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21/1382. NUP98 is fused to PMX1 homeobox gene in human acute myelogenous leukemia with chromosome translocation t(1;11)(q23;p15).

    The nucleoporin gene NUP98 was found fused to the HOXA9, HOXD13, or DDX10 genes in human acute myelogenous leukemia (AML) with chromosome translocations t(7;11)(p15;p15), t(2;11)(q35;p15), or inv(11)(p15;q22), respectively. We report here the fusion between the NUP98 gene and another homeobox gene PMX1 in a case of human AML with a t(1;11)(q23;p15) translocation. The chimeric NUP98-PMX1 transcript was detected; however, there was no reciprocal PMX1-NUP98 fusion transcript. Like the NUP98-HOXA9 fusion, NUP98 and PMX1 were fused in frame and the N-terminal GLFG-rich docking region of the NUP98 and the PMX1 homeodomain were conserved in the NUP98-PMX1 fusion, suggesting that PMX1 homeodomain expression is upregulated and that the fusion protein may act as an oncogenic transcription factor. The fusion to NUP98 results in the addition of the strong transcriptional activation domain located in the N-terminal region of NUP98 to PMX1. These findings suggest that constitutive expression and alteration of the transcriptional activity of the PMX1 homeodomain protein may be critical for myeloid leukemogenesis.
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ranking = 1
keywords = leukemia, m
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22/1382. Secondary myeloid/natural killer cell precursor acute leukemia following essential thrombocythemia.

    The de novo leukemic transformation of essential thrombocythemia is a rare event, and usually associated with previous treatments. We describe a patient who received treatments with nitrosourea for long-standing essential thrombocythemia and subsequently developed extramedullary tumors, tentatively diagnosed as lymphoblastic lymphoma. Combination chemotherapy was initially successful, but relapsed with marked bone marrow involvement. Surface marker analysis revealed that the tumor cells had CD5, CD7, CD33, CD34, and CD56 antigens but lacked other T-cell, and B-cell markers. Immunogenotypical studies revealed germline configurations for both T-cell receptors and immunoglobulin genes. These clinical and phenotypical features are consistent with a myeloid/natural killer cell precursor leukemia, a recently proposed distinct clinical entity. To our knowledge, this is the first report of secondary leukemia of myeloid/ natural killer cell precursor origin, and suggest that myeloid/natural killer cell precursor might be a potent target of therapy-related leukemia.
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ranking = 1.3999436854057
keywords = leukemia, m
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23/1382. Squamous cell carcinoma arising in a Warthin's tumor.

    Warthin's tumor is a well-defined salivary gland neoplasm consisting of epithelial and lymphoid components. However, malignant transformation is extremely rare. Such a patient who developed squamous cell carcinoma within a Warthin's tumor of the parotid gland is described and possible pathogenesis is discussed.
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ranking = 8.0449420423268E-5
keywords = m
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24/1382. Gastric adenocarcinoma associated with fundic gland polyps in a patient with attenuated familial adenomatous polyposis.

    Familial adenomatous polyposis (FAP) is a rare autosomal dominant precancerous condition of the colon caused by mutations in the adenomatous polyposis coli (APC) tumor suppressor gene. FAP is characterized by the appearance of innumerable adenomatous polyps throughout the large bowel. Fundic gland polyps are the most common gastric lesion in FAP. It is generally believed that fundic gland polyps have little or no potential for malignant transformation in the population at large, and only a few case reports describe the development of high grade dysplasia or gastric adenocarcinoma associated with diffuse fundic gland polyposis in patients with FAP. We report the second case of gastric adenocarcinoma intimately associated with fundic gland polyposis in a family with an attenuated form of FAP. The patient had undergone routine screening per current guidelines because of his known mutation in the APC gene. This suggests that malignant transformation of fundic gland polyps in patients with FAP occur more frequently than previously believed. Current screening recommendations may not be sufficient for patients with FAP or its attenuated forms.
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ranking = 0.00014627167349685
keywords = m
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25/1382. pancreatitis associated with Crohn's disease: a premalignant state for cystadenocarcinoma of pancreas?

    We report a 74-yr-old woman with Crohn's disease and acute pancreatitis who, 3 yr after resolution of the latter, developed cystadenocarcinoma of the pancreas. No drug, toxin, or other etiologies including contiguous duodenal involvement were identified as responsible for the pancreatitis, suggesting that pancreatitis was an extraintestinal manifestation of her Crohn's disease. Could Crohn's-associated pancreatitis be a premalignant state for cystadenocarcinoma of the pancreas?
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ranking = 5.1195085723898E-5
keywords = m
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26/1382. Squamous cell carcinoma arising in an intradiploic epidermoid cyst.

    A 71-year-old woman presented with the symptoms of a posterior cranial fossa mass. CT and MRI revealed a lytic lesion in the occipital bone and a tumour infiltrating the dura mater, venous sinuses and cerebellum. Histopathology demonstrated a moderately differentiated squamous cell carcinoma arising from a primarily intradiploic epidermoid cyst. Despite surgery and radiotherapy, the tumour progressed and the patient died 1 year later.
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ranking = 9.8733379610375E-5
keywords = m
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27/1382. 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 = 0.00020112355105817
keywords = m
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28/1382. Pronounced cystic transformation of the rete testis. MRI appearance.

    BACKGROUND: Cystic transformation of the rete testis is a little-known partial or complete obstruction of the spermatozoa-containing secretion of the head of the epididymis. Depending on its severity, either ectasia or a cystic transformation occurs, which spreads to the network of convoluted seminiferous tubules in the mediastinum testis. Findings in contrast-enhanced MRI examinations are characteristic and may help to differentiate this benign entity from malignant neoplasia. The authors present two pronounced cases in different stages, documenting the broad spectrum of possible involvement.
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ranking = 6.9479044911004E-5
keywords = m
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29/1382. Demonstration of HPV 24 in long-standing Heck's disease with malignant transformation.

    We report on the rare case of a 64-year-old European woman with a viral acanthoma of the oral mucosa of the clinical Heck type with unprecedented molecular-biological proof of HPV 24 dna and so far undescribed malignant transformation with a long existence of the vegetation. After surgical therapy a circumscribed relapse of viral acanthoma developed, which responded favourably to a combined antiviral and antiproliferative therapy with acitretin perorally and interferon, first subcutaneously, later intralesionally for 3 months.
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ranking = 8.0449420423268E-5
keywords = m
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30/1382. Pre-Kaposi's sarcoma: an expansion of the spectrum of Kaposi's sarcoma lesions.

    We report on a lymphoedematous form of classic Kaposi's sarcoma (KS) in which characteristic purplish lesions were surrounded by atypical oedematous, flesh-coloured papules. Histological examination of these papular lesions revealed a proliferation of grouped, rather thick-walled capillaries with inflammatory infiltrates. Hot-start PCR amplification with KS 330-233 primer sequences demonstrated the presence of human herpesvirus 8 (HHV-8) sequences. In addition, cells isolated from these oedematous papules showed morphological and immunohistochemical features similar to those reported for KS-derived spindle cells. As a whole, these results suggest that these oedematous papular lesions represent pre-KS lesions and may expand the clinico-pathological spectrum of KS. The role of oedema in their induction is discussed.
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ranking = 0.00012798771430974
keywords = m
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