Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

---

Query: EC:2.7.10.1 (ERK)
95,504 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Anaplastic large cell lymphoma (ALCL), CD30+, is a subtype of T-non-Hodgkin's lymphoma (NHL). Its most common form is a classical systemic type that involves multiple nodal and extranodal sites. In this study, morphologic, immunohistologic, and genetic studies were performed on ALCL cases in Pakistani patients. The median age of the patients in this study was 45 years (age range: 5-70 years), with a male to female ratio of 3.4:1. Thirty-seven (37) patients were diagnosed to have Ki-1 (CD30+) ALCL, which constituted 2% of all NHLs and 12.6% of all T-NHLs, over a period of 11 years (January 01, 1992-December 31, 2002). The tumors were of either T- or null-cell type with constant (100%) expression of CD30 (Ki-1). The majority of the cases (89.2%) expressed EMA, whereas 40.5% of the cases expressed either CD45 (LCA), CD45RO (UCHL1), or ALK. The mean age of ALCL patients with null-cell phenotype was 33.8 years as compared to those with T-cell phenotype having a mean age of 36.3 years. Out of the 37 cases diagnosed as ALCL, amplifiable DNA was isolated from 28 cases, which were further assessed for T-cell clonality for T-cell receptor (TCR)-beta, gamma, and immunoglobulin heavy chain (IgH) for the FR2 and FR3 regions. The polymerase chain reaction (PCR) technique demonstrated clonal rearrangement of the TCR beta, gamma, and IgH regions in 15 (53.6%), 11 (39.3%), and 2 (7.1%) ALCL cases, respectively, out of 28 cases. Association of Epstein-Barr virus (EBV) was noted in seven out of 28 cases (25%) of ALCL by PCR, whereas ISH for EBV-encoded nuclear RNA-1 (EBER-1) detected the presence of EBV in two (16.7%) out of 12 cases, where one was T-cell ALCL and the other null-cell ALCL. Immunostaining for LMP-1 could not be performed, because tissue material was not available. In conclusion, our study demonstrated that the prevalence of ALCL in Pakistan is comparable to that reported for some of the Asian communities and by the International Lymphoma Study Group and that EBV could be partly responsible for the pathogenesis of ALCL.
...
PMID:Prevalence and characterization of anaplastic large cell lymphoma and its association with Epstein-Barr virus in Pakistani patients. 1564 4

Anaplastic large cell lymphoma (ALCL) was first described by Stein et al. in 1985, at that time neoplastic cells were labeled by the monoclonal antibody CD30. ALCL was included as a differentiate entity in the reviewed Kiel and REAL classification. ALCL carries the t (2; 5) (p23; q35) translocation; the absence of ALK kinase from normal lymphoid cells indicates that immunohistochemical expression of ALK is specific for the (2; 5) translocation. This disease is characterized by a diffuse proliferation of large anaplastic cells with kidney-shaped/horse-shoe nuclei. A distinguishing feature is a perinuclear eosinophilic region that represents a prominent Golgi apparatus. These cells are named hallmark-cells being almost pathognomonic. Immunohistochemically the most important features are that tumor cells consistently express CD30 and EMA on the cell membrane and in the Golgi region, while ALK immunostaining is usually both, cytoplasmic and nuclear. To our knowledge only two cases of primary ALCL of the testis have been reported. Hereby we present a case of a typical ALCL expressing ALK and CD30, which presented with subcutaneous nodules and bilateral testicular mass, without systemic involvement.
...
PMID:Primary anaplastic large cell lymphoma of the testis. 1578 13

