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1/326. Composite ganglioglioma and dysembryoplastic neuroepithelial tumor.

    Both ganglioglioma and dysembryoplastic neuroepithelial tumors are well-recognized glial-neuronal neoplasms associated with chronic epilepsy and cortical dysplasia (neuronal migration abnormalities). The exact relationship between these 2 glial-neuronal tumors continues to be debated. This article reports a case of a composite ganglioglioma and dysembryoplastic neuroepithelial tumor occurring in a 36-year-old woman in the left temporal lobe region. The resection histologically demonstrated distinct areas of ganglioglioma and dysembryoplastic neuroepithelial tumor. A focal area of cortical dysplasia is also identified. The MIB-1 labeling indexes in both components were low (<1% of tumor cell nuclei). The coexistence of these 2 lesions and cortical dysplasia suggest a possible etiologic relationship between these 2 tumors.
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ranking = 1
keywords = neoplasm
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2/326. Giant cell carcinoma of the lung. Report of a case with cytohistologic and clinical correlation.

    BACKGROUND: Giant cell carcinoma (GCC) of the lung is an unusual tumor characterized by an aggressive outcome. CASE: A peripheral lung tumor was observed in an elderly male. At presentation the clinical symptoms were cough, thoracic pain and hemoptysis. Chest roentgenography showed a left pleural effusion and neutrophilia in the blood. Bronchoscopic examination showed a peripheral tumor mass that could not be biopsied. bronchoalveolar lavage was negative. The patient underwent a cerebrovascular accident and died. The autopsy showed a peripheral giant cell tumor of the left lung that involved regional and mediastinal lymph nodes. touch imprints showed tridimensional clusters of pleomorphic and large cells, some of which were multinucleated, containing leukocytes in their cytoplasm. CONCLUSION: This case illustrates the typical cytohistologic features of GCT of the lung, which should be considered in the differential diagnosis of any peripheral lung tumor composed of large cells. Clinical correlation is helpful in reaching the correct diagnosis.
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ranking = 8.7197484402439
keywords = carcinoma
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3/326. The inv(11)(p15q22) chromosome translocation of therapy-related myelodysplasia with NUP98-DDX10 and DDX10-NUP98 fusion transcripts.

    Chromosomal abnormalities involving the 11p15 or 11q22-23 bands have been reported in several types of human neoplasms including hematopoietic malignancies. The abnormalities are observed in therapy-related malignancies and less frequently in de novo myeloid malignancies. Abnormality of the MLL gene located on chromosome 11q23 has been well known in therapy-related myeloid malignancies, but it has been reported only recently that the inv(11)(p15q22) in de novo or therapy-related myeloid malignancies results in the fusion of NUP98 on chromosome 11p15 and DDX10 on chromosome 11q22. NUP98 is a nucleoporin that composes the nuclear pore complex and is the target gene in leukemia with the t(7;11)(p15;p15). The DDX10 gene encodes a putative adenosine triphosphate-dependent DEAD box rna helicase. Here we present another patient with acute myelocytic leukemia (M4) transformed from chronic myelomonocytic leukemia with the inv(11) chromosome who had been treated with etoposide for a germ cell tumor. By reverse transcription polymerase chain reaction (RT-PCR) of the rna from the leukemic cells of the patient, DDX10-NUP98 and NUP98-DDX10 fusion transcripts were detected. Our case confirms that the inv(11) is a rare chromosomal translocation that is associated with therapy-related or de novo myeloid malignancy and involves NUP98 and DDX10 but not MLL. RT-PCR of the fusion transcripts might be applied to the detection of a small number of leukemic cells in the bone marrow or blood of patients in remission or in the cells harvested for autologous transplantation.
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ranking = 1
keywords = neoplasm
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4/326. Angiosarcoma of the testis.

    A primary angiosarcoma of the testis in a 74-year-old patient was a highly anaplastic epthelioid angiosarcoma, which was positive for endothelial markers immunohistochemically. The tumour was unrelated to testicular germ cell neoplasm; the patient had received no previous radiation or chemotherapy.
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ranking = 1
keywords = neoplasm
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5/326. Spontaneous intralesional haemorrhage in dysembryoplastic neuroepithelial tumours: a series of five cases.

    Five patients with dysembryoplastic neuroepithelial tumour (DNT) showing extensive secondary haemorrhage, a finding not previously associated with these neoplasms, are described. The clinical presentations, neuroimaging findings, and histopathological features of these patients are reviewed. One patient, a previously asymptomatic 12 year old girl, presented with an acute intracerebral haemorrhage into a DNT. A further four young adults with histories of intractable partial and generalised seizures dating from childhood showed significant chronic haemorrhages within DNT, the MRI appearances in one patient giving a false impression of a cavernoma. Histopathology disclosed vascular abnormalities within these tumours which, together with other factors discussed, may have predisposed these tumours to haemorrhage.
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ranking = 1
keywords = neoplasm
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6/326. A frame shift mutation in the dna-binding domain of the androgen receptor gene associated with complete androgen insensitivity, persistent mullerian structures, and germ cell tumors in dysgenetic gonads.

