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1/1088. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease.

    We diagnosed phakomatosis pigmentovascularis type IIIb in an 11-month-old baby who had a giant nevus spilus, a nevus flammeus, and moyamoya disease. Development of the patient was normal until 6 months of age when he developed a sudden onset of focal seizures and left hemiparesis. This patient represents the sixth case of phakomatosis pigmentovascularis type IIIb, including three cases in the Japanese literature, reported thus far. However, to our knowledge, this is the first case with an association to moyamoya disease.
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keywords = disease
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2/1088. dermatofibrosarcoma protuberans in an infant with a history of choroid plexus papilloma.

    We describe an infant with a dermatofibrosarcoma protuberans (DFSP) who also had a choroid plexus papilloma. This report underscores the occurrence of DFSP in this age group and may support a neural pathogenesis of this tumor.
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ranking = 0.016336758449679
keywords = tube
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3/1088. Primary endometrioid carcinoma of fallopian tube. Clinicomorphologic study.

    Twenty cases of primary Fallopian tube endometrioid carcinoma (PFTEC) are presented in the paper. This accounts for 42.5% of all histologic forms of primary Fallopian tube carcinoma (PFTC) found in our Department. The youngest patient was 38, and the oldest 68 years (mean: 56 years). Seven patients were nulliparas. Only two cases were bilateral. According to FIGO staging, 13 cases were evaluated as stage I, 4 as II, and 3 as stage III. Due to the histologic grading, 8 tumors were classified as well, 7 as moderately, and 5 as poorly differentiated. In the time of preparation of the manuscript, 12 women were still alive, 2 of them with recurrent disease. The follow-up of patients without recurrence ranged from 4 to 120 months (median: 63). Eight patients had died (survival time: from 4 to 65 months; median: 26). Metastases were found in 8 patients, especially to ovaries. In 14/20 cases of PFTEC various forms of tubal wall invasion were observed. blood or lymphatic vessels involvement was found in 9 patients. Six of them had died and one is alive with the symptoms of disease. Immunohistochemical detection of the mutant form of p53 protein and oncogene product, c-erbB-2, was studied in 17 cases. Nine patients exhibited simultaneous p53 protein accumulation and c-erbB-2 expression. 2/9 of these patients are alive with recurrent tumors and 4/9 died. Endometrioid carcinoma of the Fallopian tube can be characterized by a tendency to superficial invasion of tubal wall and in a half of the cases by invasion of vessels. The majority of these tumors were diagnosed at an early stage tumors.
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ranking = 3.1475944050326
keywords = fallopian tube, disease, tube
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4/1088. Synchronous and metachronous gastric adenocarcinoma: case report and literature review.

    Whilst synchronous adenocarcinoma of the stomach is well documented, metachronous primary disease is exceedingly rare. We report a man with a family history of colonic and gastric cancer, who underwent a resection of a Duke's C adenocarcinoma of the rectum, aged 56 years, and a proximal partial gastrectomy for synchronous stage 1 gastric adenocarcinomas of the lesser curve, aged 61 years. Nine years later, a metachronous gastric primary was discovered in the gastric remnant, necessitating total gastrectomy. Total gastrectomy is the operation of choice for synchronous gastric primaries as it ensures clearance and prevents metachronous growth. However, it may not be appropriate for all gastric cancer as operative morbidity and mortality are increased, and because synchronicity and metachronicity of gastric cancer are uncommon. Moreover, there are no consistent data to demonstrate a survival advantage for total compared with partial gastrectomy for operable gastric cancer. If, after partial gastrectomy, synchronous disease is detected in the resected specimen (as in this reported case), endoscopic surveillance for metachronous disease is advised, since this may be amenable to surgical cure.
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5/1088. Multiple skull base meningioma: case report.