Meningothelial hyperplasia is a poorly characterized entity, often associated with advanced age, chronic renal failure, trauma, hemorrhage, and neoplasia. In order to elucidate the nature of this lesion, 11 cases defined by the presence of nests of 10 or more cell layers thick, were compared with normal arachnoidal cap cells and meningiomas. Immunohistochemistry and FISH were performed to determine NF2 (merlin), protein 4.1B, EMA, progesterone receptor (PR), EGFR, survivin, VEGF, PDGF-BB, PDGFR-beta, E-cadherin, and cathepsin D status. All cases had at least one putative predisposing factor, including hemorrhage (7), chronic renal disease (5), old age (5), trauma (1), and an adjacent optic nerve pilocytic astrocytoma (1). There was typically a discontinuous growth pattern, with no invasion of surrounding normal tissue. No gene deletions were found, though scattered polyploid cells were seen in 2 cases. The immunoprofile was similar to normal cap cells with one exception; whereas normal cells were uniformly negative for PR, nuclear positivity was seen in 64% of hyperplasias, a frequency similar to that of benign meningiomas. Our data suggest that meningothelial hyperplasia is a reactive process that is usually distinguishable from meningioma based on clinicopathologic and genetic features. It may be preneoplastic in some, though further studies are needed to test this hypothesis.
...
PMID:Meningothelial hyperplasia: a detailed clinicopathologic, immunohistochemical and genetic study of 11 cases. 1591 82

Only two karyotypes of perineurioma have previously been reported, 46XX,del(10)(q22q24),der(10),del(22)(q11-12q?)/47, idem,+der(10) (in a sclerosing perineurioma of the finger) and 45,XX,add(14)(p13),-22,add(22)(q11.2) (in an intraneural perineurioma). We investigated the clinicopathologic and cytogenetic findings in four consecutive perineuriomas in children, including two small (< or =1 cm) digital sclerosing perineuriomas, a 2-cm intraneural perineurioma, and a 16-cm abdominal soft tissue perineurioma. All lesions showed plump perineurial cells in a complex whorled configuration. Immunohistochemical (strong EMA immunostaining in all cases) and ultrastructural (in three of three lesions examined) evidence of perineurial differentiation was present. The sclerosing perineuriomas showed 46,XY,t(2;10)(p23;q24) and 47,XX,add(3)(q23),add(6)(q21),-5,-9,-10,-22,+mar1,+mar2,+mars; the intraneural tumor showed 46,XX,add(2)(q11.2),add(3)(q12); and the abdominal soft tissue perineurioma showed 46,XX,t(8;9)(q13;q22). Metaphase FISH analysis for an ALK gene rearrangement in the sclerosing perineurioma with t(2;10) was negative; the ALK signal remained on the der(2). We conclude that perineuriomas display mostly simple karyotypes, characterized by one or few chromosomal rearrangements or numerical changes. In conjunction with the previously published sclerosing perineurioma karyotypes, the findings of chromosome 10 aberrations, t(2;10)(p23;q24) and monosomy 10 in two sclerosing perineuriomas, indicate that rearrangements and/or deletions of 10q are a consistent finding in this variant of perineurioma. The findings also expand previous assertions that chromosome 22 abnormalities are pathogenetic in perineurioma and suggest that diverse genetic tumorigenic mechanisms may exist, possibly depending on the subtype.
...
PMID:Cytogenetic aberrations in perineurioma: variation with subtype. 1609 5

A 40-year-old previously healthy lady presented with nasal obstruction and localized plaques over the right arm. She developed complete nasal obstruction due to a mass in the right nasal cavity and skin lesions that ulcerated to present as ecthyma gangrenosum like lesions. Patient's condition deteriorated fast and she developed icterus with fatal outcome within 4 weeks of developing skin lesions. Nasal and skin biopsy revealed angiocentric T-cell lymphoma, which on immuno-phenotyping revealed CD-3 positive; and CD-20, CD-30, ALK and EMA negativity. She was seronegative for HIV. Final diagnosis of CD-3 positive extranodal T-cell lymphoma of nasal type was made. Extranodal T-cell lymphomas are very aggressive NHLs with poor prognosis. Prognosis depends on histology, stage of the disease and sites of involvement. NK/T cell lymphoma of nasal type is common with EBV association. Skin involvement is rare and is also an indicator of poor prognosis.
...
PMID:CD-3 positive extranodal T-cell lymphoma of nasal type with skin involvement. 1676 37

