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1/342. A case report including EM and dna repair investigations in a dermatosis associated with multiple skin cancers: epidermodysplasia verruciformis.

    This report describes the clinical, histological and electron microscopic observations in a 51-year-old male with epidermodysplasia veruciformis (EV). cells with early signs of malignant transformation were found closely connected with virus infected epidermal regions. skin cancers appeared initially on sun-exposed areas, such as the face and ear lobes. The UV-induced dna repair synthesis was therefore studied, utilizing peripheral leukocytes. The patient had 40% lower UV-induced dna repair synthesis than the mean of nine healthy subjects of the same age. These results suggest that a decrease in UV-induced dna repair synthesis in combination with a possibly oncogenic viral infection may enhance the disposition for somatic mutations and malignant transformation in patients with EV.
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keywords = peripheral
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2/342. Multiple basal cell carcinomas associated with hairy cell leukaemia.

    We report the case of a caucasian woman who, between the ages of 49 and 51 years, developed multiple (> 20) basal cell carcinomas (BCC). There was no family history of BCC. No abnormalities in the human homologue of the drosophila segment polarity gene patched (PTCH), glutathione S-transferases T1 and M1, or cytochrome P450 1A1 were detected by polymerase chain reaction (PCR)-based molecular analysis. There was, however, actinic damage of the skin in sun-exposed areas. The patient was diagnosed as having hairy cell leukaemia (HCL) at the age of 51 years, based upon leucocyte morphology as assessed by light and electron microscopy, tartrate-resistant acid leucocyte phosphatase (TRAP) staining, fluorescence activated cell scanning of peripheral blood leucocytes and bone marrow histology. As the leukaemia slowly progressed over a 3-month period, the patient developed four further BCCs. Given that HCL is characterized by a profound defect in T-cell function, it is conceivable that T-cell immune dysregulation can contribute to the pathogenesis of BCC, possibly enhancing the aetiological effect of ultraviolet irradiation.
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keywords = peripheral
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3/342. Neurofibromatosis type 2 with multiple primary brain tumors in monozygotic twins.

    BACKGROUND: Although monozygotic twins with neurofibromatosis complicated by brain tumors rarely have been reported, none of them fulfilled the diagnostic criteria for neurofibromatosis type 2 (NF2). METHOD: We describe here the first pair of monozygotic twins with NF2, and the result of the molecular analysis of their NF2 gene. RESULTS: One of the brothers (Case 1) developed tetraparesis and cerebellar truncal ataxia at age 12. He had no skin lesions. Radiological examinations revealed, at one time or another, bilateral vestibular schwannomas, a foramen magnum meningioma, five supratentorial meningiomas, and multiple spinal cord tumors. He underwent three operations over a 10-year period to remove tumors. The patient is now 23 years old and is in college. Although asymptomatic when examined at age 12, CT scan revealed that his brother (Case 2) also had multiple brain tumors, including meningiomas, schwannomas, and multiple spinal tumors. Tumors were removed in eight operations over a 10-year period. The patient is now deaf and confined to a wheelchair. An identical nonsense mutation caused by a C to T transition (C169) in a CpG dinucleotide of the NF2 gene was identified in both patients. CONCLUSION: These results led us to speculate that dissimilarities with respect to time of appearance, distribution, and extent of symptoms and tumors between the twins were dependent on the influence of other genetic factors.
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ranking = 56.114258534808
keywords = schwannoma
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4/342. Hybrid carcinomas of salivary glands. Report of 4 cases and review of the literature.

    OBJECTIVE: To report 4 cases of hybrid carcinoma and to review the literature on these rare neoplasms of the salivary gland. methods: hematoxylin-eosin-stained, formalin-fixed, paraffin-embedded tissue sections from 3 parotid tumors and 1 palate tumor were examined. RESULTS: The cases were classified as adenoid cystic and mucoepidermoid carcinoma, adenoid cystic and epithelial-myoepithelial carcinoma, epithelial-myoepithelial and salivary duct carcinoma, and adenoid cystic and salivary duct carcinoma. All patients were men, 28 to 71 years old; 3 patients presented with parotid mass, and 1 patient presented with palatal mass. One patient presented with facial nerve paralysis and pain. The soft palatal tumor was a slowly growing mass with maxillary sinus involvement at the time of the diagnosis. All patients were treated with surgery and radiotherapy. CONCLUSIONS: Correct identification of 2 or more neoplastic entities will help assess the aggressiveness and metastatic potential of the tumor and influence the clinical course and treatment.
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ranking = 0.37689987023514
keywords = nerve
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5/342. Combined radical retropubic prostatectomy and rectal resection.