    BACKGROUND: The incidence of multiple skull base meningiomas varies from 1 to 3% in different series. skull base meningiomas are rare. The pathogenetic role of low-dose radiation seems to be fairly well established in the oncogenesis of meningiomas. Calvarial location and multiplicity seem to be among the distinctive features of radiation-induced meningiomas. skull base location is a very rare occurrence, mainly because the path of irradiation does not significantly involve this region. CASE REPORT: We describe a rare case of simultaneous occurrence of two skull base meningiomas in a 66-year-old female. This patient underwent low-dose irradiation for tinea capitis when she was 8 years old. The patient complained of nuchal pain, paresthesias in both hands, and progressive weakness on her right side. She was admitted to the hospital in September 1994. An MRI showed two masses, one located at the level of the tuberculum sellae and the other at the foramen magnum. These seemed very likely to be multiple meningiomas. The latter lesion, which was more symptomatic and dangerous, was operated on first. Six months later, elective treatment of the suprasellar meningioma was performed with success. CONCLUSION: The actual role of previous head irradiation in the oncogenesis of the present meningiomas remains somewhat unclear. Proper management and judicious use of skull base surgery techniques were key factors in the successful treatment of the patient.
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ranking = 0.0032673516899358
keywords = tube
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6/1088. Hepatic adenomatosis: rapid sequence MR imaging following gadolinium enhancement: a case report.

    Hepatic adenomas are primary liver tumors usually associated with underlying metabolic disease or with anabolic steroid or oral contraceptive use. Hepatic adenomatosis (HA) is defined as the presence of more than four adenomas. Only 13 cases of HA have been reported in patients without glycogen storage disease or steroid use. We report a case of HA imaged by postcontrast T1-weighted images obtained during a breath-holding series. The lesions were most conspicuous 3-4 min after contrast administration; 4 of the 5 tumors were not identified on T2-weighted images. Unlike previous reports of HA in which the lesions remained hyperintense during sequential postcontrast imaging, the smaller lesions in this case demonstrated contrast washout, thereby distinguishing them from hemangiomata.
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keywords = disease
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7/1088. Detection of human papilloma virus type 58 in a case of a perianal bowen's disease coexistent with adult T-cell leukemia.

    A case of bowen's disease (BD) that appeared in the perianal region of a 65-year-old Japanese woman coexistent with chronic adult T cell leukemia (ATL) is described. Histopathological findings revealed that irregularly arranged tumor cells with atypical nuclei throughout the epidermis, which itself disclosed hyperkeratosis, dyskeratotic cells, and clumping cells. Positive staining for HPV antigens was immunohistochemically seen in several nuclei of the tumor cells. Electron microscopic study of the tumor tissue disclosed virus particles of about 50 nm in diameter form the squamous cells. A positive band at 256 bp was obtained by PCR using HPV-L1 primer. The amplified DNA by L1 primer completely corresponded to that of HPV-58.
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ranking = 0.83333333333333
keywords = disease
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8/1088. A case of multiple sebaceous epithelioma: analysis of microsatellite instability.

    Sebaceous gland tumor is a rare disease that is a sign of muir-torre syndrome, an autosomal, dominantly inherited genodermatosis characterized by the presence of at least one sebaceous gland tumor and a minimum of one internal malignancy. Recent studies have indicated that defective dna mismatch repair occurs in muir-torre syndrome. Cutaneous lesions may occur before diagnosis of the internal cancer. We describe a 64-year-old male patient with multiple sebaceous epitheliomas with no evident internal malignancy. microsatellite instability, determined by examining dinucleotide CA repeats at the microsatellite loci, was observed in DNA from one sebaceous epithelioma but not from the other two sebaceous epitheliomas or from one basal cell epithelioma with sebaceous differentiation, suggesting that this condition is unlikely to be due to germ-line mutation of mismatch repair genes.
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keywords = disease
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9/1088. Late recurrence of a uterine clear cell adenocarcinoma confined to an endometrial polyp: case report.

    A patient with an endometrial clear cell adenocarcinoma confined to a polyp developed recurrent disease in the abdomen and pelvis four years following hysterectomy. Treatment issues related to this uncommon clinical situation are discussed.
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10/1088. Paget's disease of the male breast associated with intraductal carcinoma.

    Paget's disease of the breast is a rare condition with an incidence of 3% to 5% of all mammary malignancies. Of all malignant breast cancer, 1% occurs in male patients, and thus, Paget's disease of the male breast is extremely rare. We present a case of intraductal carcinoma of the male breast presenting as Paget's disease.
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ranking = 1.1666666666667
keywords = disease
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