Malignant glioneuronal tumors of the brain are rare and poorly characterized. Herein, we report the clinicopathologic features of three examples with unusual morphologies including both glial and neuronal differentiation. Hematoxylin and eosin-stained slides were reviewed in all cases. Immunohistochemical stains were performed on formalin-fixed, paraffin-embedded sections. Transmission electron microscopy (EM) was performed on both formalin-fixed (n=1) and paraffin embedded tissue (n=2). The immunogold technique for localization of GFAP was also performed. Two patients were male and one was female, age 66, 84, and 34 years, respectively. Radiologic studies demonstrated hyperdensity on CT (n=3), multicentricity (n=2), and a cortical based solid component with a cystic extension into underlying white matter (n=2). At surgery, all three tumors were superficial and relatively circumscribed. Histologically, they were composed of large epithelioid cells (n=3), spindle cells (n=1), and poorly differentiated smaller cells with high nuclear/cytoplasmic ratios (n=1). Brisk mitotic activity and coagulative non-palisading necrosis were present in all cases. The tumors were immunopositive for GFAP (n=3), S-100 (n=3), synaptophysin (n=3), chromogranin (n=3), Neu-N (n=2), and neurofilament protein (n=2). Stains for EMA were negative. EM demonstrated convincing neurosecretory granules in one case, some in filament-containing cells immunogold labeled for GFAP. Two patients expired 3-5 weeks after surgery. True malignant neoplasms with glial and neuronal differentiation do occur in the central nervous system of adults and may pursue a highly aggressive course. The use of minimal diagnostic criteria, e.g., immunoreactivity for a single antigen like neurofilament protein, may not be sufficient and should be discouraged.
...
PMID:Unusual malignant glioneuronal tumors of the cerebrum of adults: a clinicopathologic study of three cases. 1695 28

The category of mixed glioneuronal tumors of the CNS is rapidly losing its definition as encompassing tumors composed of histologically distinct neuron variants and glia. We encountered five ependymomas with neuronal differentiation seen in two by histology, in two by immunohistochemistry alone, and in one by electron microscopy. Antibodies against GFAP, S-100 protein, neurofilament protein, chromogranin, synaptophysin, Neu-N, and EMA were applied. Ultrastructural studies were also performed. In addition, 33 randomly selected ependymomas of various histologic types were screened for these same antigens. Cases 1 and 2 were anaplastic and showed clearly defined neuropil islands or pale islands as in nodular desmoplastic medulloblastoma, respectively. The tumors affected a 16-year-old male and a 5-year-old female and involved the right frontoparietal lobe and fourth ventricle, respectively. The islands were positive for synaptophysin and Neu-N (cases 1 and 2), and chromogranin (case 1). Cases 3-5, as well as 7 of the 33 screened ependymomas, showed a suggestion of neuronal differentiation by immunohistochemistry alone, including immunoreactivity for Neu-N (n = 8), synaptophysin (n = 4), neurofilament protein (n = 4), and chromogranin (n = 2). Five tumors each were WHO grade II and III. Electron microscopy performed on the two cases with neuronal islands demonstrated microtubule bundles and dense core granules (case 1) and poorly differentiated cells with high nuclear/cytoplasmic ratios, with intermediate filament accumulation and rare cilia (case 2). Cases identified by immunohistochemistry or electron microscopy demonstrated dense core granules (n = 5) and aligned microtubules (n = 3). Neuronal differentiation occurs in ependymomas but is less frequently definitive (histologic, ultrastructural) than merely a limited immunohistochemical finding. The clinical significance of these observations is unknown but deserves further exploration.
...
PMID:Ependymomas with neuronal differentiation: a morphologic and immunohistochemical spectrum. 1706 Oct 76

