrist. The clinical course in this patient was consistent with the behaviour of the salivary duct adenocarcinoma component. CONCLUSIONS: The histogenesis of hybrid tumours is largely unknown, but in this case it may represent diverging differentiation of luminal tumour cells. Because some histological features of different salivary gland tumours overlap, immunohistochemistry is a valuable tool especially when used to delineate the components of a hybrid tumour.
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2/20. Mixed apocrine/endocrine ductal carcinoma in situ of the breast coexistent with lobular carcinoma in situ.

    An unusual mixed form of ductal carcinoma in situ (DCIS) of the breast is described, which exhibits a biphenotypic morphology encompassing a range of differential diagnostic DCIS subtypes. In addition, immunophenotypic and ultrastructural studies demonstrate neuroendocrine and apocrine differentiation, raising questions regarding appropriate classification and biological behaviour. In two cases, coexistence of this mixed form of DCIS with lobular carcinoma in situ (LCIS) in the same duct lobular units is an additional unusual feature that might, at least in some cases, indicate a closer relation between them.
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3/20. 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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4/20. CD30-positive cutaneous T-cell lymphoma with concurrent solid tumour.

    Extranodal CD30 T-cell lymphomas seldom carry classical t(2;5) translocation and are usually anaplastic large cell lymphoma kinase protein negative. They cover a wide spectrum of histological and clinical behaviour. The prognosis of CD30 cutaneous T-cell lymphoma (CTCL) is good in the absence of nodal primary or disseminated disease. These lesions can undergo spontaneous regression, and overlap with the group of lesions of lymphomatoid papulosis. Although an increased incidence of solid tumours has been reported in patients with CD30 non-Hodgkin lymphoma of the skin, reports of concurrent malignancies are rare in CD30 CTCL. We report two patients with CD30 CTCL who, respectively, had concurrent disseminated gastric carcinoma and bilateral ovarian teratoma. Despite an aggressive clinical and histological appearance, both cases ran favourable clinical courses. The CTCL responded completely to chemotherapy in one patient, who eventually succumbed to gastric cancer. In the other patient, lesions regressed spontaneously after bilateral oophorectomy. A possible relationship between the lymphoma and the solid tumours is discussed.
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5/20. Hybrid tumours of the salivary glands. A report of two cases involving the palate and a review of the literature.

    Hybrid tumours are very rare salivary gland lesions composed of two or more different tumoural entities in a single neoplasm that arise within a definite topographical region. In most cases adenoid cystic carcinoma has been the predominant component in these lesions. In this study we describe two patients with hybrid tumours located in the palate, one in a 49-year-old woman and one in a 71-year-old man. The first case involved adenoid cystic carcinoma and mucoepidermoid carcinoma, and the patient in the second case exhibited adenoid cystic carcinoma and epithelial-myoepithelial carcinoma. Both patients were treated with surgery and radiotherapy, and there has been no evidence of recurrence after 13 and 36 months of follow-up, respectively. The recognition of the histologic component with the higher grade of malignancy in every case of hybrid tumour of the salivary glands is a necessary step to determine the biological behaviour and, consequently, to determine the proper therapeutic approach.
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6/20. Intraductal papilloma and papillomatosis.

    The benign papillary neoplasms of the breast are still controversial topics in general surgery. There are two forms of the benign papillary lesions, solitary intraductal papilloma and multiple intraductal papilloma. These disorders are very rare and their clinical behaviour is different in each case. In this study, two cases of the multiple intraductal papilloma are presented and clinical findings and surgical therapy are discussed.
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7/20. Obstructive jaundice secondary to neuroendocrine tumour in a patient with von Recklinghausen's disease.

    Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder, with variable clinical manifestations and unpredictable course, associated with an increased incidence of various tumours. Plexiform neurofibromas are hallmark lesions of NF1; they are slow-growing tumours, which account for substantial morbidity, including disfigurement and functional impairment, and may even be life-threatening. Neuroendocrine tumours (NETs), a rare diverse group of neoplasms, are occasionally associated with neurofibromatosis. Pancreatic NETs are tumours with an incidence of less than 1/100 000 population/year and complex patterns of behaviour, which often need complicated strategies for optimal management. We present the case of a young adult with NF1, having a unique concurrence of plexiform neurofibroma involving the liver with an ampullary NET, and we discuss step by step the management in a specialist centre.
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8/20. (18)FDG-PET-scan in staging of primary malignant melanoma of the oesophagus: a case report.

    Primary malignant melanoma of the oesophagus is a rare disease, only 262 cases being reported up to June 2005. In general, the prognosis is dismal because of its tendency to present as an advanced neoplasm with aggressive biological behaviour. (18)FDG-PET-scan is a useful tool for evaluation of metastatic disease and locoregional lymph node metastasis. We present herein a case of a young adult with primary malignant melanoma of the oesophagus, followed by a literature review on the subject.
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9/20. Recurrent oral carcinoma in Ferguson-Smith disease.

    A patient with multiple self-healing squamous cell carcinomas of the skin is presented. mucous membrane involvement in this condition is very uncommon and the lesions may show aggressive behaviour.
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10/20. 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.
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