    OBJECTIVES: To present our experience with a small series of men who underwent simultaneous radical retropubic prostatectomy and rectal resection. methods: Three men with newly diagnosed prostate cancer were found to have concurrent rectal tumors requiring resection. All three men underwent non-nerve-sparing radical retropubic prostatectomy and abdominoperineal resection (APR) or low anterior resection (LAR) of the rectum at the same operation. In the 2 patients undergoing APR, the levators were approximated posterior to the urethra, and the bladder was secured to the pubis. The patient undergoing LAR had urinary diversion stents placed and a diverting transverse loop colostomy. RESULTS: All 3 patients had excellent return of urinary continence. One patient required reoperation in the early postoperative period for small bowel adhesiolysis and stoma revision. Another patient had a mild rectal anastomotic stricture and a bladder neck stricture; both were successfully treated with a single dilation. No other significant complications occurred in these patients. CONCLUSIONS: Radical retropubic prostatectomy can safely be performed with partial or complete rectal resection in a single operation. A few minor modifications of the standard radical retropubic prostatectomy in this setting are suggested.
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ranking = 0.37689987023514
keywords = nerve
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6/342. Radiologic features of intrahepatic bile duct adenoma: a look at the surface of the liver.

    We report the radiological features of intrahepatic bile duct adenoma (BDA) in three patients. BDA was shown as a small mass located in the peripheral region of the liver with each imaging modality: a hypervascular mass on angiography and a mass appearing as early nodular enhancement found disproportionately evident compared with their small size and distinct delayed or prolonged enhancement on CT. BDA should be included in the diseases to be differentiated from hypervascular hepatic tumors.
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keywords = peripheral
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7/342. Two-stage operation for endobronchial lipoma and lung cancer using bronchoscopy and thoracoscopy in an elderly patient with chronic obstructive pulmonary disease.

    We present an 82-year-old man with chronic obstructive pulmonary disease with endobronchial lipoma, obstructing the right lower lobe bronchus, and lung adenocarcinoma in the peripheral lung of the right upper lobe (clinically T1N0M0). The endobronchial lipoma was thus first removed by bronchoscopic snaring forceps and laser therapy, resulting in an improvement of the pulmonary function. One month later, the lung adenocarcinoma was removed using thoracoscopy. The postoperative course was satisfactory, and the patient is now doing well without any tumor recurrence 11 months after surgery. For elderly chronic obstructive pulmonary disease patients with endobronchial lipoma and T1N0M0 lung cancer, a two-stage operation, consisting of bronchoscopic resection followed by a thoracoscopic resection for lung cancer, was found to be a safe and effective method of treatment while maintaining sufficient pulmonary function.
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keywords = peripheral
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8/342. Esophageal cancer and right aortic arch associated with a vascular ring.

    We report on the problems of diagnosis and surgical technique from the standpoint of mediastinal anatomy in patients with esophageal cancer and right aortic arch associated with a vascular ring. We propose that left thoracotomy plus sternotomy is the best approach to allow visualization of the bilateral recurrent laryngeal nerves.
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ranking = 0.37689987023514
keywords = nerve
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9/342. Simultaneous occurrence of non-Hodgkin's lymphoma and acute myelomonocytic leukemia.

    The simultaneous occurrence of nodular poorly differentiated lymphocytic lymphoma (NLPD) and acute myelomonocytic leukemia (AMML) was confirmed by the histologic examination of lymph node section, peripheral blood, and bone marrow. This association in the absence of previous chemotherapy or radiotherapy has not been previously documented. There have been six previous case reports of patients with non-Hodgkin's lymphocytic lymphoma who, during treatment with alkylating agents or radiotherapy, developed acute myeloid leukemia (AML). These methods of therapy may have had a leukemogenic role in such patients. The possible relationship between these two diseases is discussed.
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ranking = 1
keywords = peripheral
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10/342. Malignant schwannoma of the stomach in a patient with von Recklinghausen's disease.

    A patient with von Recklinghausen's disease died with a malignant schwannoma of the stomach and was found at autopsy to have neurofibromatosis of the gastrointestinal tract, a plexiform neurofibroma of the myocardium and a phaechromocytoma. Malignancy of the gastrointestinal tract in von Recklinghausen's disease is rare, and this case highlights the difficulties in histological diagnosis of malignant nerve sheath tumours.
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ranking = 351.55773711406
keywords = nerve sheath tumour, sheath tumour, schwannoma, nerve sheath, sheath, nerve
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