    OBJECTIVE: To describe the molecular, cytogenetic, immunohistochemical, and endocrinologic characteristics of a young 46,XY female with persistent mullerian structures and germ cell tumors in dysgenetic gonads. DESIGN: Descriptive case study. SETTING: Mackay Memorial Hospital and National Yang-Ming University, Taipei, taiwan. PATIENT(S): A 22-year-old 46,XY female with persistent mullerian structures, a low level of serum testosterone, and no apparent adnexal masses. INTERVENTION(S): Laparoscopic removal of the dysgenetic gonads. MAIN OUTCOME MEASURE(S): Detection of an androgen receptor gene mutation by a semiautomated dna sequencer, of the chromosomal complement by cytogenetic examination, of placental alkaline phosphatase activity by immunohistochemical analysis, and of neoplasms in dysgenetic gonads by histologic studies. RESULT(S): A unilateral gonadoblastoma and a contralateral gonadoblastoma associated with a dysgerminoma were found in the excised gonads. The tumors had a 46,XY complement. Placental alkaline phosphatase was present in the tumor cells. A frameshift mutation in the dna-binding domain of the androgen receptor gene was detected in the patient's blood and the tumor tissues. A five-nucleotide "AGGAA" deletion at codons 608 and 609 of the androgen receptor gene resulted in a missing arginine and lysine as well as a frameshift that introduced a stop codon 12 amino acid downstream from the mutation. CONCLUSION(S): Molecular genetic analysis of the androgen receptor gene aids in the rapid diagnosis of complete androgen insensitivity irrespective of atypical clinical phenotypes and endocrinologic parameters.
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ranking = 1
keywords = neoplasm
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7/326. Microcystic meningioma arising in a mixed germ cell tumor of the testis: a case report.

    We report a case of a microcystic variant of meningioma arising in a mixed germ cell tumor of the testis composed predominantly of mature and immature teratoma with elements of seminoma and embryonal carcinoma. We believe this is the first such case of a meningioma arising in a teratoma within a gonadal or extragonadal site. The meningiomatous component showed positive immunohistochemical staining for epithelial membrane antigen and a lack of staining for cytokeratin, factor viii, CD31, and alpha-fetoprotein. Recognition of a non-germ cell tumor arising in the setting of a teratoma in the testis may be prognostically important depending on the nature of the non-germ cell component and whether it has spread beyond the testis.
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ranking = 1.7439496880488
keywords = carcinoma
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8/326. Diagnostic and therapeutic approach to sialoblastoma: report of a case.

    The natural history of a rare parotid tumor, the sialoblastoma (embryoma) is reported. It is a blastematous neoplasm said to recapitulate the epithelial differentiation of a gland at various stages of development. The tumor grew in a period of 5 years (from shortly after birth until it was excised) to a firm asymptomatic mass measuring 5 cm in greatest diameter. With a diagnosis of adenoid cystic carcinoma (a common misdiagnosis), the patient was referred to the reporting institution, where the diagnosis was revised. Five months later, completion parotidectomy for presumed recurrence failed to demonstrate residual tumor. One year later, the patient appears to be free of disease. The authors propose that sialoblastomas should be regarded neither as benign nor malignant, but as one single disease with local infiltrative potential. Based on this concept, sialoblastomas can be treated with early conservative surgery alone, provided that free margins are obtained.
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ranking = 2.7439496880488
keywords = carcinoma, neoplasm
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9/326. Primary testicular seminoma in a patient with a history of extragonadal non-seminomatous germ cell carcinoma.

    Extragonadal germ cell carcinoma represents between 3% and 5% of all germ cell carcinomas. A metachronous primary germ cell carcinoma is exceedingly rare in these patients. We report the eighth case, which occurred in a 29-year-old man who presented with testicular seminoma 7 years after his initial presentation with extragonadal non-seminomatous germ cell carcinoma. The seven other patients also presented with extragonadal non-seminomatous germ cell carcinoma, followed subsequently by testicular seminoma in 6 patients and non-seminomatous germ cell carcinoma in the seventh. The mean time to presentation was 8 years. Although rare, this case emphasizes the need for long-term surveillance, including testicular evaluation of patients with a history of extragonadal germ cell carcinoma.
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ranking = 19.183446568537
keywords = carcinoma
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10/326. Ovarian germ cell neoplasm in pregnancy.

    Ovarian germ cell neoplasm in pregnancy, with variable outcomes after debulking surgery is described.
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ranking = 5
keywords = neoplasm
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