Leiomyomatosis peritonealis disseminata (LPD) is a rare disease presenting as multiple peritoneal nodules of smooth muscle cells, mimicking peritoneal carcinomatosis. This disease usually pursues a benign course. We report one case of LPD in a 32-year-old woman, G2P1, without gynecological history. At term, she had an elective caesarean section during which several firm peritoneal nodules, ranging from 0.2 to 0.4 mm, were found. Microscopic examination showed a smooth-muscle cell proliferation with no mitosis, no atypia and no necrosis. Immunohistochemical analysis showed diffuse and strong staining for progesterone receptors and partial expression of estrogen receptors. The cells coexpressed smooth muscle actin, desmin, h-caldesmon, calretinin, WT1, and CD117 (KIT). They were weakly positive for EMA but negative for CD34. Proliferation index was low with 5% of cells being positive for MIB-1. This case of LPD proved to have an unusual immunohistochemistry profile raising the question of its real origin.
...
PMID:[Leiomyomatosis peritonealis disseminata: immunohistochemical profile and origin]. 1712 52

We report detailed clinical and pathologic features of four cases of anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma (ALK-DLBCL), a rare entity with only 29 currently reported cases. This study is the third largest of all reported series. Biopsies from four adult patients aged 41, 49, 53, and 71 years (three lymph nodes and one nasopharyngeal mass) exhibited immunoblastic/plasmablastic morphology. By immunohistochemistry and/or flow cytometry, they expressed cytoplasmic ALK-1, CD138, VS38 (3/3), monoclonal cytoplasmic light chain, CD45, EMA, CD4, and CD57 (2/3), and were negative for CD3, CD30, CD56, and TIA-1. Two showed variable CD79a expression, and one had rare CD20(+) cells. Two of three cases exhibited rare CD43(+) reactivity. One case showed scattered cytokeratin(+) cells, which could possibly lead to a misdiagnosis of carcinoma. After CHOP and radiotherapy, two stage I patients were free of disease at 58 and 36 months, whereas a stage IV patient was dead of disease at 22 months.
...
PMID:ALK-positive diffuse large B-cell lymphoma: report of four cases and review of the literature. 1727 65

Human primordial germ cells (PGCs) have proven to be a source of pluripotent stem cells called embryonic germ cells (EGCs). However, the developmental potency of these cells in the fetal gonad still remains elusive. Thus, this study provides a comprehensive analysis of pluripotent and germ cell marker expression in human fetal testis 7-15 weeks postfertilization (pF) and compares this expression to their ability to derive EGCs. Although the majority of germ cells expressed stem cell markers stage-specific embryonic antigen (SSEA) 1, SSEA4, EMA-1, and alkaline phosphatase, only a small percentage of those (<1%) expressed OCT4, CKIT, and NANOG. Specifically, the number of OCT4(+)/CKIT(+)/NANOG(+) cells significantly increased in the developing cords during weeks 7-9, followed by a gradual decline into week 15 pF. By week 15 pF, the remaining OCT4(+)/CKIT(+)/NANOG(+) cells were found in the cords surrounding the periphery of the testis, and the predominant germ cells, CKIT(+) cells, no longer expressed OCT4 or NANOG. Based on morphology and early germ cell marker expression, including VASA, PUM2, and DAZL, we suggest these cells are mitotically active gonocytes or prespermatogonia. Importantly, the number of OCT4(+) cells correlated with an increase in the number of EGC colonies derived in culture. Interestingly, two pluripotent markers, Tra-1-60 and Tra-1-81, although highly expressed in EGCs, were not expressed by PGCs in the gonad. Together, these results suggest that PGCs maintain expression of pluripotent stem cell markers during and after sexual differentiation of the gonad, albeit in very low numbers.
...
PMID:Expression of pluripotent stem cell markers in the human fetal testis. 1802 20


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>

---